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A 62-year-old man had a neuroendocrine tumor of the rectum treated by colon resection and radiation therapy. Three years later, he experienced diplopia and right periorbital swelling and also was found to have metastasis to the liver, lung, and groin. Indium-111 OctreoScan demonstrated a mass in the right orbit. Visual acuity was no light perception in the right eye (OD) and 20/30 in the left eye (OS). There was right eye proptosis of 10 mm on exophthalmometry and decreased ocular motility in all gazes. Magnetic resonance imaging (MRI) of the orbits revealed a 30-mm well-circumscribed intraconal mass adjacent to the medial rectus muscle displacing the optic nerve medially. The lesion was isointense on T1-weighted images with moderate enhancement after contrast. Enucleation and anterior orbitotomy were performed for the painful blind eye and tumor resection, respectively. Histopathology of the resected lesion showed metastatic neuroendocrine tumor with the tumor cells staining positive for chromogranin, synaptophysin, and neuron specific enolase. At 2 years follow-up, there was no orbital recurrence of the tumor; however, there was progression of the liver, lung, and skin metastases. Despite chemotherapy (carboplatin and TP-16) and external beam radiotherapy (EBRT) of extraorbital tumors, the patient died 3 years after orbital surgery.

Patient 2 is a 68-year-old male who was initially diagnosed with a well-differentiated G2 (Ki-67 10%) metastatic ileal NET in 2012. Post-resection of his primary, the patient began treatment with octreotide. Eventually, he enrolled on a clinical trial with 177Lu-DOTATATE PRRT. During a hospitalization in November 2017, while admitted for abdominal pain, he developed left peri-orbital swelling. This prompted an orbital MRI which demonstrated bilateral extraocular masses in his recti muscles (Fig. a). A subsequent biopsy confirmed metastatic NET. To manage his acute periorbital swelling, he was first treated with corticosteroids and later completed image-guided radiation therapy (IGRT) to 44 Gy to bilateral orbits in December 2017. The patient had a post-treatment MRI scan which demonstrated a decrease in signal abnormality and enhancement in the previously visualized lesions (Fig. b). Post-radiation, patient continued octreotide until his death in February 2018 due to progressive disease.

Write a detailed clinical case vignette based on the following key phrases: Neuroendocrine Tumor, Orbital Metastasis, Carcinoid Syndrome

A 62-year-old man had a neuroendocrine tumor of the rectum treated by colon resection and radiation therapy. Three years later, he experienced diplopia and right periorbital swelling and also was found to have metastasis to the liver, lung, and groin. Indium-111 OctreoScan demonstrated a mass in the right orbit. Visual acuity was no light perception in the right eye (OD) and 20/30 in the left eye (OS). There was right eye proptosis of 10 mm on exophthalmometry and decreased ocular motility in all gazes. Magnetic resonance imaging (MRI) of the orbits revealed a 30-mm well-circumscribed intraconal mass adjacent to the medial rectus muscle displacing the optic nerve medially. The lesion was isointense on T1-weighted images with moderate enhancement after contrast. Enucleation and anterior orbitotomy were performed for the painful blind eye and tumor resection, respectively. Histopathology of the resected lesion showed metastatic neuroendocrine tumor with the tumor cells staining positive for chromogranin, synaptophysin, and neuron specific enolase. At 2 years follow-up, there was no orbital recurrence of the tumor; however, there was progression of the liver, lung, and skin metastases. Despite chemotherapy (carboplatin and TP-16) and external beam radiotherapy (EBRT) of extraorbital tumors, the patient died 3 years after orbital surgery.

Patient 5 is a 61-year-old female who was diagnosed with a well differentiated G2 (Ki-67 5%) metastatic small intestine (not otherwise specified) NET in February 2010. She remained symptom free until April 2014 when she was started on monthly octreotide. In the setting of progressive disease, she subsequently received sunitinib and everolimus. She began to develop progressive diplopia and right ocular pain in August 2016. This prompted an orbital MRI which revealed bilateral recti masses in the right lateral and superior left medial muscles. She was evaluated by radiation oncology shortly thereafter and was treated with IGRT to 52Gy, which completed in October 2016. Although her right ocular pain improved, she had persistent diplopia. Her post-treatment orbital MRI in January 2017 revealed a mild increase in size of her right lateral rectus mass (Fig. a). She was then started on capecitabine in January 2017 on a two week on, one week off regimen schedule. Patient also established care with ophthalmology at this time. She achieved stable disease in her orbits with symptomatic improvement and did not demonstrate any evidence of visual field deficits. She continues capecitabine and her last MRI in July 2018 revealed ongoing shrinkage of her right lateral rectus mass (Fig. b); her recent 68Ga-DOTATATE PET/CT from October 2018 shows residual SSTR avidity within her bilateral recti muscles (Fig. c and d).

Write a detailed clinical case vignette based on the following key phrases: Neuroendocrine Tumor, Orbital Metastasis, Carcinoid Syndrome

A 73-year-old woman presented with drooping of her right upper lid. Twelve years prior she had undergone resection of neuroendocrine tumor in the upper lobe of her right lung and a metastasis to the ninth thoracic vertebra, which was treated with EBRT. Subsequent 5-HIAA study and Indium-111 OctreoScan were within normal limits.\nVisual acuity was 20/20 OD and 20/40 OS. There was 1 mm proptosis and 3 mm blepharoptosis on the right side. MRI of the orbit revealed a heterogeneous lesion with contrast enhancement in the superior right orbit measuring 13 × 18 × 14 mm and a smaller lesion adjacent to the lacrimal gland []. Fine needle aspiration biopsy of the orbital tumor was consistent with neuroendocrine tumor with positive staining for chromogranin, AE1/AE3, synaptophysin, and CD56 but negative staining for CD45 consistent with atypical carcinoid. Excisional biopsy revealed a low-grade non-small cell neuroendocrine tumor. Residual orbital tumor was treated with EBRT. Within the next 2–6 years, she developed recurrent lesions in both lungs and multiple metastatic lesions in the thoracic vertebra, breast, scalp, and cavernous sinus which were treated with a combination of surgical excision, chemotherapy (carboplatin, etoposide, and paclitaxel), and EBRT. Six years after initial presentation, the patient was alive with no orbital tumor recurrence [].

Patient 4 is a 72-year-old gentleman who was diagnosed with a well differentiated G2 metastatic ileal NET in 2006. He started monthly octreotide shortly thereafter in 2007. He was incidentally noted to have a left lateral rectus mass in March 2007 during a brain MRI. This lesion was monitored with serial MRI and remained stable until 2015 when it began to grow (Fig. c). The patient developed progressive diplopia and proptosis in 2015 which prompted an evaluation by radiation oncology. He received stereotactic radiosurgery (SRS) administered over 5 fractions to the site in February 2015 at VUMC and his diplopia and proptosis resolved within several months of treatment completion. He has unfortunately developed complications from his other sites of metastatic involvement including right sided nephrostomy tube placement from ureteral obstruction and an end colostomy due to recurrent small bowel obstructions from mesenteric tethering. His disease remains radiographically stable on his 68Ga-DOTATATE PET-CT from August 2018 (Fig. a and b).

Write a detailed clinical case vignette based on the following key phrases: Neuroendocrine Tumor, Orbital Metastasis, Carcinoid Syndrome

A 73-year-old woman presented with drooping of her right upper lid. Twelve years prior she had undergone resection of neuroendocrine tumor in the upper lobe of her right lung and a metastasis to the ninth thoracic vertebra, which was treated with EBRT. Subsequent 5-HIAA study and Indium-111 OctreoScan were within normal limits.\nVisual acuity was 20/20 OD and 20/40 OS. There was 1 mm proptosis and 3 mm blepharoptosis on the right side. MRI of the orbit revealed a heterogeneous lesion with contrast enhancement in the superior right orbit measuring 13 × 18 × 14 mm and a smaller lesion adjacent to the lacrimal gland []. Fine needle aspiration biopsy of the orbital tumor was consistent with neuroendocrine tumor with positive staining for chromogranin, AE1/AE3, synaptophysin, and CD56 but negative staining for CD45 consistent with atypical carcinoid. Excisional biopsy revealed a low-grade non-small cell neuroendocrine tumor. Residual orbital tumor was treated with EBRT. Within the next 2–6 years, she developed recurrent lesions in both lungs and multiple metastatic lesions in the thoracic vertebra, breast, scalp, and cavernous sinus which were treated with a combination of surgical excision, chemotherapy (carboplatin, etoposide, and paclitaxel), and EBRT. Six years after initial presentation, the patient was alive with no orbital tumor recurrence [].

Patient 2 is a 68-year-old male who was initially diagnosed with a well-differentiated G2 (Ki-67 10%) metastatic ileal NET in 2012. Post-resection of his primary, the patient began treatment with octreotide. Eventually, he enrolled on a clinical trial with 177Lu-DOTATATE PRRT. During a hospitalization in November 2017, while admitted for abdominal pain, he developed left peri-orbital swelling. This prompted an orbital MRI which demonstrated bilateral extraocular masses in his recti muscles (Fig. a). A subsequent biopsy confirmed metastatic NET. To manage his acute periorbital swelling, he was first treated with corticosteroids and later completed image-guided radiation therapy (IGRT) to 44 Gy to bilateral orbits in December 2017. The patient had a post-treatment MRI scan which demonstrated a decrease in signal abnormality and enhancement in the previously visualized lesions (Fig. b). Post-radiation, patient continued octreotide until his death in February 2018 due to progressive disease.

Write a detailed clinical case vignette based on the following key phrases: Neuroendocrine Tumor, Orbital Metastasis, Carcinoid Syndrome

A 73-year-old woman presented with drooping of her right upper lid. Twelve years prior she had undergone resection of neuroendocrine tumor in the upper lobe of her right lung and a metastasis to the ninth thoracic vertebra, which was treated with EBRT. Subsequent 5-HIAA study and Indium-111 OctreoScan were within normal limits.\nVisual acuity was 20/20 OD and 20/40 OS. There was 1 mm proptosis and 3 mm blepharoptosis on the right side. MRI of the orbit revealed a heterogeneous lesion with contrast enhancement in the superior right orbit measuring 13 × 18 × 14 mm and a smaller lesion adjacent to the lacrimal gland []. Fine needle aspiration biopsy of the orbital tumor was consistent with neuroendocrine tumor with positive staining for chromogranin, AE1/AE3, synaptophysin, and CD56 but negative staining for CD45 consistent with atypical carcinoid. Excisional biopsy revealed a low-grade non-small cell neuroendocrine tumor. Residual orbital tumor was treated with EBRT. Within the next 2–6 years, she developed recurrent lesions in both lungs and multiple metastatic lesions in the thoracic vertebra, breast, scalp, and cavernous sinus which were treated with a combination of surgical excision, chemotherapy (carboplatin, etoposide, and paclitaxel), and EBRT. Six years after initial presentation, the patient was alive with no orbital tumor recurrence [].

Patient 5 is a 61-year-old female who was diagnosed with a well differentiated G2 (Ki-67 5%) metastatic small intestine (not otherwise specified) NET in February 2010. She remained symptom free until April 2014 when she was started on monthly octreotide. In the setting of progressive disease, she subsequently received sunitinib and everolimus. She began to develop progressive diplopia and right ocular pain in August 2016. This prompted an orbital MRI which revealed bilateral recti masses in the right lateral and superior left medial muscles. She was evaluated by radiation oncology shortly thereafter and was treated with IGRT to 52Gy, which completed in October 2016. Although her right ocular pain improved, she had persistent diplopia. Her post-treatment orbital MRI in January 2017 revealed a mild increase in size of her right lateral rectus mass (Fig. a). She was then started on capecitabine in January 2017 on a two week on, one week off regimen schedule. Patient also established care with ophthalmology at this time. She achieved stable disease in her orbits with symptomatic improvement and did not demonstrate any evidence of visual field deficits. She continues capecitabine and her last MRI in July 2018 revealed ongoing shrinkage of her right lateral rectus mass (Fig. b); her recent 68Ga-DOTATATE PET/CT from October 2018 shows residual SSTR avidity within her bilateral recti muscles (Fig. c and d).

Write a detailed clinical case vignette based on the following key phrases: Neuroendocrine Tumor, Orbital Metastasis, Carcinoid Syndrome

Patient 4 is a 72-year-old gentleman who was diagnosed with a well differentiated G2 metastatic ileal NET in 2006. He started monthly octreotide shortly thereafter in 2007. He was incidentally noted to have a left lateral rectus mass in March 2007 during a brain MRI. This lesion was monitored with serial MRI and remained stable until 2015 when it began to grow (Fig. c). The patient developed progressive diplopia and proptosis in 2015 which prompted an evaluation by radiation oncology. He received stereotactic radiosurgery (SRS) administered over 5 fractions to the site in February 2015 at VUMC and his diplopia and proptosis resolved within several months of treatment completion. He has unfortunately developed complications from his other sites of metastatic involvement including right sided nephrostomy tube placement from ureteral obstruction and an end colostomy due to recurrent small bowel obstructions from mesenteric tethering. His disease remains radiographically stable on his 68Ga-DOTATATE PET-CT from August 2018 (Fig. a and b).

Patient 2 is a 68-year-old male who was initially diagnosed with a well-differentiated G2 (Ki-67 10%) metastatic ileal NET in 2012. Post-resection of his primary, the patient began treatment with octreotide. Eventually, he enrolled on a clinical trial with 177Lu-DOTATATE PRRT. During a hospitalization in November 2017, while admitted for abdominal pain, he developed left peri-orbital swelling. This prompted an orbital MRI which demonstrated bilateral extraocular masses in his recti muscles (Fig. a). A subsequent biopsy confirmed metastatic NET. To manage his acute periorbital swelling, he was first treated with corticosteroids and later completed image-guided radiation therapy (IGRT) to 44 Gy to bilateral orbits in December 2017. The patient had a post-treatment MRI scan which demonstrated a decrease in signal abnormality and enhancement in the previously visualized lesions (Fig. b). Post-radiation, patient continued octreotide until his death in February 2018 due to progressive disease.

Write a detailed clinical case vignette based on the following key phrases: Neuroendocrine Tumor, Orbital Metastasis, Carcinoid Syndrome

Patient 5 is a 61-year-old female who was diagnosed with a well differentiated G2 (Ki-67 5%) metastatic small intestine (not otherwise specified) NET in February 2010. She remained symptom free until April 2014 when she was started on monthly octreotide. In the setting of progressive disease, she subsequently received sunitinib and everolimus. She began to develop progressive diplopia and right ocular pain in August 2016. This prompted an orbital MRI which revealed bilateral recti masses in the right lateral and superior left medial muscles. She was evaluated by radiation oncology shortly thereafter and was treated with IGRT to 52Gy, which completed in October 2016. Although her right ocular pain improved, she had persistent diplopia. Her post-treatment orbital MRI in January 2017 revealed a mild increase in size of her right lateral rectus mass (Fig. a). She was then started on capecitabine in January 2017 on a two week on, one week off regimen schedule. Patient also established care with ophthalmology at this time. She achieved stable disease in her orbits with symptomatic improvement and did not demonstrate any evidence of visual field deficits. She continues capecitabine and her last MRI in July 2018 revealed ongoing shrinkage of her right lateral rectus mass (Fig. b); her recent 68Ga-DOTATATE PET/CT from October 2018 shows residual SSTR avidity within her bilateral recti muscles (Fig. c and d).

Patient 4 is a 72-year-old gentleman who was diagnosed with a well differentiated G2 metastatic ileal NET in 2006. He started monthly octreotide shortly thereafter in 2007. He was incidentally noted to have a left lateral rectus mass in March 2007 during a brain MRI. This lesion was monitored with serial MRI and remained stable until 2015 when it began to grow (Fig. c). The patient developed progressive diplopia and proptosis in 2015 which prompted an evaluation by radiation oncology. He received stereotactic radiosurgery (SRS) administered over 5 fractions to the site in February 2015 at VUMC and his diplopia and proptosis resolved within several months of treatment completion. He has unfortunately developed complications from his other sites of metastatic involvement including right sided nephrostomy tube placement from ureteral obstruction and an end colostomy due to recurrent small bowel obstructions from mesenteric tethering. His disease remains radiographically stable on his 68Ga-DOTATATE PET-CT from August 2018 (Fig. a and b).

Write a detailed clinical case vignette based on the following key phrases: Neuroendocrine Tumor, Orbital Metastasis, Carcinoid Syndrome

Patient 5 is a 61-year-old female who was diagnosed with a well differentiated G2 (Ki-67 5%) metastatic small intestine (not otherwise specified) NET in February 2010. She remained symptom free until April 2014 when she was started on monthly octreotide. In the setting of progressive disease, she subsequently received sunitinib and everolimus. She began to develop progressive diplopia and right ocular pain in August 2016. This prompted an orbital MRI which revealed bilateral recti masses in the right lateral and superior left medial muscles. She was evaluated by radiation oncology shortly thereafter and was treated with IGRT to 52Gy, which completed in October 2016. Although her right ocular pain improved, she had persistent diplopia. Her post-treatment orbital MRI in January 2017 revealed a mild increase in size of her right lateral rectus mass (Fig. a). She was then started on capecitabine in January 2017 on a two week on, one week off regimen schedule. Patient also established care with ophthalmology at this time. She achieved stable disease in her orbits with symptomatic improvement and did not demonstrate any evidence of visual field deficits. She continues capecitabine and her last MRI in July 2018 revealed ongoing shrinkage of her right lateral rectus mass (Fig. b); her recent 68Ga-DOTATATE PET/CT from October 2018 shows residual SSTR avidity within her bilateral recti muscles (Fig. c and d).

Patient 2 is a 68-year-old male who was initially diagnosed with a well-differentiated G2 (Ki-67 10%) metastatic ileal NET in 2012. Post-resection of his primary, the patient began treatment with octreotide. Eventually, he enrolled on a clinical trial with 177Lu-DOTATATE PRRT. During a hospitalization in November 2017, while admitted for abdominal pain, he developed left peri-orbital swelling. This prompted an orbital MRI which demonstrated bilateral extraocular masses in his recti muscles (Fig. a). A subsequent biopsy confirmed metastatic NET. To manage his acute periorbital swelling, he was first treated with corticosteroids and later completed image-guided radiation therapy (IGRT) to 44 Gy to bilateral orbits in December 2017. The patient had a post-treatment MRI scan which demonstrated a decrease in signal abnormality and enhancement in the previously visualized lesions (Fig. b). Post-radiation, patient continued octreotide until his death in February 2018 due to progressive disease.

Write a detailed clinical case vignette based on the following key phrases: Neuroendocrine Tumor, Orbital Metastasis, Carcinoid Syndrome

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 75-year-old man presented with recurrent pruritic eczema of the penis and scrotum that was refractory to medication of the local dermatologic clinic for one and a half years. The lesion consisted of an erythematous and indurated plaque with ulceration and exudation (). Review of the patient's history revealed that he underwent right hemicolectomy for adenocarcinoma in the ascending colon when he was 62 years old. He remained under regular follow-up for 13 years, during which no metastases were reported. Examination of skin biopsy from the inguinal folds revealed large cells with clear to eosinophilic cytoplasm that had infiltrated into the epidermis, singly and in clusters (). The initial histologic diagnosis of the skin biopsy was EMPD. The preoperative instrumental investigations showed no evidence of recurrence on the previous surgical site or other underlying internal malignancy. The patient underwent a mapping biopsy at multiple sites to accurately establish the boundary of the lesion, followed by wide excision and closure of the defect with a skin graft.\nThe resected perineum and penile shaft showed ill-defined indurated nodules with a firm consistency. The lesion in the overlying epidermis measured 6 cm, with a 1.5-cm-sized mass in the dermis. The cut surface was solid and gray-white with focal mucinous appearance. On histologic examination, the lowpower view showed a relatively well-defined mass occupying the dermis and subcutis (). The overlying epidermis showed irregular epidermal hyperplasia containing scattered large cells arranged singly or in groups, as in the previous skin biopsy, suggesting an epidermal pagetoid spread of underlying tumor cells (). The tumor showed two composite features in which solid nests of poorly differentiated carcinoma were juxtaposed with paucicellular mucinous lakes with floating tumor cell clusters (). The latter accounted for the majority of the tumor, about 70%, and the rest consisted of poorly differentiated components. Tumor cells in both the mucinous pool and more cellular area revealed the same histological features of abundant eosinophilic cytoplasm and large vesicular nuclei with frequent mitoses up to 7/10 high power field (, ). In situ lesions similar to ductal carcinoma in situ of the breast were also observed () with colonization of the hair follicles (). Overall histology suggested a mucinous carcinoma with minor poorly differentiated components.\nHistological differential diagnosis included PCMC and late metastasis of the previously diagnosed colon cancer. Histologic review of the patient’s previous colon cancer showed a 4.3-cm moderately differentiated adenocarcinoma with the assigned stage of T3N0M0. The tumor consisted of well-formed or cribriform patterns of tumor glands with frequently observed dirty necrosis (). There was no mucin production observed in the skin lesion.\nImmunohistochemical studies were performed. The Paget’s and dermal tumor cells of the skin lesion were positive for cytokeratin (CK) 7, CK20, gross cystic disease fluid protein-15 (GCDFP-15) (–), and carcinoembryonic antigen (CEA), but negative for caudal-related homeobox gene 2 (CDX-2). On the other hand, tumor cells of the colon cancer were positive for CK20, CDX-2, CEA, but not gross cystic disease fluid protein 15 or CK7. Additional staining of human epidermal growth factor receptor 2 and estrogen receptor (ER) was observed in the skin lesion (, ), but the lesion was negative for progesterone receptor (PR). Myoepithelial cells surrounding the carcinoma in situ were highlighted by CK5/6 immunostaining (). The overall immunohistochemical findings of the skin lesion were different from those of colon cancer but similar to those of breast cancer, but similar to those of breast cancer, indicating sweat gland origin. On the basis of these histologic and immunohistochemical findings, the two malignancies were considered to differ in origin, and the present case was a primary mucinous carcinoma of the skin.\nAnother challenge was to determine whether the minor poorly differentiated components were eccrine or apocrine in origin because there was no clear distinction in histologic and immunohistochemical features. We concluded apocrine differentiation of the lesion due to the location in the groin, where apocrine glands are many in number, and to the resemblance of apocrine carcinoma in the breast even though decapitation secretion was absent. In addition, close approximation and colonization of the hair follicle () favored the origin of the follicularapocrine unit.\nThe final pathologic diagnosis was PCMC with EMPD showing apocrine differentiation.\nThis case was approved by the institutional review board (IRB) of Kosin University Gospel Hospital, and informed consent was waived by the IRB (IRB reference number: 2017-10-010).

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 51-year-old male patient presented to the outpatient clinic with a painless nodule near the right lower eyelid. The mass had grown slowly over the past 3 years, and measured 0.9×0.7 cm during examination (). The lesion appeared benign in clinical examination, and under local anesthesia, the patient underwent a 3-mm-margin elliptical excision and primary closure. Histologic study of this first specimen revealed epithelial cell islands floating in a mucin lake, with the final diagnosis being mucinous carcinoma. The epithelial component showed a solid and cribriform growth pattern and the tumor cells revealed moderate nuclear atypia and a mitotic aspect. Additional immunostaining study revealed that the tumor cells were immunopositive for cytokeratins (CK)-7 and negative for CK-20 with 10% Ki-67 positivity in the active area (). We performed re-excision of the surgical site with an additional 3-mm margin, which was free of malignant histology. The possibility of a metastatic primary mucinous carcinoma (e.g., breast or gastrointestinal tract) could not be completely ruled out, but a systemic evaluation by positron emission tomography-computed tomography revealed no other suspected site of malignancy. During a close follow-up over 2 years, the patient did not experience any postoperative complication or recurrence ().

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors