bluuebunny/crossref_metadata_2025_split huggingface dataset:
Gastric Adenomyoma: Salient Imitator
Authors: Kobalka A J , Singh S 
Published: October 2021

Abstract

Introduction/Objective
Gastric adenomyoma is a lesion characterized by proliferation of epithelium and smooth muscle in the stomach or small bowel. By ultrasound, gastric adenomyoma usually appears as a submucosal nodule with a cystic center. They can mimic many benign and malignant conditions and are an important differential diagnosis to consider in fine needle aspiration of submucosal upper gastrointestinal lesions.

Methods/Case Report
We present a 30-year-old male who presented with abdominal pain, dyspepsia, and heartburn. Esophagogastroduodenoscopy demonstrated a 10 mm well-circumscribed hypoechoic mass in the antral submucosa along the greater curvature of the stomach. Two years later, the lesion was 18.6 mm in greatest dimension and fine needle aspiration was performed. Cell block showed foci of benign gastric glands and bland smooth muscle tissue, positive for smooth muscle actin and desmin, and negative for CD34 and c-kit (CD117). Gastrointestinal stromal tumor was the main differential diagnosis and adenomyosis was not considered, thus, the biopsy was thought to represent normal gastric wall, and the glands were dismissed as contaminant from the aspiration. A subsequent ultrasound two years later again demonstrated the lesion, now 21.5 mm in greatest dimension, and definitive resection was performed. Histologic examination showed a segment of stomach with gastric antral-type mucosa and an underlying mass forming lesion consisting of scattered gastric type glands admixed with smooth muscle bundles, consistent with gastric adenomyoma. The glands were completely surrounded by smooth muscle and lamina propria. Our patient has been asyptomatic one year since resection of the gastric adenomyoma.

Results (if a Case Study enter NA)
NA

Conclusion
Gastric adenomyoma is an important differential of intramural spindle cell neoplasms of the gastrointestinal tract alongside many benign and malignant conditions. Consideration of gastric adenomyoma at the time of the original biopsy would have spared the patient uncertainty regarding their prognosis and allowed for definitive resection two years earlier.
H. pylori Pattern Gastritis with Negative Helicobacter Immunohistochemical Stain: Does A Specific Comment in Pathology Report Impact Clinical Management?
Authors: Hu X , Lucas E , Hammer S , Gopal P , Bhalla A , Panarelli N , Westerhoff M , Cheng J , Nalbantoglu I 
Published: October 2021

Abstract

Introduction/Objective
The clinical significance of H. pylori (HP) pattern of gastritis with a negative Helicobacter IHC stain on gastric biopsy is unclear. Some pathologists report this pattern in cases that are highly suggestive of HP infection with a comment raising the possibility of HP infection; however, the subsequent clinical management of these patients has not been well described.

Methods/Case Report
We conducted a retrospective comparison study of patients with gastric biopsy between 2016 and 2019. Group 1 included patients with chronic active or chronic inactive gastritis and negative HP IHC with a comment stating the gastritis pattern is suggestive of HP. Group 2 included patients with chronic active or chronic inactive gastritis and negative HP IHC with no comment about HP pattern.

Results (if a Case Study enter NA)
We identified 60 patients in Group 1 which were compared to 63 patients in Group 2. Group 1 more frequently had history of HP (48.3% vs. 29.1%, p&lt;0.05). After diagnosis, Group 1 more frequently received treatment (51.7% vs. 20.6%, p&lt;0.001). Of those who received treatment, Group 1 more frequently received HP treatment (triple or quadruple therapy; 21.7% vs. 1.6%, p&lt;0.001). History of HP did not affect whether a patient was treated (p&gt;0.05). Following post-biopsy HP treatment, more patients in Group 1 received fecal antigen test (23.7% vs. 5.5%, p&lt;0.01). Age, gender, NSAID and PPI use did not differ between groups.

Conclusion
Adding the diagnostic comment raising the possibility of HP for patients with HP pattern gastritis with negative HP IHC changes clinical management and it is independent of patients’ prior HP history.
Goblet cell adenocarcinoma of the appendix: An incidental finding of a rare tumor which was recently renamed in 2019 World Health Organization classification update
Authors: Rajack F , Abdulbaki R , Chen W 
Published: October 2021

Abstract

Introduction/Objective
Goblet cell adenocarcinoma (formerly goblet cell carcinoid) is a rare tumor almost exclusively involving the appendix. It is an amphicrine tumor consisting of both epithelial and neuroendocrine elements containing goblet cells. In the 2019 WHO classification update, GCC was reclassified as goblet cell adenocarcinoma of the appendix as it is recognized to have predominantly mucin secreting cells and a minor neuroendocrine component.

Methods/Case Report
A 72 year old,morbidly obese male presented with a 2 week history of abdominal pain and bilious vomiting. Imaging revealed a peri-appendiceal abscess. Due to the chronicity of his symptoms, he was treated conservatively with drainage of the abscess and intravenous antibiotics. He responded well and underwent elective laparoscopic appendectomy; at which time, the appendix appeared grossly unremarkable, measuring 5.1 x 0.9 x 0.3 cm with a wall thickness of 0.5 cm. Microscopically small nests and clusters of goblet cells circumferentially invaded dense collagenous stroma in the appendiceal wall. The neoplastic cells had large mucin vacuoles and small basally located nuclei. The cells were positive for CK20, CDX2, CD56, synaptophysin and focally positive for chromogranin. Ki-67 was 10%. CK7 was negative. These tumors are now graded and staged similar to colonic adenocarcinomas according to the 5 th edition of the World Health Organization Classification of Tumors - Digestive System Tumors.

Results (if a Case Study enter NA)
NA

Conclusion
This case demonstrates that this rare tumor may be an incidental finding in an elderly patient presenting with appendicitis; accurate diagnosis of this rare tumor may require submission of the entire appendix for microscopic evaluation.
Case of renal cell carcinoma with immunotherapy effect mimicking xanthogranulomatous pyelonephritis
Authors: Wang Y , Ganesan S , Rayes-Danan R , Williamson S 
Published: October 2021

Abstract

Introduction/Objective
The histologic features of renal cell carcinoma (RCC) after immunotherapy are not fully established. We report the pathologic findings of one case of clear cell RCC after treatment with pembrolizumab which is a humanized monoclonal anti-PD1 antibody.

Methods/Case Report
46-year-old man completed three cycles of pembrolizumab for recurrent basal cell carcinoma. A 5.0 cm mass was incidentally found within left kidney during staging CT scan. He subsequently had left radical nephrectomy. Pathologic examination of nephrectomy specimen identified one 5.0 cm, well-circumscribed, solid mass in the upper pole, confined to the kidney with predominantly hemorrhagic, focally golden-yellow soft cut surface. The remaining parenchyma appears unremarkable. The pelvicalyceal system is not dilated. Sections of tumor show predominance of foamy histiocytes, lymphoplasmacytic infiltrate, and scattered cells having pale or eosinophilic cytoplasm, conspicuous nucleoli and low nuclear: cytoplasm ratio. Cholesterol clefts and hemosiderin deposits are noted. The immunostaining profile highlights areas suspicious for viable tumor cells by using pan-keratin, CK8/18, EMA, CA9 and CD10. Focal positivity in the same area is noted by the stains of PAX 8 and AMACR. However, with numerous histiocytes, it is difficult to determine if the tumor cells are positive for Vimentin. Moreover CD 117 is negative in the tumor cells.

Results (if a Case Study enter NA)
NA

Conclusion
Differential diagnosis includes renal cell carcinoma and xanthogranulomatous pyelonephritis. The overall findings support a clear cell RCC affected by the immunotherapy. With immunotherapy-based combinations becoming standard of care in advanced malignancies, it makes the pathological diagnosis more challenging and difficult, especially for incidental tumors.
Capillary Hemangioma of the Testis: A Case Report and Literature Review
Authors: Ahmad A , Shakil K B 
Published: October 2021

Abstract

Introduction/Objective
Testicular capillary hemangioma is a rare benign vascular tumor and typically occurs in patients younger than 20 years. Study suggests that the mean age of these patients is usually 25.1 ± 22.7 years. We report a case of a 63-year-old man who underwent an uncomplicated radical orchiectomy for scrotal irritation and testicular fullness.

Methods/Case Report
Clinically he was found to have a large testicular mass. Ultrasound showed cystocele and an intratesticular tumor. Increased LDH was also found. Gross examination showed a single large cystic cavity containing yellow-colored fluid and a well-circumscribed tan-brown nodule measuring 2.5 × 1.8 × 1.0 cm within the testicular parenchyma. There was no extension into the tunica albuginea. Microscopic examination showed groups of capillaries of varying sizes in a lobular arrangement with bland spindle-like endothelial cells lining these capillary structures. The circumscribed vascular lesion was well-demarcated from the testicular parenchyma by a fibrous 0.1-cm pseudocapsule. The benign nature of this lesion was suggested by the lack of any mitotic activity, anaplasia, or dysplasia. Immunohistochemical staining was strongly positive for endothelial markers (CD31 and CD 34), and negative for mesothelial (WT1) and lymphatic markers (D2-40), excluding an adenomatoid tumor. Based on these characteristic morphological and immunohistochemical findings, the diagnosis of testicular capillary hemangioma was made. After 6 months of follow-up, the patient was well without any clinical evidence of recurrence.

Results (if a Case Study enter NA)
NA

Conclusion
While rarity of testicular capillary hemangioma impedes significant changes in how we approach adult testicular masses, the possibility of a benign tumor should always be in mind to consider. This is especially pertinent when patients fall outside the typical age range for germ-cell tumors, and for patients with a solitary testicular mass.
Mesonephric Like-Carcinoma (MLCA) of the Uterus with a Predominant Spindled Morphology: A Case Report
Authors: Ibrahim E , Sheridan T , Mandavilli S 
Published: October 2021

Abstract

Introduction/Objective
Mesonephric-like carcinoma of the uterus is an increasingly recognized carcinoma with mesonephric differentiation, but without association with mesonephric remnants. We present a case of a 60-year-old woman presented with postmenopausal bleeding.

Methods/Case Report
Pelvic MRI showed possibly a cervical lobulated lesion (4.4cm) extending into the endocervical canal. Initial biopsy of this mass showed a spindle cell neoplasm raising possibility of an endometrial stromal sarcoma. On subsequent radical hysterectomy, there was a mass arising in the lower uterine segment (LUS) with circumferential cervical involvement. The tumor was comprised of sheets of epithelioid to spindle cells with scant cytoplasm and indistinct cell borders. Abundant mitotic figures and foci of necrosis were identified. Focal areas showed dense sclerosis with cords of cells, and only rare areas showed tubule formation with scant secretions. By immunohistochemistry (IHC), the tumor cells were positive for keratin AE1/AE3 (strong, diffuse), TTF-1, p63, p16, CD10 (with luminal accentuation); PAX8, desmin and caldesmon showed focal/rare positivity. Other markers were negative, including GATA3 (patchy, weak), ER and PR. Mismatch repair proteins were intact. Next-generation sequencing (NGS) revealed a KRAS mutation. Considering strong expression of epithelial markers, focal tubule formation with positive TTF-1 and negative GATA-3 labeling, and absence of identified mesonephric remnants, the tumor was classified as a high-grade mesonephric-like carcinoma of the uterus (LUS). Pelvic lymph nodes were negative (pT2 N0), and the patient is receiving cisplatin and external beam radiation.

Results (if a Case Study enter NA)
NA

Conclusion
MLCA with a prominent spindled/sarcomatoid component can be difficult to diagnose. Ancillary testing including a broad IHC panel with TTF-1, GATA-3 and NGS may be useful to aid in the diagnosis.
Extrauterine Epithelioid Trophoblastic Tumor (ETT) Arising from an Antecedent Undiagnosed Molar Pregnancy Presenting as an Adnexal mass: A Case Report
Authors: Gallardo J , Hummel K , McCluskey K , Masand R , Sunde J 
Published: October 2021

Abstract

Introduction/Objective
Epithelioid trophoblastic tumor (ETT) is an extremely rare neoplasm derived from chorionic type intermediate trophoblast. ETT usually follows an antecedent term pregnancy but can also follow spontaneous abortions or molar pregnancy. ETT most often arises from the endometrium, followed by the cervix. Extrauterine ETT are extremely rare, with few cases reported in literature.

Methods/Case Report
A 41-year-old woman with three term pregnancies presented with abdominal pain, ten years after her last pregnancy. Imaging findings of a 3.5 cm adnexal mass coupled with an elevated serum β-hCG (~ 900 mIU/ml), led to the suspicion of an ectopic pregnancy. Hysterectomy with salpingectomy revealed a 4.7 cm, tan- yellow, necrotic mass in the adnexal region abutting but distinct from the uterine serosa. Histologic evaluation showed a well- circumscribed tumor with pushing borders. The tumor cells were epithelioid with well-defined eosinophilic cytoplasm, monomorphic nuclei, frequent mitosis, and abundant geographic necrosis. The tumor cells were positive for β-hCG, GATA-3, PLAP and inhibin, with focal weak staining squamous markers p63 and p40. DNA fingerprinting analysis, performed to confirm the diagnosis of ETT, revealed a homozygous tumor with two copies of non-maternal genes indicating that the antecedent index gestation giving rise to the tumor was an undiagnosed hydatidiform mole. Following surgery, serum β-hCG levels were normal and the patient is currently on surveillance.

Results (if a Case Study enter NA)
NA

Conclusion
We present an extremely rare case of extrauterine ETT arising from a previously undetected molar pregnancy. The diagnosis should be suspected when a mass is observed at extrauterine sites with elevated β-hCG levels in patients with or without vaginal bleeding. Histologic differential of squamous cell carcinoma needs to be ruled out with immunostains. Due to its rarity and highly variable presentation, this entity remains a diagnostic challenge. DNA fingerprinting analysis demonstrating non-maternal genes can help confirm the diagnosis of ETT.
A Rare Case of Cavernous Angiomatosis of the Uterus
Authors: Prakash V , Middleton C E , Shenoy V 
Published: October 2021

Abstract

Introduction/Objective
Angiomatosis is a rare, non-neoplastic vascular malformation characterized by the proliferation of blood vessels which involve skin, soft tissue, and bone. Angiomatosis of the female genital tract is extremely rare with very few cases described in the English literature.

Methods/Case Report
We present a case of an unexpected finding of angiomatosis of the uterus and cervix in a 34- year-old nulliparous female with a history of abnormal uterine bleeding and dysmenorrhea of one year duration. Imaging studies showed an anteverted uterus measuring 9 cm × 6 cm × 5 cm with thickened irregular endometrium, blood clots within the endometrial cavity, and subserosal uterine fibroids. A subsequent endometrial curettage showed benign superficial endometrium. Hysterectomy was performed and histopathological examination revealed diffuse haphazardly arranged large vascular channels within the uterine wall and cervical stroma. A diagnosis of cavernous angiomatosis of the uterus and cervix was made.

Results (if a Case Study enter NA)
NA

Conclusion
Angiomatosis of the female genital tract is a rare vascular proliferation that tends to grow at a slow rate and cause heavy menstrual bleeding in otherwise healthy patients. Differential diagnoses of angiomatosis include other vascular tumors such as hemangiomas, lymphangioma, arteriovenous malformation and papillary endothelial hyperplasia. Clinical examination and investigations like hysteroscopy, endometrial curettage and ultrasonography may not help in accurate diagnosis. Definitive diagnosis relies heavily on histopathological examination. Treatment of symptomatic angiomatosis is purely surgical and in our case, was curative. Following treatment, recurrences can occur and appropriate surveillance is recommended. Malignant transformation have not been reported. It is important to be aware of this rare entity to make an accurate diagnosis.
Characterization of A Rare Case of Vulvar Epithelioid Sarcoma with local recurrence and metastases
Authors: Shkolnik E , Cai D 
Published: October 2021

Abstract

Introduction/Objective
Epithelioid sarcoma (ES) is a rare, malignant mesenchymal neoplasm that has a known tendency for local recurrence, regional lymph node involvement, and distant metastases. Two histologic variants have been recognized: classic ES also known as the distal type, and proximal-type ES (PES). The classic ES is common in young adults. It occurs more frequently in the distal upper extremities followed by the distal lower limbs and has a male prevalence of 2:1. Conversely, PES commonly involves deep tissues in the pelvic region, including the genital area. It tends to occur in older patients and follows a more aggressive clinical course. In the female genital tract, PES occurs most frequently in the vulva. The incidence of primary sarcoma of the vulva accounts for 1.5-5% of all malignant tumors, making PES a very rare incidence.

Methods/Case Report
Here we report a 60-year-old female diagnosed with vulvar epithelioid sarcoma treated with a right radical vulvectomy and bilateral inguinal lymph node dissection in 2008. In 2017, further surgery and adjuvant radiation were given for local recurrence. In 2020, the patient developed left hip pain and was found to have an expansile lytic lesion in the left proximal femur. Extensive resection was performed. Grossly the vulvar lesion was nodular with diffuse hemorrhage, degeneration, and necrosis. Microscopically, the tumor cells had large vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. Histologically, the morphology of the tumor cells are similar for the primary vulvar specimen and the bone metastases. Immunohistochemically, the tumor cells are positive for vimentin, GATA, FLI-1, SMA, SMHC, partially positive for CAM5.2, AE1/AE3, CD31, and CD163. Immunohistochemistry was negative for CDX2, CD56, S-100, TTF-1, CK5/6, CK20, P40, mammoglobin, MOC31, ER, CK7, CK903, HMB45, PAX8. A Ki-67 proliferative index was around 30-40%. NGS molecular testing detected a SMARCB1 mutation with loss of exons 1-3 and exons 7-9 supporting the diagnosis of epithelioid sarcoma.

Results (if a Case Study enter NA)
NA

Conclusion
In summary, we report a case of PES of the vulva in a 60 year old female. Grossly, the lesion was nodular with histology showing large vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. It showed loss of INI1/SMARCB1 nuclear expression. The patient is receiving further adjuvant treatment and shows no new metastases.
High-Grade Sarcoma Arising in the Background of Growing Teratoma Syndrome; A Rare Case
Authors: Sadiq Q , Mohammed S , Yadak N , Khan F 
Published: October 2021

Abstract

Introduction/Objective
Growing teratoma syndrome is a rare condition seen in patients with a history of immature teratomas or mixed germ cell tumor status post-treatment. It usually occurs within the first two years of diagnosis but rarely can be seen years later.

Methods/Case Report
We report a case of a 48-year-old female with a previous history of ovarian malignancy in adolescence status post-TAH/BSO and chemotherapy, now presenting with a 23 cm multi cystic septate mass with some solid components in the subdiaphragmatic area, extending into the liver on imaging. Nodular implants along the surface of the right hepatic lobe and multiple peritoneal implants were seen. Histology of peritoneal implants showed fibro adipose tissue admixed with rare scattered glandular elements, epithelial fragments, and mature neuroglial tissue, consistent with mature teratoma. Liver biopsy revealed short fascicles of spindled, epithelioid and rhabdoid tumor cells admixed with foci of the myxoid stroma. Scattered moderate cytologic atypia, atypical mitosis, and necrosis were appreciated. Tumor cells showed strong and diffuse reactivity to vimentin while being negative for epithelial, neural/mesenchymal, mesothelial, sex cord-stromal, and germ cell differentiation markers. Differential diagnostic considerations included spindle cell transformation of a germ cell tumor or high-grade sarcoma, not otherwise specified arising in the background of growing teratoma syndrome. FISH testing for isochromosome 12p was negative excluding sarcomatoid germ cell tumor.

Results (if a Case Study enter NA)
NA

Conclusion
This case study highlights the importance of considering the rare complication of high-grade sarcoma arising in the background of growing teratoma syndrome.
HPV-Related Multi-Phenotypic Sinonasal Carcinoma with Aggressive Clinical Behavior; A Rare Case
Authors: Ha L , Sadiq Q , Singh J , Khan F 
Published: October 2021

Abstract

Introduction/Objective
Human Papilloma Virus (HPV)-related multiphenotypic sinonasal carcinoma, previously known as HPV-related sinonasal carcinoma with adenoid cystic carcinoma-like features, is a rare type of sinonasal carcinoma with both epithelial-derived and salivary gland-type morphologic features. It is associated with high-risk HPV, but lacks MYB gene rearrangements.

Methods/Case Report
We report a case of a 59-year-old male who presented with a rapidly growing sinonasal mass. On MRI, a left nasal cavity lesion was identified growing laterally along the frontal process of the maxilla, extending into the middle meatus and into the maxillary sinus. Patient underwent a complex left medial maxillectomy, spheno- ethmoidectomy, and sinusotomy. On gross evaluation, the left inferior turbinate and sidewall demonstrated a 4 cm unremarkable turbinate with attached friable soft tissue. Microscopic examination revealed sections of carcinoma with various architectural patterns comprised of foci with adenoid cystic carcinoma-like morphology, basaloid squamous cell carcinoma and adenocarcinoma. The tumor showed positive immunostaining for P40, but focal reactivity to S100 and rare scattered reactivity with CD117. INI-1 immunostain was retained in tumor cells. P16 immunostain was strong and diffuse and high-risk cocktail HPV RNA ISH was positive. However, MYB FISH testing was equivocal.
Morphologic and immunophenotypic findings were consistent with HPV-related multiphenotypic sinonasal carcinoma. The tumor involved the olfactory nerve fibers requiring a skull base resection and showed extension into the dura mater.

Results (if a Case Study enter NA)
NA

Conclusion
HPV related multiphenotypic sinonasal carcinoma is a recently described entity that can pose significant diagnostic challenge. It typically has an indolent clinical course with potential for late recurrences. This case study highlights the potential aggressive nature of this type of sinonasal carcinoma, despite association with high-risk HPV, and use of ancillary testing in aiding diagnosis.