bluuebunny/crossref_metadata_2025 huggingface dataset:
Small Bowel Obstruction as a Rare Complication of Progressive Sclerosing Mesenteritis
Authors: Karimi S S , Hassan M , Mehta V
Published: October 2021

Introduction/Objective

Sclerosing Mesenteritis is an uncommon, idiopathic, localized inflammatory syndrome involving the small intestine and colonic mesentery. It is considered a benign condition that commonly occurs in elderly with a gender predilection for males, and its etiology remains unknown. Small Bowel Obstruction (SBO) is a rare, unexpected, but detrimental complication of progressive Sclerosing Mesenteritis. Herein, we present a case of an enlarging, progressive Sclerosing Mesenteritis with extensive involvement of the small bowel and mesentery requiring two consecutive major surgical interventions.

Methods/Case Report

A 72-year-old male with Myelodysplastic Syndrome (MDS) and recent history of loop ileostomy due to distal intestinal obstruction secondary to enlarging Sclerosing Mesenteritis, presented to our institution with non-specific symptoms of worsening abdominal pain and multiple episodes of gastrointestinal bleeding. Radiographic investigation revealed SBO and he subsequently underwent exploratory laparotomy resulting in total enterectomy with excision of mesenteric mass, extended right colectomy, Whipple procedure, and gastrostomy. The specimens were sent to pathology for histopathological evaluation and gross examination revealed several bosselated, tan-white, firm and rubbery, fibrotic lesions with associated lobulated fibroadipose tissue. Microscopic examination showed extensive mesenteric fibrosis with dense bundles of collagen fibers, areas of fat necrosis, mucosal ischemia and calcification involving the small bowel and serosal surface of large intestine and peritoneum. The lesional cells showed minimal atypia, mitoses, and lacked the Beta-catenin nuclear staining seen in mesenteric fibromatosis. Given the clinical history and histopathological findings of the lesion, we favored the diagnosis of Sclerosing Mesenteritis.

Results (if a Case Study enter NA)

N/A

Conclusion

The etiology of Sclerosing Mesenteritis is not well-understood and there are cases of Sclerosing Mesenteritis reported in the literature in association with trauma, surgery, malignancy, and IgG4-related disease. Our patient’s post-operative history was complicated by short gut syndrome, and he is currently requiring small bowel transplant. We report this case for its unusual and aggressive clinical presentation, and to heighten clinical awareness for detrimental consequences of this seemingly benign condition.
A Rare Medullary Carcinoma of Jejunum
Authors: Fathima S , Bredeweg A
Published: October 2021

Introduction/Objective

Medullary carcinoma of jejunum is an extremely rare condition. These tumors account for less than 0.04% of all colorectal cancers and less than 3 cases to date has been reported in the small intestine

Methods/Case Report

We present a case of 78-year-old woman with a celiac disease and collagenous colitis, chronic diarrhea, chronic anemia and 2.1 cm apple core lesion on mid to distal jejunum on CT leading to partial obstruction.

Results (if a Case Study enter NA)

Histologically tumor showed invasive carcinoma in a solid growth pattern with pushing border. The tumor cells were uniform, enlarged with prominent nucleoli and brisk mitotic activity. There was prominent inflammatory response within and around the tumor. Immunohistochemical stains were positive for CK7, CDX2 CK19, CKAE1-3 and negative for CD45, CK20, Chromogranin Synaptophysin, PAX-8. MLH1 &PMS2 showed loss of nuclear expression and MSH2 & MSH6 with Intact nuclear expression. Microsatellite instability was High (MSI- H) with instability in two or more microsatellite markers. Diagnosis of medullary carcinoma of jejunum was made.

Conclusion

Although the clinical manifestations can be consistent with signs of intestinal obstruction, often these rare tumors are discovered incidentally. Conditions such as celiac disease, Crohn’s disease, and other chronic inflammatory illnesses have been linked to contributing risk factors. Imaging and appropriate tumor markers have less role in diagnosis; however, biopsy is needed for definitive diagnosis. Even though the development of these tumors in the small bowel is rare, further enhancement of awareness can aid in the appropriate early detection and appropriate treatment modalities.
A case of osseous metaplasia in a gastric hyperplastic polyp: An unexpected finding in a common polyp
Authors: Zhang Y , Nakanishi Y
Published: October 2021

Introduction/Objective

Foveolar hyperplastic polyp is a common gastric polyp characterized by foveolar hyperplasia with erosion, acute and chronic inflammation, granulation tissue formation, and smooth muscle strands extending from the muscularis mucosae. Although foveolar hyperplastic polyps may rarely contain foci of dysplasia or invasive carcinoma, osseous metaplasia/heterotopic bone formation in foveolar hyperplastic polyps of the stomach is extremely rare with a few case reports.

Methods/Case Report

A 63-year-old female with a history of hypertension, sick sinus syndrome, and Hashimoto’s thyroiditis was referred to our facility for evaluation of a mass in segment eight of the liver. The liver biopsy showed a moderately differentiated adenocarcinoma, most consistent with intrahepatic cholangiocarcinoma. A screening gastrointestinal endoscopy revealed a 7-mm sessile polyp in the antrum. The polyp was removed with a cold snare. No other abnormalities were identified in the stomach. Sections of the polyp showed fragments of antral-type gastric mucosa with foveolar hyperplasia, erosion, acute and chronic inflammation, and focal granulation tissue formation. In addition, multiple foci of woven bone formation without bone marrow surrounding dilated gastric foveolae were identified. No Helicobacter infection, intestinal metaplasia, dysplasia or malignancy was identified histologically. Osseous metaplasia/heterotopic bone formation is a well-known finding reported in various neoplastic and non- neoplastic conditions. However, osseous metaplasia in foveolar hyperplastic polyps of the stomach is extremely rare. There have been only four previous case reports published in English language. Our current case shows clinicopathologic features similar to those of the previous case reports including the findings of small-sized polyp found incidentally in middle-aged patients with no clinical history of hypercalcemia or any other abnormalities causing heterotopic bone formation.

Results (if a Case Study enter NA)

N/A

Conclusion

Although the pathogenesis of osseous metaplasia in a gastric hyperplastic polyp remains unknown, the finding of osseous metaplasia in a gastric hyperplastic polyp is very intriguing.
Use of Elastic Stain Technique to Detect Histologically Occult Vascular Invasion in Resected Colorectal Carcinomas at Danbury Hospital
Authors: Karki S , Steven S , Kaur G , Das D , Agarwal R
Published: October 2021

Introduction/Objective

Colorectal carcinoma (CRC) represents a leading cause of cancer related death in the western world and is by far the most common malignancy of the gastrointestinal tract. Vascular invasion (VI), in particular extramural VI by tumor is recognized as a feature of colorectal cancer progression, and the latter is an independent prognostic indicator of disease recurrence and survival in colorectal carcinoma. The purpose of this study is to detect VI in resected colorectal tumor samples and compare sensitivity of elastic staining to routine H&E staining at Danbury Hospital.

Methods/Case Report

A total of 50 colorectal cancer specimens from segmental colon resection or endoscopic polypectomy performed at Danbury Hospital over a 3-year period were included. Histologic sections of each tumor were assessed for the presence of VI by routine H&E staining. Superficial recuts of each tumor were subjected to elastic Von Giesen staining and reassessed for the presence of vascular invasion. Sensitivity of routine H&E staining for the detection of VI was calculated with elastic stain serving as the “gold standard”.

Results (if a Case Study enter NA)

In this study, VI by carcinoma was identified by H&E stain and/or elastic stain in 17 (34%) out of 50 cases. H&E stain and elastic stain detected VI in 12 (24%) and 16 (32%) out of 50 cases, respectively. There was a single case for which H&E stain showed vascular invasion that was not confirmed by elastic stain, representing a false negative for the H&E technique. Elastic staining detected VI in 5 cases for which the corresponding H&E stain was falsely negative for VI. Overall, vascular invasion status (absent or present) was concordant in 44 (88 %) of the 50 cases. With elastic stain deemed the “gold standard” for VI detection, the sensitivity, specificity, negative predictive value, and positive predictive value of H&E stain were found to be 68.7%, 97.0%, 86.8%, and 91.6%, respectively.

Conclusion

Although H & E staining of colorectal carcinoma tissue sections have a very good specificity and positive predictive value for the detection of VI, this technique suffers from a suboptimal sensitivity. For these reasons, we agree with previously published literature reports that the use of elastic stain as a supplement to H & E staining should be strongly considered for the proper evaluation and prognostication of colorectal carcinoma specimens.
Expression of Estrogen and Progesterone Receptors in a Tailgut Cyst with Carcinoid Tumor
Authors: Mohamed N , Rampisela D , Lopez L M
Published: October 2021

Introduction/Objective

Tailgut cysts (TC) are congenital lesions that arise in the presacral space. They originate from the embryonic hindgut and usually present between the ages of 30-60, with female predominance. TCs are usually asymptomatic or can present with lower back, perianal or pelvic pain. Malignant transformation of a TC is a rare complication, with adenocarcinoma being the most common, followed by carcinoid tumor. About thirty cases of carcinoid tumors arising in a TC have been reported in the literature so far with a slight female predominance (1.5:1).

Methods/Case Report

We describe a 40-year-old Hispanic woman with a 9-year history of cyclic pelvic pain related to her menstrual cycles. She was diagnosed with adenomyosis and underwent hysterectomy that did not completely resolve her symptoms. Follow-up serial imaging showed a growing complex-cystic presacral mass, which was eventually excised. Grossly, there was a 3.6×3.1×2.5 cm multiloculated cystic mass filled with mucoid material. Microscopically, there were multiple cystic spaces lined by benign squamous and mucinous columnar epithelium and surrounded by smooth muscle cells. A 6-mm carcinoid tumor was found within a cyst wall and confirmed by positive synaptophysin and chromogranin staining with a Ki-67 proliferation index of <2%. Estrogen and progesterone receptor immunostains were positive in the epithelial cyst linings and the stromal cells but not in the carcinoid tumor cells.

Results (if a Case Study enter NA)

NA

Conclusion

We conclude that the diagnosis of TC should be considered in the differential of gynecologic patients with unresolved cyclic pelvic pain and that estrogen and progesterone could have an important role in the pathogenesis of TCs. Furthermore, estrogen receptors can be a therapeutic target in patients with this entity.
Adenoid basal carcinoma with adenoid cystic-like features—a rare diagnostic dilemma
Authors: Salahi N , Jabbar A , Lavitan D
Published: October 2021

Introduction/Objective

Adenoid basal carcinoma (ABC) of the cervix is a rare, indolent tumor with no known metastatic potential. The tumor is composed of small nests of bland, basaloid cells, which do not elicit a desmoplastic response within the surrounding stroma. Cystic and clear cell change, as well as glandular differentiation, are not unusual. As such, it is important to differentiate ABC from adenoid cystic carcinoma (ACC), particularly on account of the aggressive clinical course of the latter. We herein present a rare case of ABC with adenoid cystic-like features.

Methods/Case Report

A 74-year-old woman with an incidental ABC, diagnosed on an excisional procedure performed for a prior diagnosis of a high grade squamous intraepithelial lesion. Subsequent hysterectomy revealed a tumor composed of nests of basaloid cells, extending into the outer third of the cervical wall, but exhibiting no peri- tumoral desmoplasia. Cribriforming associated with increased mitotic activity was focally present and showed increased Ki-67 labeling compared to the solid nests of tumor. PAS special stain highlighted rare pseudolumina with basement membrane-like material. While these features were concerning for an ACC—the lack of any significant cytologic atypia, destructive stromal invasion, and a clinically apparent lesion—was consistent with an ABC with so- called adenoid cystic-like features.

Results (if a Case Study enter NA)

NA

Conclusion

Making an accurate diagnosis requires clinicopathologic correlation and awareness of histologic overlap. Additionally, adenoid cystic-like morphology in ABCs is a little-studied phenomenon, which necessitates further research to shed light on potential clinical implications.
Heterotopic Sex Cord-Stromal Proliferation: A Case Report
Authors: Lagerstrom I , Uy M , Teschan N
Published: October 2021

Introduction/Objective

Sex cord-stromal cells at extraovarian sites are extremely rare and due to the low number of cases reported, the true incidence is unknown. We present the case of a patient with symptomatic uterine fibromas that had a benign extraovarian heterotopic sex cord-stromal proliferation.

Methods/Case Report

A 44-year-old female presented with pelvic pressure and discomfort. Past medical history is significant for breast cancer treated with bilateral mastectomy in 2019 followed by radiation treatments. The patient had no menses since January 2020 but had begun noticing spotting prior to the onset of her pelvic pressure and discomfort. Pelvic ultrasound revealed an enlarged uterus consistent with fibroids and a right adnexal mass. Endometrial biopsy following the ultrasound was normal. The patient desired definitive treatment and a total abdominal hysterectomy and bilateral salpingo-oophorectomy was subsequently performed. Gross examination of the surgical specimen revealed a fibroid uterus and normal appearing bilateral ovaries and fimbriated fallopian tubes. Histopathology showed a uterus with leiomyomata and serous cystadenofibromas in bilateral ovaries. The left fallopian tube fimbria showed a well-circumscribed proliferation of pale, ovoid cells in a microfollicular pattern. Rare cells with grooved nuclei were present. No significant atypia and no mitotic activity were identified. Immunohistochemical staining was positive for inhibin, WT-1, and vimentin. Negative stains include EMA, GATA-3, GCDFP 15, mammaglobin, chromogranin, and calretinin. Final diagnosis was determined to be benign fallopian tube with heterotopic sex cord-stromal proliferation.

Results (if a Case Study enter NA)

NA

Conclusion

This case highlights a rare, incidental finding. These lesions are postulated to represent non-neoplastic embryologic remnants. However, most heterotopic sex cord-stromal proliferations are found in fimbriae, which could suggest that they arise from heterotopic ovarian tissue exposed during ovulation. The incidence of these benign proliferations is expected to increase as routine sampling of adnexal structures is becoming more commonplace, which presents diagnostic challenges for pathologists.
Higher Prevalence of Multi-Drug Resistant Gonococcal Infections Among Patients with Prior Gonococcal and Chlamydia Infections
Authors: Mendoza R P , Gabutan E , Connelly C , Gupta R , Alejandro N
Published: October 2021

Introduction/Objective

Neisseria gonorrhoeae (NG) is the 2nd most common cause of sexually transmitted infection worldwide. WHO data from 2009-14 showed widespread resistance to ciprofloxacin (97%), azithromycin (81%), and oral cefixime and injectable ceftriaxone (66%). We have investigated local epidemiology of antimicrobial resistance in PCR-confirmed NG by chromosomal multi-locus sequence typing and analysis.

Methods/Case Report

Clinical specimens of 85 patients who tested positive for NG using automated PCR (COBAS 4800; Roche Diagnostics) were used in this study. The samples were amplified by PCR using 10 primers (Integrated DNA Technologies, USA) targeting seven antibiotic resistance-associated loci in the bacterial chromosome (penA, mtrR, ponA, porB, gyr, parC and 23s rRNA) and sequenced on a 48-channel ABI 3730 Sanger sequencer (Thermo Fisher). The resulting sequences were analyzed using the NG Sequence Typing for Antibiotic Resistance (NG-STAR) database for matching alleles, correlating to clinical resistance. Results were analyzed by Chi-square or Fisher’s exact tests.

Results (if a Case Study enter NA)

Most patients positive for NG were non-Hispanic Black males (60%), with mean age of 25 years (range: 14-53 years). 23.5% had Chlamydia trachomatis (CT) co-infection, and 29.4% had other sexually transmitted infections, such as syphilis, HIV and HPV. 15.0% had previous NG infection, and 12% had previous CT infection. Sixty-five samples (76.5%) yielded at least one gene locus with a DNA sequence with >95% similarity in the NG-STAR database. About half (50.6%) of these sample had confirmed resistance mutations, 5 of which indicated resistance to multiple antibiotics. All specimens with porB and gyr mutations were resistant to penicillins, ceftriaxone, cefixime, erythromycin, tetracycline and ciprofloxacin. Most of those with mtrR mutations were resistant to ciprofloxacin. Most of those with mutations (with or without resistance) had previous NG infection. Significant correlation was observed between those with at least 1 mutated gene locus and CT co-infection (p=0.0001).

Conclusion

A significant proportion of our patients tested positive for resistance mutations, a majority of whom had previous NG infection. There was significant correlation between the presence of mutations and CT co-infection. These findings warrant utilization of culture and sensitivity testing in patients with previous NG and CT co-infection.
Preferential HER2 Expression in Lymphovascular Invasion of a Uterine Carcinosarcoma, a Case Report
Authors: Gillam J
Published: October 2021

Introduction/Objective

Uterine carcinosarcoma (UCS) is a rare, highly aggressive cancer accounting for <5% of reported uterine cancer cases. Commonly, myometrial invasion and lymphovascular invasion (LVI) are found at diagnosis, and recurrence rates of >40% are seen after therapy accounting for poor prognosis and high rate (16%) of death. Histologically, it is composed of both carcinoma and sarcoma components. HER2-targeted therapy without adjunctive chemotherapy for the treatment of UCS has generally not shown beneficial clinical responses, but a more recent phase II clinical trial revealed that the addition of Trastuzumab, a HER2-targeted monoclonal antibody, to carboplatin-paclitaxel chemotherapy showed an increase in progression free survival.

Methods/Case Report

Immunohistochemical (IHC) staining for HER2 was performed on histologic sections of UCS, areas of LVI, and lymph node metastasis (LNM). Confirmatory HER2 fluorescent in situ hybridization (FISH) studies were performed on the primary UCS and the LVI portion. A 67-year-old female presented with postmenopausal uterine bleeding. CT showed an enlarged uterus, widened endometrial canal, and enlarged lymph nodes. Initial endometrial biopsy showed UCS. Exploratory laparotomy with total abdominal hysterectomy, bilateral salpingo-ophorectomy, omentectomy, and lymph node dissection was performed with pathology confirming UCS with 86% myometrial invasion and LVI.

Results (if a Case Study enter NA)

NA

Conclusion

HER2 expression in this patient was solely seen in foci of LVI and within LNM. This finding suggests inclusion of histologic sections with LVI and LNM when evaluating for the presence of HER2 expression in uterine carcinosarcoma with reflex FISH testing when indicated may be helpful to provide additional treatment opportunities for patients. Further studies need to be performed to determine if this finding in generalizable to carcinosarcoma and endometrial serous carcinoma samples.
Unexpected endometrial malakoplakia in a 74-year-old-female: A case report
Authors: Abdelwahed M , Chavarria Bernal H , Truskinovsky A
Published: October 2021

Introduction/Objective

Malakoplakia is a chronic inflammatory disease, usually associated with bacterial infection. The urinary system is the most commonly involved site. Involvement of the female genital tract is extremely rare. Histopathologically, the disease is manifested by solid sheets of histiocytes with abundant granular eosionophilic cytoplasm, and intra- and extracellular basophilic bodies. It should be differentiated from other types of granulomatous inflammation and from signet ring cell carcinoma. Treatment consists of antibiotics, but surgery is sometimes necessary.

Methods/Case Report

A 74-year-old woman experienced postmenopausal bleeding for several years. A previous endometrial biopsy showed a mucinous glandular proliferation of uncertain etiology. She also had coronary heart disease, hypertension, diabetes mellitus and hyperlipidemia. A prior urinalysis showed abundant bacteria, and she had been hospitalized recently for urinary tract infection (UTI). She underwent laparoscopic total hysterectomy. Grossly, the endometrium was mostly uniform, 0.1 cm in thickness, with one 0.4-cm polyp. Microscopically, the endometrium showed simple hyperplasia without atypia, benign endometrial polyp, and multifocal malakoplakia, manifested by sheets of histiocytes with granular eosinophilic cytoplasm, many of them containing variably-sized basophilic spherules imparting to them a targetoid, or bull’s eye, appearance. Multiple endometrial plasma cells were also seen.

Results (if a Case Study enter NA)

NA

Conclusion

Very few cases of endometrial malakoplakia have been reported in the medical literature. Here we present unexpected malakoplakia coexisting with endometrial simple hyperplasia without atypia. Reports from the electron microscopy literature suggest that the basophilic spherules, named Michaelis-Gutmann inclusion bodies, are electron-dense granules that result from coalescence of phagolysosomes, reflecting failed digestion of bacteria. In our patient, longstanding chronic endometritis, confirmed by the presence of endometrial plasma cells, possibly coexisting with chronic UTI, culminated in malakoplakia, which clinically and pathologically mimicked endometrial hyperplasia or even carcinoma, given her old age, chronic bleeding, and the mucinous epithelium in the endometrial biopsy. The finding of endometrial malakoplakia should remind us of the bacterial etiology of chronic endometritis and the need for timely treatment of urogenital infections with antibiotics.
Anti-NMDA-Receptor Encephalitis in a Patient with Ovarian Teratoma, Harboring Brain Histology of Varying Developmental Stages and Regions with Chronic Inflammation
Authors: Fatemi S , Qu E , Fullmer J
Published: October 2021

Introduction/Objective

Anti-NMDA-receptor encephalitis is a subacute, autoimmune disorder thought to be caused by autoantibodies directed against the N-methyl-D-aspartate (NMDA) receptor. Clinical symptoms of anti-NMDAR encephalitis may mimic schizophrenia and psychotic spectrum disorders or substance-induced psychosis. Although initially described in association with ovarian teratomas in women, anti-NMDAR encephalitis has been reported in individuals without paraneoplastic association, as well as in males. Herein, we report a case of a 29-year-old woman with suicidal ideation and other neuropsychiatric manifestations who was found to have a right ovarian cystic mass by imaging study. Microscopically, the resected ovarian mass is composed of mature skin, fat, cartilage and neural tissues. Nerve, ganglions and multiple brain tissues are present. Cerebellum including external granular cell layer (normally only seen in infants), cerebrum-like, choroid plexus and other neural elements are present. There is peripheral lymphoplasmacytic infiltrates around and within the neuroglial matrix. Cerebral spinal fluid was concurrently tested positive for Anti-NMDAR. The combined clinical, histological, and laboratory findings confirmed the above diagnosis. Although Anti-NMDAR encephalitis is a familiar entity to many clinical psychiatrist and neurologists, it is less commonly reported in the pathology literature. Its resultant relationship to cystic teratoma warrants awareness of this condition by pathologists.

Methods/Case Report

Case Report

Results (if a Case Study enter NA)

NA

Conclusion

Anti-NMDA-receptor encephalitis is related to cystic teratoma, therefore pathologists need to be aware of this condition.
Rhabdomyosarcoma in Ovary- Pathologist’s Diagnostic Dilemma
Authors: Srivastava A C , Hussain F , Patel T
Published: October 2021

Introduction/Objective

Rhabdomyosarcoma is the most common pediatric soft tissue sarcoma. However, rhabdomyosarcoma in the ovary is exceptionally unusual and presents a diagnostic challenge. We report one such case and its clinical and pathological perspective.

Methods/Case Report

16-year-old female presented with an enlarging pelvic mass, abdominal distension, and pain. Imaging revealed a heterogeneous left ovarian mass, right axillary lymphadenopathy, and right-hand soft tissue mass. CA-125 was 1833.0 U/ml. Patient underwent left salphingo-oophorectomy, omentectomy, peritoneal and mesenteric biopsy for ovarian staging, incidental appendectomy, and right axillary lymph node excision with suspicion of ovarian epithelial tumor. Pathological evaluation revealed a 16-cm intact ovary with multiple solid and cystic nodules with areas of hemorrhage and necrosis. Microscopic examination of ovarian tumor showed a high-grade malignant tumor consisting of sheets of small round blue cells with severe cytologic atypia, increased mitoses, and features suggestive of rhabdoid morphology. The other specimens were positive for metastases. The tumor cells were immunopositive for Myogenin and MyoD1 while immunonegative for Cytokeratin AE1/AE3, SF-1, PLAP, SALL4, MelanA, and S-100. INI-1 and BRG-1 demonstrated intact nuclear expression. FISH testing identified rearrangement of the FOX01 gene at 13q14. Based on these findings, diagnosis of alveolar rhabdomyosarcoma was rendered. Currently, the patient is receiving rhabdomyosarcoma chemotherapy treatment and has responded well.

Results (if a Case Study enter NA)

NA

Conclusion

In cases with complex and urgent clinical presentation, where the existence of a primary tumor is unknown and where symptoms attributable to primary ovarian tumor dominate the clinical picture, rhabdomyosarcoma is rarely proposed in the differential diagnoses of small round blue cell tumors of the ovary. At present, the right-hand mass is under evaluation; thus, the true nature of the ovarian mass, primary or metastatic is unknown. Our case illustrates the importance of exact diagnoses, as treatment of rhabdomyosarcoma, is different from other ovarian tumors.
Invasive Fungal Sinusitis: Clinical Pathologic Characteristics of Acute vs. Chronic Infections
Authors: Kitagawa Y , Mesa H , Lavik J
Published: October 2021

Introduction/Objective

Invasive fungal sinusitis is a serious condition requiring early diagnosis and treatment. It has been classified into acute, chronic and granulomatous forms. This study aims to investigate clinical pathologic aspects such as a) Frequency of mass forming lesions, b) Frequency of granulomatous reaction, c) Frequency of acute inflammatory reaction and d) Frequency of angioinvasion, perineural invasion and necrosis, to determine if these factors allow a more meaningful clinical-pathologic classification.

Methods/Case Report

Cases of invasive fungal sinusitis with surgical pathology specimens available in our laboratory since January 1, 2006 to date were gathered. Electronic medical records, histopathologic diagnostic material and laboratory fungal identification results were reviewed.

Results (if a Case Study enter NA)

Thirty-one cases of invasive fungal sinusitis were found: Twenty-two were acute (< 4wk duration) and 9 chronic. Patient comorbidities in acute cases were malignancies: 45%, diabetes mellitus: 26% and solid organ transplant: 10%.

Among patients with malignancies, 5 cases had relapsed/refractory acute myeloid leukemia with neutropenia < 1000/uL. Patients with diabetes mellitus exhibited an average HbA1c of 10.0%. Two out of 3 transplant patients had graft versus host disease.

The most common causative fungi were species of Aspergillus, Candida and mucormycetes. By contrast, a third of the chronic cases had a history of recreational drug use and six presented with space occupying lesions seen on imaging studies. Upon histologic review, four of these showed granulomas and the majority of cases exhibited extensive necrosis. Among necrotic cases, perineural and vascular invasion by fungal organisms was identified.

Conclusion

We report the contrasting clinical pathologic characteristics of acute and chronic invasive fungal sinusitis in a series of cases treated at University affiliated tertiary/quaternary-care Hospitals. Acute invasive sinusitis is usually a complication of severe systemic diseases. Chronic cases are caused by various medical conditions including the use of recreational drug and may mimic neoplasms on imaging.
Mass-forming chronic invasive fungal sinusitis; an uncommon mimicker of head & neck malignancy
Authors: Muldoon J , Hou T
Published: October 2021

Introduction/Objective

Invasive fungal sinusitis is a progressive infection affecting the nasal cavity and associated sinus spaces. Acute forms affect immunocompromised patients with debilitating systemic diseases while chronic forms usually occur in immunocompetent individuals.

Methods/Case Report

A 26-year-old immunocompetent male with daily Marijuana smoking consulted for acute swelling of the right eye with dull pain, photophobia, loss of vision and pressure of 4 day duration. He reported nasal congestion for approximately one year and over the previous 2 months developed exophthalmos. Imaging demonstrated a 4.9 x 4.3 x 4.1 cm invasive soft tissue mass in the right lamina papyracea with involvement of the paranasal sinus, right orbit, and intracranial space. The mass was presumed to represent a high-grade malignancy.

Biopsy was performed and demonstrated granulomatous invasive fungal sinusitis. The patient underwent right orbitotomy and right extranasal ethmoidectomy. He was begun on empiric amphotericin B. After culture and sequencing identification of Curvularia species, voriconazole was added. Follow up imaging studies after two month of treatment still demonstrated residual mass forming lesion with 10-20% decrease in size. The patient has regained his vision after 4 month of treatment and he is expected to complete a six months course. If the mass forming lesion persists, a second round of surgical debridement will be performed.

Results (if a Case Study enter NA)

N/A

Conclusion

Curvularia is a dematiaceous fungus most commonly associated with allergic sinusitis, but can rarely become invasive in immunocompetent individuals. Histopathologically large fungal elements are easily recognizable but specific identification requires culture or ribosomal DNA sequencing by PCR. Mass-forming chronic fungal sinusitis overlaps clinically and radiologically with head and neck malignancies. Our report brings attention to this uncommon clinical variant of invasive fungal sinusitis that affects immunocompetent individuals.
Pediatric Epithelioid Angiosarcoma of the Maxillary Sinus: Clinicopathologic, Immunophenotypic and Molecular Study of a Rare Entity
Authors: Rastegar S , Suster D
Published: October 2021

Introduction/Objective

Angiosarcomas are aggressive neoplasms of the vascular endothelium and comprise approximately 2% of all soft tissue sarcomas. The occurrence in the sinonasal cavity is rare. A case of epithelioid angiosarcoma of the maxillary sinus in a 19-year-old patient is presented and its clinical presentation, histologic, immunohistochemical, and molecular features are discussed.

Methods/Case Report

A 19-year-old male evaluated at Rutgers University Hospital for 3 months of left-sided epistaxis. He reports left side nasal obstruction, facial pain, and epiphoria. Computed tomography revealed a heterogeneous soft tissue mass within the left maxillary sinus; with dehiscent segments extending into the premaxillary space, infratemporal fossa, and the left orbital floor. Bilateral rigid nasal endoscopy showed bowing of the left maxillary sinus medial wall, exposing a mucosalized mass. He underwent left maxillary antrostomy and left subtotal maxillectomy. Positron emission tomography scan showed no metastasis.

The tumor was within the submucosa of the respiratory epithelium and focally demonstrated anastomosing vascular spaces lined by atypical endothelial cells. The neoplastic cells showed epithelioid morphology with increased nuclear size, prominent nucleoli, and atypical mitotic figures up to 22 mitoses per 10 high power fields. The tumor cells were strongly and diffusely positive for CD31, CD34, FLI1, ERG, vimentin, and cytokeratin AE1/AE3. The tumor was negative for HMB45, desmin, HHV8, CAMTA1, FOSB, and S-100 and retained the expression of INI-1. The proliferation index was 60-70%.

A next generation sequencing disclosed stable tumors microsatellite status and a mutational burden of 1 mutation/Mb. Numerous variants of uncertain significance were identified including amplification of BRIP1, GNA13, and PRKAR1A as well as various point mutations including FLT4 G1296S and G71R, MET R359Q, ROS1 R442Q, DOT1L R757W, PDCD11 R1610L, TLL2 R849C, FANCD2 D400E, LEF1 D177V, PRDM1 A408P, FLT1 F989L, and MAP3K1 S939C.

Results (if a Case Study enter NA)

NA

Conclusion

Sinonasal angiosarcoma is incredibly rare with a poor overall prognosis. Few lesions have been reported in the pediatric population. Angiosarcoma may be confused with other types of malignant neoplasms when epithelioid morphology is present. The use of multiple vascular and epithelial markers with clinical correlation will be helpful in the diagnosis.
Myoepithelial Carcinoma ex Pleomorphic Adenoma: Rare Case Report with Clinicopathologic and Immunohistochemical Features
Authors: Khazaeli M , Kamaluddin Z , Taherian M
Published: October 2021

Introduction/Objective

Myoepithelial carcinoma is identified by nearly exclusive myoepithelial differentiation and evidence of malignancy. It may arise de novo or in preexisting benign tumors including pleomorphic adenoma and benign myoepithelioma. It occurs most commonly in the parotid gland followed by submandibular glands, minor salivary glands, and occasionally in the sublingual gland. Nasopharyngeal origin has been rarely reported.

Methods/Case Report

An afebrile 19-year-old female presented to the emergency department with persistent facial pressure, otalgia (right side greater than left side), rhinorrhea, and several episodes of epistaxis. One month before, she has been treated with Amoxicillin for three days with no relief, where Augmentin started for her for five days. About four days prior presentation, she was placed on Prednisone and Cefdinir. Over the next several days, she began having throat pain with difficulty swallowing. Routine blood work revealed a leukocytosis of 14.2 with normal differentiation. CT of the neck showed a large soft tissue mass centered at the right nasopharynx. Flexible nasal endoscopy performed bilaterally to reveal the nasopharynx is entirely obstructed with a lobulated mass filling the nasopharynx. On the right side, it extends into the posterior nasal passage filling the sphenoethmoid recess and the posterior floor of the nasal passage. Outpatient biopsy from nasopharynx mass is also performed.

Results (if a Case Study enter NA)

Mass biopsy reveals a mucinous and chondromyxoid background with mixed epithelial and myoepithelial differentiation. The is squamous metaplasia of myoepithelial cells and prominent mitotic activity and apoptotic activity. Immunohistochemistry was positive for CK5/6, calponin, BCL2, SMA, BerEp4, Sox10, and a proliferative index up to 40%. Based on this information Myoepithelial Carcinoma ex-pleomorphic adenoma of the nasopharynx is the diagnosis.

Conclusion

Carcinoma ex pleomorphic adenoma is usually a high-grade malignancy. It occurs most commonly in the parotid gland, followed by submandibular glands, minor salivary glands, and occasionally in the sublingual gland. Our case is one of the few cases of myoepithelial carcinoma arising in nasopharyngeal pleomorphic adenoma.
Lipid droplets are dysregulated in the adult dentate gyrus during seizure
Authors: Ahamad A , Ge S
Published: October 2021

Introduction/Objective

Dentate gyrus (DG), a neurogenic niche, is a metabolically dense subregion of the hippocampus. Continuous production and integration of new neurons in the existing circuit and harmonious relationship between excitatory and inhibitory neurons accompanied by neuron-glia coupling is essential to maintain hippocampal homeostasis throughout adulthood. Imbalance in the neuronal activity generates seizures and can result in mesial temporal lobe epilepsy (MTLE). MTLE affects 50 million people across the globe and impairs the overall hippocampal network and its associated functions such as memory and cognition. Although altered lipid metabolism has been associated with status epilepticus, the role of lipid droplets (LDs), the minuscule metabolically active organelle known to provide a substrate for cellular energy, has not been explored in DG during seizure. LDs are composed of neutral lipids and surrounded by phospholipid monolayer, which is studded with a structural Perilipin family of proteins 1-5, reported to be involved in lipid metabolism.

Methods/Case Report

To study LDs in the brain, we used a novel approach by injecting Bodipy, a lipid dye in the tail vein of mice, and successfully labeled LDs in the DG. We used the pilocarpine-induced seizure model. After Bodipy injection followed by seizure induction, mice were sacrificed at four time-points 0.5, 1-, 3- and 18 hours.

Results (if a Case Study enter NA)

We found a significant increase in Bodipy signal and Perilipin 4, LDs specific marker expression at four time-points post-seizure than in the control cohort. To elucidate the role of neuron-glia metabolic coupling in DG, we measured LDs in microglia and astrocytes and found a significant increase in LDs in seizure mice than control groups suggesting the role of glia in lipid regulation in DG.

Conclusion

Overall, this novel study will highlight the undiscovered role of LDs in dentate gyrus during seizure and, in the future, can be used as a therapeutic target to alleviate the MTLE phenotype.
Adenomatoid odontogenic tumor: a report of two unusually large lesions in the mandible
Authors: Lazim A , Kuklani R , Sundararajan D
Published: October 2021

Introduction/Objective

Adenomatoid odontogenic tumor (AOT) is an uncommon benign odontogenic tumor representing 3 to 7% of all odontogenic tumors. This tumor was first reported as adeno-ameloblastoma by Bernier and Tiecke in 1950 as it was initially assumed to be a type of ameloblastoma. In 1969, Philipsen and Bern proposed the term adenomatoid odontogenic tumor which was subsequently adopted by WHO and became the accepted terminology for this tumor. AOT is classified as a tumor of odontogenic epithelium but occasionally abnormal hard tissues consistent with dentinoid material may also be present as part of the tumor. AOT tends to occur in younger patients and 50% of the cases are diagnosed in teenagers. It occurs twice as commonly in females and frequently involves the anterior maxilla. Radiographically, AOT can appear as a radiolucent or mixed lucent-opaque lesion and may be associated with an impacted tooth.

Methods/Case Report

We report two unusually large expansile lesions of AOT that presented in the mandible. In the first case, the tumor presented as an expansile radiolucent lesion involving the right posterior mandible in a 32 year old female. In the second case, the tumor presented as an expansile mixed lucent-opaque lesion involving the left anterior mandible in a 21 year old female. The clinical presentation, radiographic and imaging findings, histopathologic features and treatment of these two cases will be discussed. The recommended treatment for AOT is surgical excision. The prognosis is good as this tumor seldom recurs after excision.

Results (if a Case Study enter NA)

NA

Conclusion

AOT is considered to be a non-aggressive, non-invasive and slow growing benign neoplasm. It is usually discovered on routine radiographic examination as the lesion is usually small and asymptomatic at the time of diagnosis but occasional cases that are larger in size have been reported in the literature.
Challenge in Early Diagnosis of Rare Lymphomas – A Case of Monomorphic Epitheliotropic Intestinal T-cell Lymphoma Masquerading as Enteritis
Authors: Qiu W , Khokhar H , Dewenter T A , Jetly R
Published: October 2021

Introduction/Objective

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is an extranodal lymphoma arising from intestinal intraepithelial T-lymphocytes. Diagnosis is challenging obscured by non-specific gastrointestinal (GI) symptoms and radiographic findings, with potential delay in implementing therapy, as was encountered in a 40- year-old man of African-American descent with cachexia.

Methods/Case Report

The patient presented with a two month history of nausea, vomiting, diarrhea and 10-15 pounds weight loss. Computed tomography revealed wall thickening of multiple large- and small-bowel loops. The clinical differential diagnoses included infectious enteritis and inflammatory bowel disease. Since symptoms improved during the hospital stay, he was discharged with future outpatient endoscopy planned but returned a month later with abdominal swelling and pain. On repeat imaging, he had ascites and small-bowel mechanical obstruction versus ileus. Straw-colored ascites, dilated friable segments of small-bowel, matted mesenteric lymph nodes, and adhesions were found on exploratory laparotomy. Small-bowel segments and appendix were resected. A prolonged hospital course ensued post-operatively with death due to multiple complications.

Results (if a Case Study enter NA)

The intestinal wall was grossly thickened with no discrete masses and microscopically had extensive infiltrates of small- to intermediate-sized, mature, minimally pleomorphic, CD3+/CD4- /CD5-/CD7+/CD8+/CD10-/CD20-/CD30-/CD56+/EBER-ISH- T-lymphocytes, focally infiltrating into the epithelium. Ascitic fluid cytology had small lymphocytes, CD2+/CD3+/CD4-/CD5-/CD10-/CD7+/CD8+/CD25-/CD30-/CD56+ /TCRαβ+/TCRγδ- by flow cytometry immunophenotyping. A diagnosis of MEITL was rendered based on these findings.

Conclusion

MEITL is rare, aggressive, unassociated with celiac disease and usually seen in Asian and Hispanic populations. It presents with non-specific GI symptoms of abdominal pain, perforation, diarrhea, weight loss and intestinal obstruction underscoring the need for a high index of suspicion. Upper GI endoscopy has a limited role since involvement of small-bowel, specifically jejunum, is common. The evolving capsule endoscopy techniques may help in an early diagnosis. Due to limited literature, optimal treatment is unclear and various strategies using chemotherapy, surgery and stem-cell transplant have been tried with variable outcomes.
Immunohistochemical Characterization of H3K4Me3 in Reactive Lymph Nodes and Follicular Lymphoma
Authors: Xu G , Dresser K , Bledsoe J
Published: October 2021

Introduction/Objective

Genes involved in histone methylation are frequently mutated in non-Hodgkin’s lymphomas. For instance, frequent mutations in genes encoding histone methytransferases MML2 and EZH2 are present in diffuse large B cell lymphoma (DLBCL) and follicular lymphoma (FL). The aim of this study was to characterize the immunohistochemical expression of H3K4Me3 in benign/reactive lymph nodes (LNs) with comparison to follicular lymphoma (FL).

Methods/Case Report

Immunohistochemical staining with an anti-H3K4Me3 antibody was performed on FFPE whole slide section from patients with benign/reactive LNs (n=21), low grade (grade 1-2) FL (n=21). H3K4Me3 reactivity was scored for staining intensity and percentage of lymphocytes showing reactivity.

Results (if a Case Study enter NA)

The majority of the reactive LN sections (15 out of 21 cases) showed a distinct distribution of H3K4Me3 staining, with the majority of cells in the mantle zone and the interfollicular zones showing moderate-strong staining, whereas reactive germinal centers (GCs) showed significantly decreased or close to negative staining. Neoplastic follicles in all the FL cases contained positive cells with significantly stronger staining compared to that in the germinal centers in benign lymph nodes. The interfollicular zones, while diminished in FL due to expanded neoplastic follicles, showed retained H3K4Me3 staining. The difference in staining intensity between follicles and mantle/interfollicular zones became indistinct in FL.

Conclusion

H3K4Me3 expression in benign/reactive LNs is characterized by positive expression in lymphocytes in interfollicular and mantle zones and significantly decreased in GCs. However, the expression pattern is different in FL, which showed significantly increased expression in the follicles compared to that in reactive GCs. In FL, the expression in GCs is similar to that in the interfollicular and mantle zone lymphocytes. It is reported that about 90% of FL have MLL2 mutation and MLL2 is the enzyme responsible for the methylation of H3K4. So far, it is unclear whether the mutation of MLL2 in FL affects the overall methylation activity of the enzyme. In our study, there is increased methylation of H3K4 in the follicles of FL, which raises the possibility that MLL2 mutation somehow increases H3K4 methylation.