bluuebunny/crossref_metadata_2025_split huggingface dataset:
Primary Meningioma of the Middle Ear - A Rare Presentation
Authors: Zaheer S , Kleinman G M 
Published: October 2020

Abstract

Introduction/Objective
Meningiomas are the benign intracranial neoplasm, most commonly occurring in the third and sixth decade of life. The most common intracranial locations for meningiomas are parasagittal sinuses and large dural sinuses. Very rarely extracranial meningiomas can also be observed. We present a case of fibrous meningioma presenting as middle ear mass, in which a 13 years old female was presented with drainage from the left ear. Biopsy showed fibromyxomatous tissue. CT scan of the head showed soft tissue filling the middle ear and extending into the mastoid.

Methods
The mass was resected and sent to pathology, where H&E staining and immunohistochemistry was done for diagnosis.

Results
The H&E stained section showed a spindle-shaped neoplasm with areas of sheet-like growth interspersed with areas showing tumor cell growth in short fascicles, and some areas of decreased cellularity. Tumor cells have moderate cytoplasm, fine nuclear chromatin and inconspicuous nucleoli. The tumor cells were positive for EMA, PR(nuclear staining) and pan-TRK and negative for D-240, SOX-10, S-100 and desmin. These histological features alone with immunohistochemistry profile were consistent with meningioma WHO grade 1.

Conclusion
Primary middle ear meningioma although a rare diagnosis, but should be considered while dealing with middle ear pathologies. Histomorphology and immunohistochemistry play an important role in making diagnosis.
Metastatic Alveolar Soft Part Sarcoma to the Breast: A Case Report
Authors: Cheriyan A , Bousamra A 
Published: October 2020

Abstract

Introduction/Objective
Metastases to the breast are rare (less than 3% of all breast malignancies). In adults, malignant melanoma is the most common type of metastasis followed by carcinomas from various primary sites. Here, we describe a case of metastatic alveolar soft part sarcoma (ASPS) to the breast. Only a handful similar presentations are reported in the literature.

Methods
Here we describe the case of a 43-year-old female patient with a recent diagnosis of left base of tongue alveolar soft part sarcoma (in an outside hospital), presenting with a palpable mass in her left breast. Sonographic study of the left breast demonstrated a 1.4 cm oval hypoechoic mass with partially microlobulated margins at 1 o’clock.

Results
Histopathologic examination of the ultrasound guided core biopsy showed a “pink” neoplasm, composed of plump epithelioid tumor cells with an eosinophilic and granular cytoplasm, round to oval nuclei and conspicuous nucleoli. They are arranged in organoid nests separated by sinusoidal spaces. An immunohistochemical study shows the tumor cells to be negative for keratin AE1/AE3, keratin 7, keratin 5/6, GATA-3, Pax-8, estrogen receptor, HER2, p63, S100 and CD117. FISH test for TFE3 (Xp 11.23) is positive. PAS with diastase staining highlights the intracytoplasmic granules. Theses morphologic, clinical and molecular findings confirm the diagnosis of metastatic ASPS.

Conclusion
The differential diagnosis of “pink” tumors of the breast include primary tumors like secretory carcinoma and apocrine carcinoma and metastatic lesions of renal, thyroid, skin (melanoma), oral cavity and soft tissue. Although extremely rare, in the right clinical context, metastatic ASPS to the breast can occur and should be included in the differential diagnosis.
Ovarian Carcinosarcoma with Malignant Neuroectodermal Components (Teratoid Carcinosarcoma) - A Rare Entity
Authors: Cheriyan A , Liu Y , Patel S , Liang S , Munns S , Krivak T 
Published: October 2020

Abstract

Introduction/Objective
Ovarian carcinosarcoma is an aggressive tumor accounting for 2% of all ovarian malignancies. Ovarian carcinosarcoma with malignant neuroectodermal components is exceedingly rare and only four cases have been previously reported. Here we report a series of three cases in 65(case 1), 61 (case 2) and 72 (case 3) year old females.

Methods
All patients presented with pelvic mass and elevated CA125. They underwent hysterectomy, bilateral salpingo-oophorectomy, omentectomy and tumor-debulking, along with lymph node dissection (2,3) and appendectomy (3). The FIGO stages were IIIC (1,3) or IIA (2). Case #2 experienced pelvic recurrence three months after initiating platinum based chemotherapy and was treated with BEP. She died of metastatic disease ten months after initial surgery. Case #3 is a current case and is being treated with chemotherapy. Case #1 was an outside consult and was lost to follow–up.

Results
Grossly the dominant ovarian mass measured 25 (left), 20 (left) and 21 (right) cm. The cut surfaces were hetergenous tan, yellow, partially cystic and solid with hemorrhage and necrosis. Microscopically, they showed mixed morphology of carcinomatous, sarcomatous, and undifferentiated neuroectodermal component. The carcinomatous element showed squamous cell carcinoma and adenocarcinoma. The sarcomatous component included rhabdomyosarcoma, chondrosarcoma and liposarcoma. The undifferentiated neuroectodermal component consisted of nested or solid primitive cells. All three cases showed fetal-appearing teratoid squamous cell epithelium with clear cytoplasm. The contralateral ovary and extraovarian metastatic implants were either carcinosarcoma, carcinoma or contained all three components.

Conclusion
Case #1 and 3 also showed a yolk sac component. All lymph nodes (2,3) were negative for metastatic tumor at the time of initial surgery. Case #2 had first recurrence in retroperitoneal lymph node showing mature neural tissue. Her second recurrence in abdomen was undifferentiated neuroblastoma. Immunohistochemical studies included CK7, CK20, CDX2, NapsinA, S100, GFAP, PAX-8, CK5/6, P63, AE1/AE3, desmin, Myf-4, CD56, glypican3, AFB, CD99, chromogranin, synaptophysin, NF, NeuN, calretinin, and inhibin. The staining pattern was consistent with histologic interpretation. These cases histologically resemble to so-called “teratoid carcinosarcoma” of paranasal sinuses. The histogenesis remains unknown.
Primary Pleural Angiosarcoma With Extensive And Rapid Metastasis To Brain And Bilateral Adrenal Glands
Authors: Ullah A , Mattox S N , Heneidi S , White J , Sinkler M , Wehrle C 
Published: October 2020

Abstract
Casestudy: Angiosarcomas represent 1% of all soft tissue malignancies. Primary pleural angiosarcomas are aggressive and extremely rare. The etiology of pleural angiosarcoma is largely unknown but in the few reported cases it is associated with asbestos exposure. Common presenting symptoms are chest pain, cough, dyspnea, and weight loss. Localized tumors are generally resected, with other options including chemotherapy and radiotherapy. Limited literature describes this rare neoplasm’s metastatic potential and patterns.
We present a 64-year-old male with shoulder pain and chronic cough. CT thorax showed a large 6.7 x 8 cm mass with central necrosis in the left upper lung invading the chest wall with partial destruction of the second and third ribs, and axillary adenopathy. Bilateral adrenal nodules were also identified. Histology of the adrenal lesion reveal sheets of infiltrative polygonal/round cells with large nuclei, prominent nucleoli and eosinophilic cytoplasm. Tumor cells were positive for CD31, CD34, FLI-1, AE1/AE3 and CK7 immunohistochemical stain, correlating with primary epithelioid angiosarcoma. Based on the patients new development of confusion, dizziness and ataxia, an MRI was performed showing multiple metastatic brain lesions. One month after initial presentation, the patient again presented with worsening symptoms. Work up showed enlargement of the bilateral adrenal masses, doubling of the brain lesions and an additional lesion posterior to the left kidney.
This case shows the metastatic potential and pattern of an aggressive primary neoplasm that is not described in current literature. It also highlights the importance of timely intervention based on the rapid metastatic progression of primary pleural angiosarcomas.
Patterns of Cytokeratin 7 Expression in Cervical Squamous Intraepithelial Lesions
Authors: Youssef M , Canete-Portillo S , Yemelyanova A , Ronnett B 
Published: October 2020

Abstract

Introduction/Objective
It has been suggested that cervical high-grade squamous intraepithelial lesions (HSIL/CIN2-3) arise from squamocolumnar junction cells that express cytokeratin 7 (CK7). Significant CK7 expression (gradation or full-thickness) has been proposed as a marker of progression of low-grade squamous intraepithelial lesions (LSIL/CIN1) to HSIL and of persistence of HSIL/CIN2. The goal of the study is to survey patterns of CK 7 expression in the different grades of squamous intraepithelial lesions (SILs).

Methods
65 cervical specimens (biopsies and excisions) containing 95 lesions of different grades were assessed by immunohistochemical analysis (IHC) for CK7 expression. 26 cases contained more one lesion grade. The diagnosis of HSIL (CIN2-3) was confirmed by p16 IHC. CK7 expression was scored negative, patchy, gradation (i.e., top-down), or full-thickness pattern. In cases with heterogeneous staining, the strongest pattern was used for analysis.

Results
There was significant variation in patterns within morphologically contiguous lesional foci; staining heterogeneity was noted in 42% of cases. All patterns of expression were encountered in all lesion grades. LSIL/CIN1 (n=47), either alone (n=27) or in combination with HSIL (n=20), often lacked CK7 expression (53%) or were patchy (17%). The frequency of significant (gradation or full-thickness) CK7 expression in LSIL with concomitant HSIL was greater than LSIL occurring alone (40% vs. 22%, respectively). HSIL/CIN3 (n=19) was dominated by full-thickness expression (57%). HSIL/CIN2 (n=29) had a very heterogeneous spectrum of expression with 34% of cases lacking expression.

Conclusion
CK7 expression is variable across all grades of SILs. LSIL with concomitant HSIL was associated with significant CK7 expression more frequently than LSIL alone. Significant proportion of HSIL, particularly CIN2, lacks CK7 expression. Given this variability, caution is advised regarding the use of CK7 expression as a marker of progression.
Pediatric Acute Hemorrhagic Leukoencephalitis (Weston-Hurst syndrome): A Rare Entity
Authors: Tomac Pavosevic H , Zaheer S , Kleinman G M 
Published: October 2020

Abstract

Introduction/Objective
Acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome) is a rare, fulminant inflammatory hemorrhagic demyelination of the brain white matter, and it is considered to be the most severe form of acute disseminated encephalomyelitis (ADEM). Herein we describe a case of 6-year-old boy presenting with elevated intracranial pressure and uncal herniation. Brain MRI showed a large right temporoparietal T2 hyperintense lesion with edema, abnormal perivascular enhancement and midline shift. Emergent decompressive craniotomy with brain biopsy was performed.

Results
H&E sections of the brain lesion revealed neutrophilic small vessel vasculitis with extravasation of blood and fibrin. Predominantly in a perivascular distribution were infiltrates of neutrophils and in areas foamy macrophages.
Additionally, the white matter was studded with hemorrhages of varying size. Sections stained with Luxol Fast Blue for myelin and with a neurofilament immunostain for axons were compared to reveal selective perivascular loss of myelin with relative sparing of axons.

Conclusion
The constellation of acute hemorrhages, neutrophilic infiltration and selective loss of myelin with relative sparing of axons is characteristic of this exceptional, hyperacute variant of acute disseminated encephalomyelitis.
Histomorphologic diagnosis is characteristic and may be crucial for timely decision for treatment of this often fatal disease.
Exogenous Lipoid Pneumonia Presenting as an Enlarging Lung Nodule in a Patient with a Long-standing Usage of Petroleum Jelly
Authors: Onyenekwu C P , Sheinin Y 
Published: October 2020

Abstract

Introduction/Objective
Lipoid pneumonia is an uncommon inflammatory lung lesion resulting from the aspiration of endogenous or exogenous oily substances. It has no age or sex preponderance, and its mode of presentation is non- specific. Radiologic imaging findings may mimic that of a malignant lung lesion.

Methods
We report a case of a 69-year-old female with a history of chronic obstructive pulmonary disease, rheumatoid arthritis and a 30-pack year smoking. She was being followed for an enlarging PET positive spiculated left upper lung nodule. She underwent a CT-guided core biopsy of the lung nodule.

Results
Biopsy showed histiocytes and multinucleated giant cells with lipid vacuoles and associated marked fibrosis. Review of a percutaneous lung biopsy performed two years prior showed a chronic inflammatory process with associated fibrosis in the presence of nonpolarizable vacuoles, many of which were intracytoplasmic within multinucleate foreign body type giant cells. A re-assessment of the patient with further history taking revealed a longstanding practice of nighttime application of petroleum jelly to nostrils and lips. Together, these findings are consistent with an exogenous lipoid pneumonia.

Conclusion
Lipoid pneumonia although an entity with limited literature description, should be considered in patients presenting with enlarging lung nodules. Early diagnosis of this lesion ensures appropriate treatment and prevents unnecessary invasive procedures.
Prognostic Marker Expression In Microinvasive Breast Cancer
Authors: Dimopoulos Y , Sidawy M 
Published: October 2020

Abstract

Introduction/Objective
Microinvasive breast carcinoma (Mi) is defined as invasive carcinoma (IC) less than 1 mm in greatest dimension. Depletion of the focus during further immunohistochemical investigation is commonly observed. Studies on prognostic marker expression report high concordance between the Mi and concomitant ductal carcinoma in-situ (DCIS). Compared to IC, Mi has a higher rate of Her2 positivity, indicating aggressive phenotype and potential variation in marker expression over time. We aimed to verify the concordance between Mi and DCIS in patients with pure Mi, as well as between foci of Mi and IC in patients with both.

Methods
A total of 21 cases of Mi and 9 cases with both IC and Mi disease were identified and evaluated for pathologic characteristics and ER, PR, and Her2 expression by immunohistochemistry. The rate of Her2 in Mi was compared to national benchmarks for IC.

Results
Mi was associated with high grade DCIS. 100% concordance of Her2 expression between Mi and DCIS was present in 14/21 cases where Mi was not depleted. Discrepancy between ER/PR was seen in two cases (Mi negative/DCIS focally positive). 50% of these Mi cases were Her2 positive, compared to the 13–25% generally reported for IC. Overall, by extrapolating Mi status from the concomitant DCIS when the Mi became depleted, 38% of Mi was positive for Her2. 100% concordance between foci of Mi and IC in patients presenting with both was observed.

Conclusion
Mi had a more aggressive prognostic marker phenotype compared to IC based on Her2 positivity. Concordance of prognostic marker expression between Mi and concomitant DCIS supports reporting of the DCIS status when the Mi becomes depleted, with only rare ER/PR discrepancies observed. Concordance between foci of Mi and IC in patients with both does not support time-based variation in marker expression. However, further investigation is warranted to uncover potential changes.
Isolated Congenital Bronchoesophageal Fistula in a Pediatric Patient: Case Report
Authors: Anderson S A , Liu S , Galliani C , Weaver K 
Published: October 2020

Abstract

Introduction/Objective
Congenital bronchoesophageal fistula (BEF) results from an undue passageway caudal to the foregut lung anlage. BEF may be associated with esophageal atresia and commonly involves the right lung. Herein we present a pediatric case of isolated congenital BEF masqueraded by severe dyspeptic symptoms.

Methods
A five-year-old child with a longstanding history of gastroesophageal reflux was hospitalized because of acute onset sharp lower chest/epigastric abdominal pain associated with emesis. Imaging disclosed a left lung abscess and empyema that responded to intravenous antibiotics, chest tPA instillation and drainage. After resolution of her empyema, she experienced severe dysphagia, chocking sensation, vomiting, and intermittent hematemesis. Esophogram disclosed a fistulous tract to the left lower chest cavity. Esophagoscopy revealed severe esophagitis and a stricture just proximal to a 0.6 to 0.8 cm ostium of a fistulous tract at 26 cm from the incisors, 2 cm above the gastroesophageal junction.

Results
A 103 g, 12 x 7.5 x 5.0 cm thoracoscopic left lower lobectomy showed a focally ulcerated fistulous tract that entered a 1.9 cm in diameter cyst that connected to a basilar-medial segmental bronchus. The passage was lined by stratified squamous epithelium, continuous with columnar ciliated epithelium, and was surrounded by intense chronic inflammation. The fistulous lumen contained partially degraded vegetable matter.
Diagnosing BEF can be clinically challenging. Symptoms are often intermittent, alternating between digestive and pulmonary in nature. Often, cough, gastroesophageal reflux, pneumonia, and bronchopulmonary suppuration can lead to medical management that is only masking, not definitively treating the underlying pathology. While tracheoesophageal fistulas are relatively common in presentation, and usually diagnosed in the newborn period, BEFs are rare and with varied clinical expression, which may explain delayed diagnosis.

Conclusion
BEF is a rare anomaly that may manifest ambiguous symptoms, thus delaying the diagnosis and options for earlier, less invasive curative interventions.
Intraoperative Fine Needle Aspiration Parathyroid Hormone Assay Can Be Used as an Alternative to Frozen Section Analysis for the Identification of Parathyroid Tissue
Authors: Olivas A D , van Wijk X M , Angelos P 
Published: October 2020

Abstract

Introduction/Objective
Histologic frozen section analysis is typically used for parathyroid tissue identification during parathyroidectomy when needed, in conjunction with a rapid intraoperative plasma parathyroid hormone (PTH) assay to confirm falling levels of circulating PTH after parathyroid excision. As an alternative to frozen section consults, we hypothesize that automated analysis of intraoperative fine needle aspiration (ioFNA) tissue samples using a rapid PTH immunoassay can accurately identify parathyroid tissue and reduce the need for frozen section consults.

Methods
A rapid PTH immunoassay (Elecsys PTH STAT; Roche Diagnostics, Indianapolis, IN), currently used for intraoperative plasma samples, was validated for FNA samples on a Cobas® e411 using tissue aspirates of ex vivo parathyroid and control specimens rinsed in 1mL saline. ioFNA PTH results during parathyroidectomy were then prospectively assessed for accuracy over a 4-month period by comparing values to final histopathologic diagnoses. The number of frozen section consults requested was compared to a 5-month period prior to the availability of the ioFNA PTH assay.

Results
Ninety patients underwent parathyroidectomy (128 excised parathyroids) during the study period, performed by a single experienced endocrine surgeon. Indications included primary (81/90), tertiary (5/90), and recurrent (4/90) hyperparathyroidism. Thirty-nine cases (55.5 excised parathyroids) were performed after the availability of the ioFNA PTH assay. ioFNA samples were sent for PTH analysis in 7/39 cases (18%; 12 samples total) and had a sensitivity/specificity of 100% (parathyroid [n=7] PTH values 1968 - &gt;5000pg/mL; non-parathyroid [n=5] PTH values &lt;2 - 16pg/mL). Parathyroidectomies requiring frozen section consult significantly decreased from 41% (21/51 cases; 40 specimens) to 10% (4/39 cases; 9 specimens) with the availability of the ioFNA PTH assay (p&lt; 0.05, Fisher exact test).

Conclusion
Analysis of ioFNA tissue samples using an automated rapid PTH immunoassay can accurately identify parathyroid tissue and can be used as an alternative to frozen section consult when needed.
Pyoderma Gangrenosum as the Initial Presentation of Inflammatory Bowel Disease
Authors: Karimi S S , Guzman G 
Published: October 2020

Abstract

Introduction/Objective
Pyoderma gangrenosum is a neutrophilic dermatosis, commonly associated with arthritis, psoriasis, and systemic lupus erythematosus. It is also an aggressive manifestation of extra-intestinal inflammatory bowel disease affecting less than 5% of patients with ulcerative colitis and Crohn disease. We present a case of pyoderma gangrenosum as an initial and rapidly progressive manifestation of Crohn disease.

Methods
A 35-year-old man presented with fecal urgency, periumbilical pain, and frequent diarrhea, with stool cultures revealing no infectious etiology. Colonoscopy demonstrated active inflammatory bowel disease consistent with Crohn disease. Concurrently, patient developed an ulcer on right lower extremity not otherwise contributed to history of trauma or injury. Biopsy of right lower extremity ulcer revealed histopathologic findings consistent with pyoderma gangrenosum. Despite medical management, and prolonged use of wound care and wound vac, patient’s non-healing ulcer continued to geographically expand, resulting in ankle contracture. A right below the knee amputation was performed and amputation specimen was sent to pathology for further diagnostic evaluation.

Results
Gross examination revealed an extensive cutaneous ulcer measuring 22.1cm x 11.7cm x 0.3cm and involving the anterior medial aspect of the right lower extremity with medial and posterior extension down to the dorsum of the foot with broad deep tissue destruction and exposure of fascia and tendon. Microscopic examination revealed severe ulceration, suppurative necrosis, superficial and deep vasculitis confined to the ulcer bed.
Surrounding soft tissue revealed chronic myopathic changes secondary to ischemia. Foci of commensal filamentous gram-positive bacterial colonization in a nidus of necrosis were also identified. The inflammatory pattern involved predominantly neutrophils with weak recruitment of other inflammatory cells, consistent with pyoderma gangrenosum.

Conclusion
This case highlights the debilitating extent of Crohn disease, its detrimental effects on the patient’s quality of life, and the diagnostic and treatment challenges it poses to clinicians in managing Crohn disease and its complications.