Novel high-grade endometrial stromal sarcoma: a morphologic mimicker of myxoid leiomyosarcoma

Endometrial stromal sarcomas (ESS) are often underpinned by recurrent chromosomal translocations resulting in the fusion of genes involved in epigenetic regulation. To date, only YWHAE-NUTM2 rearrangements are associated with distinctive high-grade morphology and aggressive clinical behavior. We identified 3 ESS morphologically mimicking myxoid leiomyosarcoma of the uterus and sought to describe their unique histopathologic features and identify genetic alterations using next-generation sequencing. All cases displayed predominantly spindled cells associated with abundant myxoid stroma and brisk mitotic activity. Tumors involved the endometrium and demonstrated tongue-like myometrial infiltration. All 3 were associated with an aggressive clinical course, including multisite bony metastases in 1 patient, progressive peritoneal disease after chemotherapy in another and metastases to the lung and skin in the last patient. All 3 ESS were found to harbor ZC3H7B-BCOR gene fusions by targeted sequencing and fluorescence in situ hybridization. On the basis of the review of these cases, we find that ESS with ZC3H7B-BCOR fusion constitutes a novel type of high-grade ESS and shares significant morphologic overlap with myxoid leiomyosarcoma

Undiagnosed fatal malignancy in adult autopsies: a 10-year retrospective study.

Autopsies reveal undiagnosed malignancies even in this era of modern diagnostic imaging and ancillary laboratory studies. The incidence of clinically undiagnosed malignancies related to primary cause of death in a university hospital setting has rarely been studied. Our objective was to determine the incidence of clinically undiagnosed fatal malignancies in an urban teaching hospital and to assess whether this incidence was influenced by the duration of hospital stay. We performed a retrospective analysis of consecutive adult hospital autopsies in which the primary cause of death was related to undiagnosed malignancy. The detailed autopsy report, clinical records, and relevant laboratory results from laboratory and hospital databases were reviewed. Eight hundred twenty-one adult autopsies were performed over 10 years. Sixty-nine clinically undiagnosed malignant neoplasms were found in 66 cases (8%). In 26 of these 66 cases, the undiagnosed malignant neoplasm was related to the primary cause of death (3.1%). The duration of hospital stay was less than 24 hours in 8 cases (30.7%), 2 to 7 days in 9 cases (34.6%), and greater than 7 days in 9 cases (34.6%). In 10 of these 26 cases (1.2%), there was suspicion of malignancy without definitive tissue diagnosis, and the average hospital stay was 4.9 days. In 16 (1.9%) of these 26 cases, malignancy was not clinically suspected, and the average hospital stay was 9.9 days. We conclude that despite the recent advances in diagnostic imaging and laboratory techniques, a subset of adult autopsies (3.1%) performed in an urban university hospital have clinically undiagnosed malignancy related to the primary cause of death. Malignancy was not clinically suspected in 1.9% of these cases. There is a need to conduct comprehensive root cause analysis in these cases for improvement of clinical care

Aggressive Subcutaneous Panniculitis-Like CD30+ Peripheral T-Cell Lymphoma with Diffuse EBER Expression

T-cell lineage lymphoma with an intense membranous and paranuclear CD30 expression in the absence of ALK1 raises a differential diagnosis of peripheral T-cell lymphoma (PTCL), NOS and anaplastic large cell lymphoma (ALCL), ALK negative. However, Epstein-Barr virus is consistently negative in ALCL and is not considered an implicating factor in its pathogenesis. We describe a case of T-cell lymphoma showing anaplastic large cell morphology with scattered hallmark cells and a uniform CD30 and Epstein-Barr virus encoded early RNA (EBER) expression that primarily involved the subcutaneous tissue at presentation. On incisional biopsy, the neoplastic cells were positive for CD3, CD2, and CD30 while negative for LCA, CD20, PAX5, CD56, ALK1, and cytotoxic granules. Molecular analysis identified a positive T-cell receptor (beta and gamma) gene rearrangement by PCR. Proliferation index approached 100% and the patient had a rapidly progressive course; the subcutaneous lesions more than doubled in size within couple of weeks with new evidence for widespread systemic involvement. This case emphasizes a rare EBV association with a CD30 positive T-cell lymphoma where the morphologic and immunophenotypic findings are otherwise nondiscriminatory between PTCL, NOS and ALCL, ALK negative

Adrenal cortical adenoma arising in the setting of renal–adrenal fusion: a case report and review of the literature

Abstract Background Renal–adrenal fusion is a rare entity resulting from incomplete encapsulation of the adrenal gland and kidney. Only 18 cases have been reported in English literature to date. Case presentation Our patient is a 77-year-old African American female who presented with a systolic blood pressure of 200 mmHg. Computed tomography showed an enhancing 9 × 6 cm mass anterior and medial to the left kidney. The patient underwent a left adrenalectomy with partial nephrectomy. Gross and histologic examinations revealed an adrenal cortical adenoma and renal–adrenal fusion. Conclusion Renal–adrenal fusion may pose a diagnostic challenge, particularly when there is a concurrent adrenal adenoma. It is important to be aware of this uncommon anomaly to avoid misdiagnosis and overtreatment

Multi-organ involvement of bacillary angiomatosis masquerading as Kaposi sarcoma and pyogenic granuloma: A case report and literature review

Bacillary angiomatosis is a vasoproliferative neoplasm primarily seen in immunocompromised individuals. There are overlapping clinical and histopathologic manifestations of bacillary angiomatosis, Kaposi sarcoma, and pyogenic granuloma. Distinguishing an accurate diagnosis between these entities utilizing clinical, histopathologic, and molecular analysis becomes significant as prognosis and treatment differ. Here, we describe a case of bacillary angiomatosis masquerading as a Kaposi sarcoma and pyogenic granuloma and also highlight unusual presentations and the diagnostic approach to this entity

Microscopic Examination of Clots from Percutaneous Mechanical Embolectomies in Pulmonary Embolism

: Pulmonary embolism (PE) is a very common clinical entity with clinical symptoms that range from no symptom to complete hemodynamic collapse, sometimes with similar-appearing clot burden on computed tomographic pulmonary angiogram. Given highly variable clinical presentation, the authors wanted to investigate if there is clinical correlation based on the age of a clot with microscopic examination to clinical presentation. Thirteen thrombectomy aspirates from patients with an acute PE were microscopically analyzed. The goal was to age the thrombus based on histologic features and correlate it to clinical course