Malignant perivascular epithelioid cell tumor (PEComa) of the femur: a case report and literature review
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Malignant perivascular epithelioid cell tumor (PEComa) of the femur: a case report and literature review
BACKGROUND We describe a case of malignant perivascular epithelial cell tumor (PEComa) arising primarily in the distal left femur of a 47-year-old male. CASE PRESENTATION The patient presented with pain accompanied by progressive swelling of his left thigh. Computed tomography (CT) scan and magnetic resonance imaging (MRI) revealed an osteolytic lesion. Curettage of the lesion was reported as...
متن کاملMalignant perivascular epithelioid cell tumor of the orbit: Report of a case and review of literature
Perivascular epithelioid cell tumor (PEComa) is a rare neoplasm considered to arise from myomelanocytic cell lineage. The uterus is reportedly the most common site to be involved. Orbital PEComa is extremely rare with only two cases reported till date. A 5-year-old male presented with a right medial orbital mass for the last 6 months. The patient was diagnosed with alveolar soft part sarcoma el...
متن کاملMalignant perivascular epithelioid cell tumor (PEComa) of cervix with TFE3 gene rearrangement: a case report.
In this study, we reported the first PEComa arising within the cervix with TFE3 gene rearrangement and aggressive biological behavior. Morphologically, the tumor showed infiltrative growth into the surrounding parenchyma. The majority of tumor cells were arrayed in sheets, alveolar structures, or nests separated by delicate fibrovascular septa. There was marked intratumoral hemorrhage, necrosis...
متن کاملPEComa: A Perivascular Epithelioid Cell Tumor in the Liver—A Case Report and Review of the Literature
Perivascular epithelioid cell tumors are soft tissue tumors that can occur in various locations in the body whose incidence is rising. Hepatic PEComas are quite rare and diagnosis involves positivity of Melan-A and HMB45 on immunohistochemistry. Usual treatment is surgery for benign tumors and chemotherapy including mTOR inhibitors for malignant tumors. Here we discuss the radiological and path...
متن کاملPrimary Perivascular Epithelioid Cell Tumor (PEComa) of the Liver - A Case Report and Review of the Literature -
Perivascular epithelioid cells (PECs) were initially proposed, by Bonetti et al. in 1992, to describe an “unusual atypical cell type” of epithelioid shape and harboring a clear to eosinophilic cytoplasm with perivascular distribution in both renal angiomyolipoma (AML) and clear cell sugar tumor (CCST) of the lung. This perception gradually became accepted and led to the concept of perivascular ...
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ژورنال
عنوان ژورنال: Diagnostic Pathology
سال: 2015
ISSN: 1746-1596
DOI: 10.1186/s13000-015-0292-2