C-Kit-positive metastatic malignant pigmented clear-cell epithelioid tumor arising from the kidney in a child without tuberous sclerosis |
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Authors: | Yu Weiming Fraser Robert B Gaskin David A Fernandez Conrad V Wright James R |
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Institution: | aDepartment of Pathology and Laboratory Medicine, Alberta Children's Hospital, (Calgary Laboratory Services), Calgary, Alberta, Canada T2T 5C7;bDepartment of Pathology, IWK Health Centre, Halifax, Nova Scotia, Canada B3K 6R8;cDepartment of Pediatrics, IWK Health Centre, Halifax, Nova Scotia, Canada B3K 6R8 |
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Abstract: | We report the first pediatric case of malignant pigmented epithelioid clear-cell tumor arising from kidney; the lesion occurred in a 12-year-old girl without tuberous sclerosis. The tumor was widely metastatic to the retroperitoneum and chest, and the patient died of the disease 9 months after diagnosis, despite active chemotherapy. Pigmented epithelioid clear-cell tumor of the kidney is a rare variant of epithelioid angiomyolipoma and a member of the family of perivascular epithelioid cell tumors (PEComas). The tumor demonstrated overlapping features between clear-cell sugar tumor and epithelioid variant of angiomyolipoma. Tumor cells were positive for HMB-45 expression, negative for any evidence of muscular differentiation, and contained melanin pigment and premelanosomes in the cytoplasm. Diffuse C-Kit (CD117) positivity was identified throughout the tumor. This is the first report of C-Kit–positive malignant PEComas. |
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Keywords: | Epithelioid angiomyolipoma C-Kit Kidney Malignant PEComa |
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