title: Malignant pigmented clear cell epithelioid cell tumor (PEComa) in an adolescent boy with widespread metastases: a rare entity in this age group.
abstract: Malignant pigmented clear cell epithelioid cell tumor of the kidney is a rare variant of perivascular epithelioid cells tumors (PEComa) or epithelioid angiomyolipoma (AML). PEComa is characteristically composed purely of epitheloid cells. The fat cells and the blood vessels that are typical of classic AML are absent. Most epithelioid AML cases are benign; however, malignant epithelioid AML of the kidney has been occasionally reported in adults in association with tuberous sclerosis. We report the radiological-pathological features of a malignant pigmented clear cell epithelioid renal tumor in a 15-year-old boy presenting with extensive metastases but without clinical evidence of tuberous sclerosis.
authors: Department of Imaging and Interventional Radiology, Faculty of Medicine, The Chinese University of Hong Kong, Prince of Wales Hospital, Ngan Shing St, Shatin, New Territories, Hong Kong.
authors: Darshana D
authors: DD
authors: Rasalkar
authors: Winnie Chiu-wing
authors: WC
authors: Chu
authors: Anthony W H
authors: AW
authors: Chan
authors: Frankie W T
authors: FW
authors: Cheng
authors: Chi K
authors: CK
authors: Li
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