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.
Pediatr Radiol. 2011 Dec;41(12):1587-90. doi: 10.1007/s00247-011-2125-0. Epub 2011 May 20.
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.
肾恶性色素性透明细胞上皮样细胞瘤是血管周上皮样细胞瘤（PEComa）或上皮样血管平滑肌脂肪瘤（AML）的罕见变异型。PEComa 主要由上皮样细胞组成，缺乏经典 AML 中典型的脂肪细胞和血管。大多数上皮样 AML 为良性，但肾恶性上皮样 AML 偶见于成人，与结节性硬化症有关。我们报告了一名 15 岁男孩的恶性色素性透明细胞上皮样肾肿瘤的放射病理学特征，该患者广泛转移但无结节性硬化症的临床证据。
