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Title of Journal: Pediatr Radiol

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Abbravation: Pediatric Radiology

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Springer-Verlag

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DOI

10.1002/eji.201570032

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1432-1998

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Malignant pigmented clear cell epithelioid cell tu

Authors: Darshana D Rasalkar Winnie Chiuwing Chu Anthony W H Chan Frankie W T Cheng Chi K Li
Publish Date: 2011/05/20
Volume: 41, Issue: 12, Pages: 1587-1590
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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 radiologicalpathological features of a malignant pigmented clear cell epithelioid renal tumor in a 15yearold boy presenting with extensive metastases but without clinical evidence of tuberous sclerosis

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