Malignant pigmented clear cell epithelioid cell tumor (PEComa) in an adolescent boy with widespread metastases: A rare entity in this age group
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AbstractMalignant 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. © 2011 Springer-Verlag.
All Author(s) ListRasalkar D.D., Chu W.C.-W., Chan A.W.H., Cheng F.W.T., Li C.K.
Journal namePediatric Radiology
Volume Number41
Issue Number12
PublisherSpringer Verlag
Place of PublicationGermany
Pages1587 - 1590
LanguagesEnglish-United Kingdom
KeywordsAdolescent, Kidney, Pigmented epitheloid tumor

Last updated on 2021-13-10 at 00:01