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.
All Author(s) ListRasalkar DD, Chu WCW, Chan AWH, Cheng FWT, Li CK
Journal namePediatric Radiology
Year2011
Month12
Day1
Volume Number41
Issue Number12
PublisherSPRINGER
Pages1587 - 1590
ISSN0301-0449
LanguagesEnglish-United Kingdom
KeywordsAdolescent; Kidney; Pigmented epitheloid tumor
Web of Science Subject CategoriesPediatrics; PEDIATRICS; Radiology, Nuclear Medicine & Medical Imaging; RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING

Last updated on 2020-24-11 at 00:24