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RESEARCH ARTICLE Rheb/mTOR/p70s6k Cascade and TFE3 Expression in Conventional and Sclerosing PEComas of the Urinary Tract Marı´a Planelles, MD,* Laura Macı´as, MD,w Gloria Peiro´,MD,* Stela Bulimbasicˇ,MD,z Ondrej Hes, MD,y Antonio Robles, MD,w Michal Michal, MD,y Whitney Davidson, BS, PSF,8 and Jose´ I. Lo´pez, MDz Bonetti et al in 1992. Although these tumors show a wide Abstract: Perivascular epithelioid cell tumors (PEComas) are topographical distribution, few cases have been reported 2–5 rarely found in the urinary tract. The clinicopathologic char- within the urinary tract. The histologic features and acteristics of 10 cases, retrospectively collected from 5 medical typical immunohistochemical profile of conventional institutions in 3 different European countries, are presented in PEComas are well known. Sclerosing PEComa is a dis- this study. Male/female ratio was 3:7 and the average age at tinct variant that displays prominent stromal hyaliniza- diagnosis was 62.7 years. Nine cases were sporadic and 1 showed tion and a predisposition for the retroperitoneum. germline mutation of the TSC2 gene. Eight cases were located in Molecular genetic studies repeatedly reveal allelic the kidney, 1 in the left adrenal and 1 in the right ureter. All of loss of the TSC2 locus on 16p13 in PEComas, angio- the patients
Applied Immunohistochemistry & Molecular Morphology – Wolters Kluwer Health
Published: Aug 1, 2015
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