title: Perivascular epithelioid cell tumor (PEComa) of the urinary bladder: report of 3 cases and review of the literature.
abstract: The perivascular epithelioid cell family of tumors (PEComas) includes familiar lesions such as angiomyolipoma, lymphangioleiomyoma, and clear-cell "sugar" tumors of the lung. Less frequently, PEComas arise in various other locations throughout the body including soft tissue, bone, and visceral organs. We report 3 cases of PEComa arising in the urinary bladder in 2 men in their fourth decade, and 1 woman in her third decade. All 3 tumors showed histologic features characteristic of PEComa including spindled and epithelioid cell morphology with variable clear cell change, and all coexpressed melanocytic and smooth muscle associated markers by immunohistochemistry. Follow-up demonstrated an indolent course for 2 patients with no evidence of disease at 10 and 21 months, respectively, and the third case was recently diagnosed. We also provide a review of the 4 previously reported PEComas occurring in the bladder. PEComas of the urinary bladder should be carefully distinguished from a variety of histologically similar, but clinically dissimilar entities.
authors.affiliation: Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA.
authors.forename: William R
authors.initials: WR
authors.lastname: Sukov
authors.forename: John C
authors.initials: JC
authors.lastname: Cheville
authors.forename: Mahul B
authors.initials: MB
authors.lastname: Amin
authors.forename: Ruta
authors.initials: R
authors.lastname: Gupta
authors.forename: Andrew L
authors.initials: AL
authors.lastname: Folpe
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D054973:Perivascular Epithelioid Cell Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D001749:Urinary Bladder Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery