Perivascular epithelioid cell tumor (PEComa) of the urinary bladder: Report of 3 cases and review of the literature

William R. Sukov, John C. Cheville, Mahul B. Amin, Ruta Gupta, Andrew L. Folpe

Research output: Contribution to journalArticlepeer-review

47 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)304-308
Number of pages5
JournalAmerican Journal of Surgical Pathology
Issue number2
StatePublished - Feb 1 2009


  • Eiomyosarcoma
  • HMB45
  • Perivascular epithelioid cell neoplasm
  • Urinary bladder

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine


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