Perivascular epithelioid tumor of urinary bladder and vagina

Ann Diagn Pathol. 2005 Oct;9(5):275-8. doi: 10.1016/j.anndiagpath.2005.05.003.


Perivascular epithelioid cell tumors are recently characterized neoplasms composed of large clear HMB-45-positive epithelioid cells arranged in an organoid pattern. The histogenesis of these neoplasms remain obscure. We report here 2 such tumors in young female patients aged 19 and 16 years involving the urinary bladder and vagina, respectively (in the absence of the tuberous sclerosis complex) . Microscopically, both tumors were composed of perivascularly arranged cells with granular eosinophilic fluffy cytoplasm, round-to-oval nuclei, and prominent nucleoli. Melanin pigmentation was present in the vaginal tumor. Immunohistochemically, both tumors strongly expressed HMB-45. They were negative for cytokeratin, vimentin, and S-100. The vaginal tumor recurred 10 months after resection and chemotherapy. The patient with the bladder tumor was lost to follow-up.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Biomarkers, Tumor / analysis
  • Epithelioid Cells / metabolism
  • Epithelioid Cells / pathology*
  • Female
  • Humans
  • Immunohistochemistry
  • Neoplasm Recurrence, Local / metabolism
  • Neoplasm Recurrence, Local / pathology
  • Urinary Bladder Neoplasms / metabolism
  • Urinary Bladder Neoplasms / pathology*
  • Vaginal Neoplasms / metabolism
  • Vaginal Neoplasms / pathology*


  • Biomarkers, Tumor