Perivascular Epithelioid Cell Tumor of Urinary Bladder

Am J Clin Pathol. 2021 Jun 17;156(1):56-71. doi: 10.1093/ajcp/aqaa204.


Objectives: To review the clinicopathologic features of perivascular epithelioid cell tumor (PEComa) of the urinary bladder.

Methods: Seven cases of bladder PEComa were studied by light microscopy, immunohistochemistry, and fluorescence in situ hybridization (FISH).

Results: In our 7 cases, 5 patients were female and 2 were male, with ages between 26 and 78 years. Patients presented with hematuria and recurrent abdominal discomfort as the main clinical symptoms. Microscopically, the epithelioid and spindle-shaped tumor cells with clear to granular eosinophilic cytoplasm were arranged in fascicular, acinar, or nested patterns. The tumor cells were positive for HMB45, melan-A, and SMA, but no TFE3 gene rearrangement was detected in any of the 7 samples by FISH. The analysis of all 35 cases from the literature and ours showed a patient age range from 16 to 78 years (mean age, 39 years), a male-to-female ratio of 1:1.3, maximal tumor diameters from 0.6 to 18.8 cm (mean, 4.5 cm). With a mean follow-up of 27 months, the recurrence, metastasis, and mortality rates were 10.7%, 10.7%, and 7.1%, respectively.

Conclusions: Bladder PEComa is extremely rare, remains a diagnostic challenge, and needs more attention. Strengthening the understanding of this tumor will improve diagnostic accuracy.

Keywords: Bladder; Diagnosis; FISH; PEComa; Perivascular epithelioid cell tumor; Prognosis; TFE3; Treatment.

Publication types

  • Review

MeSH terms

  • Adult
  • Aged
  • Female
  • Humans
  • Male
  • Middle Aged
  • Perivascular Epithelioid Cell Neoplasms / pathology*
  • Urinary Bladder Neoplasms / pathology*