PEComa: Another member of the MiT tumor family?

Alberto Righi, Kypros Dimosthenous, Juan Rosai

Research output: Contribution to journalArticlepeer-review


We report 2 cases of PEComa, one occurring in the colon of an 11-year-old boy and the other in the bone (fibula) of a 92-year-old woman. Both tumors consisted of nests of large epithelioid cells surrounded by a fibrovascular stroma. The nuclei were large and vesicular, with prominent centrally located nucleoli. The cytoplasm was eosinophilic, with a fine to coarse granularity. Mitoses and individual cell necrosis were infrequent. Immunohistochemically, both tumors showed strong cytoplasmic expression of HMB-45 and intense nuclear positivity for TFE3. To our knowledge, nuclear positivity for TFE3 has been previously reported in only 5 cases of PEComa. Reactivity to this marker suggests that PEComa should be added to the growing list of human tumors of the so-called MiT family gene.

Original languageEnglish
Pages (from-to)16-20
Number of pages5
JournalInternational Journal of Surgical Pathology
Issue number1
Publication statusPublished - Jan 2008


  • Malignant tumor
  • MiT tumor family
  • PEComa

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine
  • Surgery


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