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International Journal of Surgical Pathology
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PEComa: Another Member of the MiT Tumor Family?

Alberto Righi, MD

Centro Diagnostico Italiano (CDI), Milan, Anatomic Pathology, University of Bologna, Bellaria Hospital, Bologna Italy, alberto.righi{at}gmail.com

Kypros Dimosthenous, MD

Centro Diagnostico Italiano (CDI), Milan

Juan Rosai, MD

Centro Diagnostico Italiano (CDI), Milan

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.

Key Words: PEComa • MiT tumor family • malignant tumor

International Journal of Surgical Pathology, Vol. 16, No. 1, 16-20 (2008)
DOI: 10.1177/1066896907309733
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Malignant perivascular epithelioid cell tumour of the fibula: a report and a short review of bone perivascular epithelioid cell tumour
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