PEComa: Another Member of the MiT Tumor Family?

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 locat...

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Bibliographic Details
Published in:International journal of surgical pathology 2008-01, Vol.16 (1), p.16-20
Main Authors: Righi, Alberto, Dimosthenous, Kypros, Rosai, Juan
Format: Article
Language:English
Subjects:
Aged, 80 and over
Antigens, Neoplasm
Basic Helix-Loop-Helix Leucine Zipper Transcription Factors - biosynthesis
Bone Neoplasms - metabolism
Bone Neoplasms - pathology
Breast Neoplasms - pathology
Child
Colonic Neoplasms - metabolism
Colonic Neoplasms - pathology
Epithelioid Cells - metabolism
Epithelioid Cells - pathology
Female
Humans
Immunohistochemistry
Male
Melanoma-Specific Antigens
Microphthalmia-Associated Transcription Factor - metabolism
Neoplasm Proteins - biosynthesis
Neoplasms, Second Primary - pathology
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Summary: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.
ISSN:1066-8969
1940-2465
DOI:10.1177/1066896907309733