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perivascular epithelioid cell neoplasm

Wednesday 26 June 2013

PEComa; PEComas

Molecular variants

- TFE3-asscociated PEComas

  • A small subgroup of PEComas has been recognized to harbor rearrangements involving TFE3, a gene also involved in rearrangements in translocation-associated renal cell carcinomas and alveolar soft part sarcomas.
  • The few TFE3 rearrangement-associated PEComas reported have exhibited distinctive pathologic characteristics contrasting to PEComas in general, including predominantly epithelioid nested or alveolar morphology and underexpression of muscle markers by immunohistochemistry.
  • PEComas with TFE3 rearrangement, indicating that both can pursue an aggressive course.
  • The few reported TFE3-rearranged PEComas have predominantly lacked a spindle cell component and expression of smooth muscle actin and MiTF by immunohistochemistry, but these features are sometimes present in TFE3-rearranged PEComas. (23797724)


- Malignant Perivascular Epithelioid Cell Neoplasm (PEComa) of the Urinary Bladder With TFE3 Gene Rearrangement: Clinicopathologic, Immunohistochemical, and Molecular Features. Williamson SR, Bunde PJ, Montironi R, Lopez-Beltran A, Zhang S, Wang M, Maclennan GT, Cheng L. Am J Surg Pathol. 2013 Jun 20. PMID: 23797724