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sinonasal PEComa

Saturday 28 January 2017

Pathology

- NONO-TFE3 Fusion in PEComa

  • Perivascular epithelioid cell neoplasms (PEComas) are a family of mesenchymal tumors with features of both smooth muscle and melanocytic differentiation, with or without true melanin pigment.
  • The highly variable morphology of PEComas results in a broad differential diagnosis that is also dependent on anatomic site.
  • A subset demonstrates rearrangements involving the TFE3 (Xp11) locus, which can be used in diagnostically difficult cases.
  • PEComa must enter the differential for pigmented lesions of the sinonasal mucosa, where malignant melanoma would be much more likely to occur.

Paywall references

- Melanotic PEComa of the Sinonasal Mucosa With NONO-TFE3 Fusion: An Elusive Mimic of Sinonasal Melanoma. McGregor SM, Alikhan MB, John RA, Kotler H, Bridge JA, Mujacic I, Kadri S, Segal J, Krausz T. Am J Surg Pathol. 2016 Dec 22. doi : 10.1097/PAS.0000000000000778
PMID: 28009605