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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