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TFE3-associated PEComa

Monday 26 August 2019

TFE3-associated PEComas; Perivascular epithelioid cell neoplasms (PEComas)

Perivascular epithelioid cell neoplasms (PEComas) include the common renal angiomyolipoma, pulmonary clear cell sugar tumor, lymphangioleiomyomatosis, and less common neoplasms of soft tissue, gynecologic, and gastrointestinal tracts.

Immunoreactivity for TFE3 protein (a sensitive and specific marker of neoplasms harboring TFE3 gene fusions) has been reported in as many as 100% of PEComas

Although numbers are small, distinctive features of these cases include a tendency to young age, the absence of association with tuberous sclerosis, predominant alveolar architecture and epithelioid cytology, minimal immunoreactivity for muscle markers, and strong (3+) TFE3 immunoreactivity.

Despite significant morphologic and immunohistochemical overlap with other PEComas, PEComas harboring TFE3 gene fusions may represent a distinctive entity.



See also

- TFE3-associated tumors


- A distinctive subset of PEComas harbors TFE3 gene fusions. Argani P, Aulmann S, Illei PB, Netto GJ, Ro J, Cho HY, Dogan S, Ladanyi M, Martignoni G, Goldblum JR, Weiss SW. Am J Surg Pathol. 2010 Oct;34(10):1395-406. doi : 10.1097/PAS.0b013e3181f17ac0 PMID: 20871214