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. Recently,
aberrant 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;
however, TFE3 gene status in these neoplasms has not been systematically investigated …