Multicystic Clear Cell Renal Tumors With Low-grade Nuclear Features: Time to Include TFE3 Translocation-associated Carcinomas

Abstract

TFE3-rearranged renal cell carcinoma (RCC) is a distinct, uncommon entity with more than 20 different fusion partners identified; however, histomorphology may be suggestive of specific fusion partners in select TFE3-rearranged RCCs. For example, most MED15::TFE3 fusion associated RCCs exhibit multilocular cystic morphology, mimicking multilocular cystic renal neoplasm of low malignant potential. Here we present a case of MED15::TFE3 RCC in an older adult and review the literature with an emphasis on practical diagnostic approaches for predominantly cystic, low-grade, clear cell renal tumors.