Tumors with clear cells represent the most common group of renal neoplasms, with clear cell renal cell carcinoma (RCC) remaining the prototypical entity. Consequently, it is important to distinguish clear cell RCC from other entities with clear cell morphology, including TFE3-rearranged RCC, multiloculated cystic renal neoplasm of low malignant potential, clear cell (tubulo)papillary renal cell tumor, ELOC -mutated RCC, and RCC with fibromyomatous stroma. Some of these tumors can be aggressive, while others are almost always indolent. Accurate diagnoses prevent indolent cases from receiving unnecessary therapy or from inappropriately being enrolled in clinical trials with the potential of confounding their results. Molecularly classifying tumors may also lead to drugs that selectively target an oncogenic driver, thereby more effectively targeting the tumor. In current clinical practice, high-quality immunohistochemistry and molecular assays are continually enhancing our diagnostic ability. A discussion of these histotypes with clear cell morphology and how to discriminate them is the focus of this review.
Nick Baniak (Wed,) studied this question.