MiT family translocation renal cell carcinoma in an elderly male

Translocation-associated renal cell carcinoma (t-RCC) is a relatively uncommon subtype of renal cell carcinoma characterized by recurrent gene rearrangements involving the TFE3 or TFEB loci. TFE3 and TFEB are members of the microphthalmia transcription factor (MiT) family, which regulate differentiation in melanocytes and osteoclasts. Renal cell carcinomas (RCCs) associated with Xp11 translocations have gene fusions involving TFE3, which has multiple gene partners; RCCs with t(6:11) translocations have MALAT1-TFEB gene fusions. These tumors are histologically diverse, often have papillary, alveolar, and nested growth pattern with clear and eosinophilic cells and psammoma bodies and are seen commonly in children and young adults, accounting to 40% of pediatric RCCs and 1.6%-4% of adult RCCs. The mean and median patient age is 31 years. Thus, distinguishing t-RCC from its morphologic, immunophenotypic, and molecular mimics has important clinical implications. Directed ancillary testing is an essential aspect to t-RCC cases and may include a panel of immunohistochemical stains, such as PAX8, pancytokeratins, AMACR, CD10, and TFE-3. We, hereby report a case of TFE3 positiveXp11 translocation renal cell carcinoma in a 52-year-old male which is unusual.