An Adult Case of Melanotic Xp11 Translocation Renal Cancers: Distinct Entity or Sub-entity?

Melanotic Xp11 translocation renal cancer is a recently recognized aggressive epithelioid neoplasm with features overlapping between PEComa, carcinoma, and melanoma, in which TFE3 gene fusions coexist with melanin synthesis. These findings support the idea that melanotic Xp11 translocation renal cancer is a distinct variant of the MiT/TFE3 family neoplasms. The authors describe a pigmented renal tumor occurring in a 30-year-old woman with distinct morphology and immunohistochemical characteristics as Xp11 translocation renal cancer.