For its diagnosis it is mandatory to exclude other types of Renal Cell Carcinomas (RCC), such as MiTT translocated RCC (positive TFE3 or TFEB immunohistochemistry-IHC, break apart in situ hybridization, FISH), Fumarate hidratase deficient RCC (negative fumarate hidratase IHC), Clear Cell RCC (positive CAIX-Vimentin IHC), renal medullary carcinoma (SMARC-B1 deficiency by IHC) and urothelial carcinomas with glandular differentiation (positive CK7, GATA3, Uroplakin, negative PAX8). The gene discussed is PAX8; the disease is renal cell carcinoma.