FH and renal cell carcinoma: As a rare subtype of renal cell carcinoma (RCC), fumarate hydratase-deficient renal cell carcinoma (FH-dRCC) is characteristic of pathologic germline/somatic mutations in the FH gene [1–3] while lesions with germline mutations are associated with hereditary leiomyomatosis and RCC (HLRCC) syndrome.