Among familial RCC syndromes, the fumarate hydratase (FH) germline mutation that interferes with the function of the Krebs cycle has been highlighted [18], causing hereditary leiomyomatosis and renal cell cancer (HLRCC) and being associated with an aggressive form of papillary RCC (pRCC) [19]. The gene discussed is FH; the disease is renal cell adenocarcinoma.