Other autosomal dominant inherited syndromes associated to aggressive kidney cancers are hereditary Leiomyomatosis and Renal Cell Carcinoma (HLRCC) caused by loss of function of Fumarate Hydratase (FH), key enzyme of TCA cycle [8], Hereditary Papillary RCC (HPRC) which is linked to activating germline mutations in MET Proto-Oncogene tyrosine kinase receptor (MET) gene [9]. The gene discussed is FH; the disease is kidney cancer.