The hereditary form is associated with biallelic inactivation of the gene encoding the Krebs cycle enzyme fumarate hydratase (FH), which leads to hereditary leiomyomatosis and RCC (HLRCC) syndrome, which is characterized by a high incidence of RCC, uterine leiomyoma, and cutaneous leiomyomatosis [13, 14]. Here, FH is linked to Uterine leiomyoma.