Uterine leiomyomatosis is also associated with cutaneous leiomyomas and renal cell carcinoma in the hereditary leiomyomatosis and renal cell cancer (HLRCC) syndrome an autosomal dominant disorder with a mutation in the tumor suppressor gene coding for fumarate hydratase (fumarase).47 Two hundred families are known in the literature.47 Other organs may also develop tumors in this syndrome.26 This evidence concerns the gene FH and hereditary clear cell renal cell carcinoma.