Definition

A rare, inherited disorder in which benign (not cancer) skin lesions called leiomyomas form in the smooth muscle tissue around the hair follicles. They usually form on the arms, legs, chest, and abdomen. Benign leiomyomas may also form as fibroids in the uterus in females. Patients with HLRCC have an increased risk of kidney cancer. The signs and symptoms of HLRCC usually do not appear until young adulthood. HLRCC is caused by a mutation in the FH gene. It is inherited in an autosomal dominant manner. Also called hereditary leiomyomatosis and renal cell cancer syndrome.