Hereditary leiomyomatosis and renal cell cancer (Q92888)

Hereditary leiomyomatosis and renal cell cancer (HLRCC) is an autosomal dominant hereditary cancer syndrome characterised by a predisposition to cutaneous pilar leiomyomata, uterine leiomyomata in women and renal cell cancer. The condition is associated in up to 16% of cases with aggressive renal cell carcinoma. Diagnosis is based on either the presence of multiple cutaneous leiomyomas with at least 1 histologically confirmed leiomyoma or the presence of a single leiomyoma with a positive family history of the disease.