Leiomyomatosis and renal cell cancer- hereditary


Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a hereditary condition associated with multiple leiomyomas (fibroid skin tumors), uterine fibroids (non-cancerous growths), and type 2 papillary renal (kidney) cancer. A person with HLRCC can have a few skin tumors or many skin tumors. These tumors generally develop in adulthood and occur on the chest, back, arms, and legs; the tumors can be painful, but they are not cancerous. Women with HLRCC can develop uterine fibroids as young as their teens or early 20s.


HLRCC is a genetic condition. This means that the risk of cancer and other features of HLRCC can be passed from generation to generation in a family. A specific gene called the fumarate hydratase (FH) gene is believed to cause most cases of HLRCC. Research is ongoing to learn more about this condition.


HLRCC is suspected when a person has a history of multiple leiomyomas. A family history of fibroid tumors and type 2 papillary renal cancer also raises the possibility of HLRCC. Genetic testing to look for mutations in the FH gene is available for people suspected of having HLRCC.