Rarely, it is a component of multiple tumor syndromes, such as familial adenomatous polyposis (inactivating mutations in the APC gene), multiple endocrine neoplasia type 1 (MEN1 [loss of function mutations in the MEN1 gene]), or hereditary leiomyomatosis and renal cell carcinoma (inactivating mutations in the FH gene) [27–29]. Here, MEN1 is linked to hereditary clear cell renal cell carcinoma.