Rare cases of PBMAH as part of other tumor predisposition syndromes have been described including familial adenomatous polyposis (APC), multiple endocrine neoplasia type 1 (MEN1), hereditary leiomyomatosis (fumarate hydrogenase, FH), and renal cell carcinoma (fumarate hydrogenase, FH) [66,67,68,69]. The gene discussed is FH; the disease is renal cell carcinoma.