Table 3b. [Genetic Disorders Associated with Renal Cell Carcinoma]. - GeneReviews®

Gene	Disorder	MOI	Clinical Characteristics
FH	FH tumor predisposition syndrome	AD	Characterized by cutaneous leiomyomata, uterine leiomyomata (fibroids), &/or renal tumors. Pheo & PGL have been described in small number of families. Median age of detection is ~40 yrs.
FLCN	Birt-Hogg-Dubé syndrome	AD	Cutaneous manifestations (fibrofolliculomas, acrochordons, angiofibromas, oral papules, cutaneous collagenomas, & epidermal cysts), pulmonary cysts/history of pneumothorax, & various types of renal tumors. Median age of renal tumor diagnosis is 48 yrs.

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