Table 1.

Inherited renal cancer syndromes.

Syndrome	Chromosome location	Predisposing gene	Renal tumor histology	Recommended surgical management	Potential therapeutic targets
Von Hippel-Lindau disease (VHL)	3p25	VHL	Clear cell	Active surveillance <3 cm; surgical excision ≥3 cm	HIF-VEGF pathway
Hereditary papillary renal carcinoma (HPRC)	7q31	MET	Type 1 papillary	Active surveillance <3 cm; surgical excision ≥3 cm	Met kinase
Birt-Hogg-Dubé syndrome (BHD)	17p11.2	FLCN	Chromophobe, hybrid oncocytic, clear cell, oncocytoma	Active surveillance <3 cm; surgical excision ≥3 cm	mTOR pathway
Hereditary leiomyomatosis and renal cell carcinoma (HLRCC)	1q42–43	FH	Type 2 papillary	Wide margin surgical excision	HIF-VEGF pathway; antioxidant response pathway; reductive carboxylation pathway
Succinate dehydrogenase-deficient renal cancer (SDH-RCC)	1p36.13 1q23.3 11q23.1	SDHB SDHC SDHD	Clear cell, chromophobe, oncocytic neoplasm	Surgical excision	HIF-VEGF pathway; reductive carboxylation pathway
Tuberous sclerosis complex (TSC)	9q34 16p13.3	TSC1 TSC2	Angiomyolipoma, RCC, variable	AML, embolization; RCC, surgical excision	mTOR pathway
BAP1 tumor predisposition syndrome	3p21.2	BAP1	Clear cell, can be high grade	Surgical excision	TBD
MiTF-associated cancer syndrome	3p14.1-p12.3	MiTF	ND	TBD	TBD

ND, not determined; TBD, to be determined; RCC, renal cell carcinoma.