Genetic Counseling in Renal Masses

Table 2

Hereditary renal cell carcinoma (RCC) syndromes and histological subtypes.

Renal tumors	Manifestation	Disease	Gene
Clear cell RCC
	Bilateral and multiple	Von Hippel-Lindau	VHL, 3p25-26
	Bilateral and multiple	Chromosome 3 translocations	Unknown, VHL?
		Hereditary paraganglioma	SDHB, 1p36
	Angiomyolipomas	Tuberous sclerosis	TSC1, 9q34
			TSC2, 16q13
Papillary RCC	Solid, bilateral and multiple (type 1)	Hereditary papillary RCC	MET, 7q31
	Unilateral solitary, aggressive (type 2)	Hereditary leiomyomatosis	FH, 1q42-43
	Hamartomas, Wilm’s tumor	Hyperparathyroidism-jaw tumor	HRPT2, 1q25-32
	Oncocytoma	Familial papillary thyroid cancer	?, 1q21
Chromophobe RCC	Oncocytic-chromophobe	Birt-Hogg-Dubé	BHD, 17p11.2