Genetic Counseling in Renal Masses

Table 1

Classification of renal epithelial tumors.

Histological type	Frequency	Cell of origin	Behavior	Gene involved	Chromosomal abnormalities
Conventional (clear-cell) renal-cell carcinoma	75%	Proximal renal tubule	Malignant	VHL, BHD	−3p, +5q, −Y, −8p, −9p, −14q;
					t(3;5)(p;q)
Papillary renal-cell carcinoma	10–15%	Proximal renal tubule	Malignant	MET, FH, HRPT2	+7, +17, −Y, +12, +16, +20;
					t(X;1)(p11.2;q21.2),
					t(X;17)(p11.2;q25.3)
Chromophobe renal carcinoma	5%	Intercalated cell of renal collecting duct	Rarely malignant	BHD	−1, −2, −6, −10, −13, −17, −21
Oncocytoma	5%	Intercalated cell of renal collecting duct	Benign	BHD	−1, −Y; t(5;11)(q35;q13),
					t(9;11)(p23;q13)
Collecting-duct carcinoma	2%	Renal collecting duct	Aggressively malignant	FH	−1p32, −6p, −8p, −21q

BHD, Birt-Hogg-Dubé (encoding folliculin); FH, fumarate hydratase; HRTP2, hyperparathyroidism 2; VHL, von Hippel-Lindau.