Renal cell carcinoma causes

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Overview

Most renal cell carcinomas develop sporadically. Only a few have genetic predisposition and are associated with known syndromes, such as von Hippel Lindau (VHL), hereditary paragangliomas, leiomyomatosis, and Birg-Hott-Dube (BHD) syndrome, among several others.

Causes

Sporadic development of renal cell carcinomas contributes to the majority of cases of the most common renal cell carcinoma, the conventional (or clear cell) carcinoma.

The following tables show the sporadic and hereditary renal-cell carcinomas and associated causes:

Sporadic Forms of Renal Cell Carcinoma

Sporadic Forms of Renal Cell Carcinoma

Histologic Appearance	Incidence	Gene	Frequency
Conventional (Clear Cell)	75	VHL	60 %
Papillary	12	MET TFE3	13 % < 1 %
Chromophobe	4
Oncocytoma	4
Collecting Duct	< 1
Unclassified	3 - 5

^{Adapted from Cohen HT, McGovern FJ. Renal-cell carcinoma.N Engl J Med. 2005; 353:2477-90}

Hereditary Forms of Renal Cell Carcinoma

Hereditary Forms of Renal Cell Carcinoma

Syndrome	Associated Gene	Common Histological Appearance
VHL Disease	VHL	Conventional (Clear Cell)
FCRC	Chromosome 3p translocation	Conventional (Clear Cell)
Hereditary Paraganglioma	SDHB	Conventional (Clear Cell)
HPRC	MET	Papillary
HLRCC	FH	Papillary
Birt-Hogg-Dube Syndrome	BHD	Chromophobe / Oncocytoma

^{Adapted from Cohen HT, McGovern FJ. Renal-cell carcinoma.N Engl J Med. 2005; 353:2477-90}