Hereditary renal tumors

The heterogeneous group of kidney tumors is characterized by varied degree of malignancy, origination from different tissues even outside the kidneys, and diverse genetic background.

Epidemiology

Renal cancer is the 7th leading cancer among men an the 12th among women. 2.6% of renal cancers are hereditary disorders.

Systematic

Hereditary kidney diseases
	Congenital abnormalities of the kidney and urinary tract
	Cystic kidney disease
	Disorders of tubular solute transport
	Hereditary glomerular disease
	Hereditary metabolic kidney disease
	Hereditary renal tumors
		Hereditary leiomyomatosis and renal cell cancer
			COL4A6
			FH
		Hereditary papillary renal cell carcinoma
			Hereditary papillary renal cell carcinoma 1
				MET
			PRCC
		Nonpapillary renal cell carcinoma
			DIRC2
			FLCN
			HNF1A
			HNF1B
			OGG1
			RNF139
			VHL
		Wilms tumor
			Aniridia-Wilms-tumor syndrome
				PAX6
				WT1
			Denys-Drash syndrome
				WT1
			Somatic nephroblastoma
				GPC3
				WT1
			WAGR syndrome
				PAX6
				WT1
		von Hippel-Lindau syndrome
			VHL
	Interstitial kidney disease
	Kidney disease appearing as endocrinological disorders
	Thrombotic microangiopathies
	Urolithiasis

References:

1.	Cohen HT et al. (2005) Renal-cell carcinoma.

2.	Orphanet article Orphanet ID 217071