Skip to main content
. Author manuscript; available in PMC: 2016 Sep 26.
Published in final edited form as: J Clin Oncol. 2009 Jul 13;27(23):3731–3733. doi: 10.1200/JCO.2009.23.0045

Table 1.

Hereditary Renal Cancer Syndromes

Syndrome Histology Gene
von Hippel-Lindau (VHL) Clear Cell
Renal Cysts		 VHL
Hereditary Papillary Renal Cell Carcinoma (HPRCC) Type 1 Papillary MET
Birt-Hogg-Dubé (BHD) Chromophobe
Hybrid-Oncocytic
Clear Cell
Oncocytoma
Renal Cysts		 BHD
Hereditary Leiomyomatosis Renal Cell Carcinoma (HLRCC) Type 2 Papillary
Renal cysts		 FH
Tuberous Sclerosis (TSC) Angiomyolipoma
Clear Cell
Oncocytoma		 TSC1
TSC2
Succinate Dehydrogenase B (SDHB)-associated renal cancer Clear Cell
Chromophobe
Type 2 papillary
Oncocytoma		 SDHB

Abbreviations: FH, fumarate hydratase