Table 1: Familial renal cancer syndromes.

SyndromePhenotypeRenal cancer manifestationGeneChromosomeMendelian Inheritance in Man (MIM) number

Von Hippel-Lindau (VHL)Renal tumors, adrenal pheochromocytomas, retinal angiomas, central nervous system hemangioblastomas, pancreatic cysts and neuroendocrine tumors, endolymphatic sac tumors, epididymal and broad ligament cystadenomasClear cell renal cell carcinomaVHL3p25193300

Hereditary papillary renal carcinoma (HPRC)Bilateral, multifocal renal tumorsPapillary renal cell carcinoma type 1MET7q31164860

Hereditary leiomyomatosis and renal cell carcinoma (HLRCC)Skin and uterine leiomyomas, renal tumorsPapillary renal cell carcinoma type 2FH1q42-43605839

Birt-Hogg-Dubé (BHD)Cutaneous fibrofolliculomas, lung cysts, spontaneous pneumothorax, renal tumorsHybrid oncocytic, chromophobe, and clear cell renal cell carcinoma; oncocytomaFLCN17p11135150