| Syndrome | Gene/protein | Chromosomal locus | Potential pathway | Clinical features |
| Hereditary papillary renal cancer | c-MET | 7q31 | HGFR | Papillary type I renal cell carcinoma |
| Hereditary leiomyomatosis renal cell carcinoma | Fumarate hydratase | 1q42 | Krebs cycle/HIF1 | Papillary type II renal cell carcinoma/skin carcinoma, and uterine leiomyoma |
| Birt-Hogg-Dube | Folliculin | 17p11 | mTOR | Chromophobe, oncocytic, hybrid, and clear cell renal cell carcinoma, fibrofolliculoma, pulmonary cysts, pneumothorax |
| Hereditary paraganglioma and pheochromocytoma | Succinate dehydrogenase | 5p15 | Krebs cycle/hypoxia | Clear cell, chromophobe renal cell carcinoma, pheochromocytoma, paragangliomas |
| Tuberous sclerosis complex (TSC) | TSC1 TSC2 | 9q34 16p13 | mTOR | Clear cell renal cell carcinoma, angiomyolipoma |
| von Hippel-Lindau (VHL) | VHL gene | 3p25 | HIF-1 | Clear cell renal cell carcinoma, hemangioblastomas, retinal angiomas, pheochromocytomas, endolymphatic sac tumors of the middle ear |
