| Tumour type | Syndrome | Clinical manifestation | Chromosome | Candidate gene |
| ccRCC | Von Hippel-Lindau (VHL) | Retinal, cerebellar and spinal hemangioblastoma; pheochromocytoma; pancreatic, epididymal and renal cysts; hemangiomas of the adrenals, liver, and lungs; endolymphatic sac tumours; cystadenoma of the epididymis or broad ligament and ccRCC (40–75%) [19, 20] | 3p25 | VHL | Constitutional chromosome 3 translocation | ccRCC | Various breakpoints in chromosome 3 | Possible breakpoint associated genes: NORE1, LSAMP, DIRC1, DIRC2, DIRC3, FHIT, TRC8 Possible known common gene lost from translocation: VHL |
| pRCC | Hereditary papillary RCC (HPRC) | pRCC | 7q31 | MET | Hereditary leiomyomatosis and RCC (HLRCC) | Cutaneous leiomyomas, uterine fibroids, type II Renal tumours (14–18%) for example pRCC and CDRCC [149, 152, 154, 203] | 1q42-43 | FH |
| chRCC or RO | Birt-Hogg-Dubé (BHD) | Skin fibrofolliculomas, pulmonary cysts, spontaneous pneumothorax, and renal cancer (20–29%) (chRCC, RO, ccRCC and pRCC) [204–206] | 17p11.2 | BHD, FLCN |
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