Despite the vast majority of renal cancers being sporadic, there are a number of hereditary renal cancer syndromes:
- von Hippel Lindau syndrome: predominantly clear cell type
- tuberous sclerosis: predominantly clear cell type (also associated with angiomyolipoma)
- hereditary paraganglioma-pheochromocytoma syndromes: predominantly clear cell type
- hereditary papillary renal cell carcinoma: papillary type 1
- hereditary leiomyomatosis and renal cell carcinoma: predominantly papillary type 2
- Birt-Hogg-Dubé syndrome: predominantly chromophobe type, oncocytoma, or a hybrid of both
- hereditary non-polyposis colon cancer (Lynch type II): predominantly upper tract urothelial carcinoma
- sickle cell trait or hemoglobin SC disease: renal medullary carcinoma
- hereditary renal oncocytoma: oncocytoma