Treatment Strategies for Hereditary Kidney Cancer: Current Recommendations and Updates

Discov Med. 2022 Nov-Dec;34(173):205-220.


A subset of renal tumors (5-8%) are associated with syndromes such as von Hippel-Lindau (VHL) syndrome, Birt-Hogg-Dubé syndrome (BHD), tuberous sclerosis complex (TSC), hereditary papillary renal carcinoma (HPRC), hereditary leiomyomatosis and renal cell cancer syndrome (HLRCC), and BRCA1 associated protein (BAP1) tumor predisposition syndrome, succinate dehydrogenase RCC (SDHB/C/D). These syndromes have their specific defined genetic alterations and associated extrarenal manifestations. Due to varying histopathology and aggressiveness of the tumors amongst these syndromes, the management strategies can range from active surveillance to upfront surgical resection. This review delineates specific characteristics of the most common familial renal cancer syndromes and discusses current management strategies.

Publication types

  • Review

MeSH terms

  • Birt-Hogg-Dube Syndrome* / genetics
  • Carcinoma, Renal Cell* / therapy
  • Humans
  • Kidney Neoplasms* / genetics
  • Kidney Neoplasms* / pathology
  • Kidney Neoplasms* / therapy
  • Neoplastic Syndromes, Hereditary* / diagnosis
  • Neoplastic Syndromes, Hereditary* / genetics
  • Neoplastic Syndromes, Hereditary* / therapy
  • von Hippel-Lindau Disease* / genetics
  • von Hippel-Lindau Disease* / therapy