Treatment Strategies for Hereditary Kidney Cancer: Current Recommendations and Updates

Shiva Singh; Aditi Chaurasia; Nikhil Gopal; Ashkan Malayeri; Mark W Ball

Treatment Strategies for Hereditary Kidney Cancer: Current Recommendations and Updates

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

Authors

Shiva Singh¹, Aditi Chaurasia², Nikhil Gopal², Ashkan Malayeri¹, Mark W Ball^{2

3}

Affiliations

¹ Radiology and Imaging Sciences, Warren Grant Magnuson Clinical Center, National Institutes of Health, 10 Center Drive, Bethesda, MD 20892, USA.
² Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bldg. 10, 10 Center Drive, Bethesda, MD 20892, USA.
³ Corresponding author.

PMID: 36602871

Abstract

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