Diagnosis and Management of Hereditary Renal Cell Cancer

Fred H Menko1, Eamonn R Maher2

  • 1Antoni van Leeuwenhoek Hospital, The Netherlands Cancer Institute, Amsterdam, The Netherlands. f.menko@nki.nl.

Insights

Hereditary renal cell cancer (RCC) involves genetic factors in 2-4% of cases. This review covers major hereditary RCC syndromes, genetic assessment criteria, and management for affected individuals and families.

Area of Science:

  • Oncology
  • Genetics
  • Nephrology

Background:

  • Renal cell cancer (RCC) encompasses diverse tumor types classified by histology and molecular pathogenesis.
  • Understanding molecular drivers has enabled targeted systemic therapies for RCC.
  • Genetic susceptibility accounts for 2-4% of RCC cases, termed hereditary RCC.

Purpose of the Study:

  • To describe the primary hereditary renal cell cancer syndromes.
  • To outline criteria for referring RCC patients for genetic assessment.
  • To discuss management strategies for hereditary RCC patients and at-risk relatives.

Main Methods:

  • Review of established hereditary renal cell cancer syndromes.
  • Analysis of current criteria for clinical genetic assessment in RCC.
  • Synthesis of management options for hereditary RCC and family members.

Main Results:

  • Identified key hereditary RCC syndromes, including von Hippel-Lindau disease as the most frequent.
  • Established referral criteria for genetic evaluation in RCC patients.
  • Outlined comprehensive management approaches for hereditary RCC.

Conclusions:

  • Hereditary factors play a significant role in a subset of renal cell cancer cases.
  • Systematic genetic assessment and tailored management are crucial for hereditary RCC.
  • Effective management strategies benefit both affected patients and their at-risk family members.

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