Genetics & Genomics

Renal Cell Cancer Syndromes: Identification and Management of Patients and Families at Increased Risk

Melissa Paquin

Tracy Fasolino

hereditary renal cell cancer syndromes, mutation, autosomal dominant, genetics
CJON 2020, 24(4), 356-359. DOI: 10.1188/20.CJON.356-359

There are many inherited renal cell cancer syndromes that increase an individual’s risk of developing renal cell cancer. The age of onset for these renal cell cancer syndromes ranges from infancy to age 65 years. Clinical manifestations vary widely, and multiple body systems can be involved and present unique challenges to the healthcare team. With the advancement of genetic panels, clinicians can screen individuals with known hereditary syndromes for genetic mutations. This article offers clinically relevant information specific to various major renal cell cancer syndromes.


  • Most renal cell cancer syndromes are autosomal dominant and increase an individual’s risk of developing renal cell cancer and other malignancies. 
  • A wide range of clinical manifestations include benign and malignant histology. 
  • Heightened surveillance and preemptive management of individuals with known renal cell cancer syndromes can improve outcomes and quality of life. 
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