CLINICAL CONSENSUS STATEMENT: GENETIC RISK ASSESSMENT IN HEREDITARY KIDNEY CANCER

INTRODUCTION AND OBJECTIVE: Renal cell carcinoma (RCC) is believed to have a strong hereditary component; yet, consensus guidelines for genetic risk assessment are lacking. A panel of experts was convened to gauge current opinions on who should be evaluated, what testing should be done, and when/how it should be performed. METHODS: A national multidisciplinary panel with expertise in hereditary kidney cancer was convened. All centers with a VHL program were invited as well as individuals attending the Kidney Cancer Research Summit (KCRS) who had relevant publications in hereditary RCC. Prior to the summit, a modified Delphi methodology was employed to generate, review, and curate a set of consensus questions regarding RCC genetic risk assessment, defined as genetic counseling and/or laboratory gene testing. Uniform consensus was defined as at least 85% agreement, consistent with the National Comprehensive Cancer Network criteria. RESULTS: Twenty-six panelists, including urologists (50%), medical oncologists (27%), genetic counselors (15%), clinical geneticists (8%), and patient advocates (4%) reviewed 52 curated consensus questions. Uniform consensus was achieved on 30 statements addressing for whom, what, when, and how genetic testing should be performed (Table 1). Areas with significant controversy included age cutoffs to prompt genetic risk assessment as well as interpretation of variants of unknown significance. CONCLUSIONS: In this first ever consensus panel on RCC genetic risk assessment, there was high consensus in several areas including who should be tested and how testing should be performed. Areas which require further research and discussion were also identified with a second future meeting planned. This consensus statement should provide initial guidance for clinicians when evaluating patients with an increased risk of a hereditary kidney cancer syndrome.Source of Funding: N/A