Hereditary Cancer Syndromes > RNF43
GENETIC CANCER RISK
RISK FOR GENERAL POPULATION
To age 80
AGE TO BEGIN
RNF43 mutation carriers who do meet WHO criteria for a diagnosis of SPS should be managed based on guidelines from NCCN and other professional societies. These guidelines include recommendations for frequent colonoscopy screenings, beginning at young ages, and surgical evaluation based on family history and personal polyp history.
When identified as meeting WHO criteria for SPS
Currently there are no specific management guidelines for colorectal cancer risk in RNF43 mutation carriers who do not meet WHO criteria for a diagnosis of SPS. However, the possibility of an increased risk for colorectal cancer warrants consideration of individualized colorectal cancer risk-reduction strategies, such as the modification of standard population screening recommendations by starting screening at younger ages and/or performing screening more frequently.