source: Medscape

year: 2017

authors: Elwyn C. Cabebe

The American Society of Colon and Rectal Surgeons recommends that patients with familial adenomatous polyposis (FAP) or with personal or family risk factors for FAP be referred to center registries and genetic counselors with experience in the multidisciplinary management of these individuals.

Additional recommendations include:

Prophylactic colectomy or proctocolectomy should be routine; the frequency and type of surgery should depend on the severity of the polyposis phenotype.

Use of chemoprevention as primary therapy is not recommended Small tubular adenomas with mild dysplasia can be kept under surveillance, but adenomas with severe dysplasia must be removed Duodenectomy or pancreaticoduodenectomy is recommended for persistent or recurrent severe dysplasia in the papilla or duodenal adenomas Clinically inert tumors should be treated with sulindac or not treated at all Slowly growing or mildly symptomatic tumors may be treated with less toxic regimens such as sulindac and tamoxifen or with vinblastine and methotrexate Rapidly growing tumors need aggressive therapy with either very-high-dose tamoxifen or antisarcoma-type chemotherapy Radiation is an option if collateral damage is not a major concern.