Surveillance

Examination of the funds at birth to detect CHRPE can identify susceptible family members at young age. In family members of a FAP-positive patient, sigmoidoscopy is recommended annually starting at age 10-12 years to age 24 years, every 2 years until age 34 years, every 3 years until age 44 years and every 3-5 years thereafter. While a true negative genetic test is considered conclusive, some centers recommend interval flexible sigmoid-oscopy around the ages of 18, 25, 35, and then per standard American Cancer Society guidelines, if an at-risk individual tests negative.[21] If the genetic test was indeterminate, at-risk individuals should undergo screening sigmoidoscopy every year from 10 to 25 years of age, biannually from 26 to 35, and every 3 years from 36 to 50, and then per ACS guidelines.[21] After colectomy has been performed most authors recommend screening the retained rectum every 6 months or the intestinal pouch every 1-3 years.[22] Screening for AFAP falls under similar guidelines, although screening is usually initiated at a later date, includes upper endoscopy for the gastric polyposis, and requires total colonoscopy because of the predilection for proximal lesions.[1,7]

Screening for extracolonic malignancy has proven less fruitful. Unlike colorectal cancer screening, there has been no reported evidence that early screening or therapy has improved the prognosis of duodenal and periampullary adenomatous disease.[23] Nevertheless, screening for am-pullary tumors is also recommended with upper endos-copy every 1-3 years, beginning at age 20-25 years. Screening should be performed with both forward and side viewing scopes with random biopsies taken. In regard to other common extracolonic tumors, annual physical exam is recommended for thyroid disease.[21] Hepatoblastoma screening includes annual liver ultrasound with alpha-fetoprotein level until the age of 6 in patients diagnosed with FAP in infancy.[1]

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