Table 6 -- Surveillance of Cancers associated with Familial Adenomatous Polyposis Syndromes and Its Variants

Syndrome Cancer Intervention Surveillance Recommendations Familial Adenomatous Polyposis (FAP) Colon Flexible sigmoidoscopy Annually, starting at puberty* (greater than 100 polyps) Periampullary/ Duodenum EGD with side-viewing scope Q1-4 years beginning age 20, yearly if adenomas are present   Thyroid Pancreas Hepatoblastoma Bone Skin TFT’s/thyroid scan** none Alpha-fetoprotein Physical Exam Physical Exam Annual Birth to age 5 Crail syndrome Brain Consider head CT/MRI Annual neurologic exam beginning age 10   Colon Same as FAP   I1307K APC mutation in Ashkenazi Jews Colon Colonoscopy Q2 years starting at age 35* Attenuated adenomatous coli Colon Colonoscopy Annually, starting during second decade after puberty *** (fewer than 100 polyps) Duodenal/Periampullary Upper endoscopy with side-viewing scope and biopsies Q1-4 years beginning age 20, yearly if adenomas are present

* Colectomy in teens or earlier if polyps become problematic: e.g. greater than 100 polyps, multiple polyps >1cm, or polyps with features of high-grade dysplasia or villous architecture, whichever comes first. ** TFT= Thyroid Function Tests *** Colectomy is considered treatment of choice once polyps have developed.