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Familial Adenomatous Polyposis (FAP) (Classic FAP (A minimum number of…
Familial Adenomatous
Polyposis (FAP)
Familial Polyposis Syndromes
Most are due to germline mutations in tumor suppressor genes
Increased risk of intestinal and (in some syndromes) extra-intestinal cancers
Uncommon AD disorders.
AD syndrome Mutation in APC gene
Classic
FAP
A minimum number of 100 polyps
is required for diagnosis
Innumerable colonic adenomas carpet
the mucosal surface
Most polyps are
tubular adenomas
anywhere in the GI including
ampulla of Vater and stomach
100% lifetime risk of progression
to cancer at younger age
prophylactic colectomy is performed
Attenuated
FAP
Mainly in the proximal colon
Increased risk of colonic
adenocarcinoma in 50% of cases
Fewer polyps (average 30)
Gardner syndrome
Abnormal dentition
Fibromatosis the proliferation of fibroblasts Any organ
Epidermal inclusion cysts in skin
Multiple osteomas in the jaw
Intestinal polyps as classic FAP
presence of extraintestinal manifestations in
addition to the intestinal polyps (Difference)
Turcot syndrome
Adenomatous colonic polyposis as FAP
CNS tumors. (Difference)