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Table 71-2

Hereditable (Autosomal Dominant)

Gastrointestinal Polyposis Syndromes
SyndromeDistribution of PolypsHistologic TypeMalignant PotentialAssociated Lesions
Familial adenomatous polyposisLarge intestineAdenomaCommonNone
Gardner's syndromeLarge and small intestinesAdenomaCommonOsteomas, fibromas, lipomas, epidermoid cysts, ampullary cancers, congenital hypertrophy of retinal pigment epithelium
Turcot's syndromeLarge intestineAdenomaCommonBrain tumors
Nonpolyposis syndrome (Lynch syndrome)Large intestine (often proximal)AdenomaCommonEndometrial and ovarian tumors
Peutz-Jeghers syndromeSmall and large intestines, stomachHamartomaRareMucocutaneous pigmentation; tumors of the ovary, breast, pancreas, endometrium
Juvenile polyposisLarge and small intestines, stomachHamartoma, rarely progressing to adenomaRareVarious congenital abnormalities