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Table 72-2

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
MYH-associated polyposisLarge intestineAdenomaCommonNone
Nonpolyposis syndrome (Lynch's syndrome)Large intestine (often proximal)AdenomaCommonEndometrial and ovarian tumors (most frequently) gastric, genitourinary, pancreatic, biliary cancers (less frequently)
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