家族性腺瘤样息肉病 ,是一种异型合子显性遗传的先天性疾患。本病多发于青春期 ,可伴发严重并发症及各种肠外表现 ,如Ｐ -Ｊ综合征 ,Ｇａｒｄｎｅｒ′ｓ综合征 ,视网膜色素沉着及全身表现。初期多为良性 ,最后不可避免地发展成恶性 ,文献报道癌变的发生最早在 17～ 19岁出现。 Familial adenomatous polyposis is a congenital disorder of heterotrophic dominant inheritance. The disease occurs in adolescence, may be associated with serious complications and various parenteral manifestations, such as P-J syndrome, Gardner’s syndrome, retinal pigmentation and systemic manifestations. Initially mostly benign, and finally inevitably develop into malignant, reported in the literature the earliest occurrence of cancer in 17 to 19 years old.