Gardner E J, Burt R W, Freston J W
West J Med. 1980 Jun;132(6):488-99.
Adenomas and hamartomas, two genetically transmitted histologic types of gastrointestinal polyposis, are associated in syndromes with extragastrointestinal manifestations. Adenomas that predispose to adenocarcinoma are basic to familial polyposis coli, the Gardner syndrome and the Turcot syndrome. Gastrointestinal polyps and extragastrointestinal lesions serve as a warning, providing time for diagnosis and treatment of adenomas to prevent their malignant transformation in patients and their relatives. Hamartomas with no malignancy potential, but having a tendency toward bleeding and bowel obstruction, are associated with the Peutz-Jeghers syndrome, juvenile polyposis, multiple hamartoma syndrome, basal-cell nevus syndrome and the Cronkhite-Canada syndrome. Most of these lesions and syndromes follow the inheritance pattern of a single autosomal dominant gene.
腺瘤和错构瘤是胃肠道息肉病的两种遗传组织学类型，在伴有胃肠道外表现的综合征中出现。易引发腺癌的腺瘤是家族性结肠息肉病、加德纳综合征和图尔科特综合征的基础。胃肠道息肉和胃肠道外病变起到警示作用，为腺瘤的诊断和治疗提供时间，以防止患者及其亲属发生恶变。无恶变潜能但有出血和肠梗阻倾向的错构瘤与黑斑息肉综合征、幼年性息肉病、多发性错构瘤综合征、基底细胞痣综合征和克-卡综合征相关。这些病变和综合征大多遵循单一常染色体显性基因的遗传模式。
