遗传性家族性腺瘤型息肉病 (FAP)以遍及十二指肠和结肠发生数千百枚恶化前期的腺瘤型息肉为特征。FAP和大多数散发性结直肠肿瘤两者均起自结肠腺瘤型息肉病基因 (APC)的突变或丢失后。该肿瘤抑制基因胚系突变不仅引起肠息肉 ,也联系着疾病的种种肠外表现 ,包括可能致命的纤维性 Hereditary familial adenomatous polyposis (FAP) is characterized by tens of thousands of premalignant adenomatous polyps occurring throughout the duodenum and colon. Both FAP and most sporadic colorectal tumors arise from the mutation or loss of the colon adenomatous polyposis gene (APC). This tumor suppressor gene germline mutation causes not only intestinal polyps but also various parenteral manifestations of the disease, including potentially fatal fibrosis