遗传性非息肉性结直肠癌(HNPCC)也称癌性家族症侯群(CFS),被视为结直肠癌最常见的危险因素,占所有结直肠癌的4％～6％。在结直肠之外的肿瘤中与CFS关系密切的是胃癌和子宫内膜癌。本文分析芬兰40个CFS家族的18例胰胆管癌病人,以期发现胆管癌的起源并评价其与遗传特征的关系。 40个CFS家族,患肿瘤总人数315人,男174例,女141例。患原发性胰胆管癌18人,其中14例病人有完整的病史及组织学诊断资料。确诊 Hereditary non-polyposis colorectal cancer (HNPCC), also known as cancerous family syndrome (CFS), is considered the most common risk factor for colorectal cancer and accounts for 4% to 6% of all colorectal cancers. Closely related to CFS in colorectal tumors is gastric cancer and endometrial cancer. This paper analyzes 18 cases of cholangiocarcinoma of the pancreas in 40 CFS families in Finland in order to discover the origin of cholangiocarcinoma and evaluate its relationship with genetic characteristics. In 40 CFS families, there were 315 total tumors, 174 males and 141 females. There were 18 patients with primary pancreatic and cholangiocarcinoma, 14 of whom had complete history and histological diagnosis. Confirmed