Cancer characteristics in Swedish families fulfilling criteria for hereditary nonpolyposis colorectal cancer
Cancer characteristics in Swedish families fulfilling criteria for hereditary nonpolyposis colorectal cancer
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DOI：
10.1053/j.gastro.2005.09.012
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发表时间：
2005-12-01
期刊：
影响因子：
29.4
通讯作者：
Hemminki, K
中科院分区：
文献类型：
--
作者：
Bermejo, JL;Eng, C;Hemminki, K
背景和目的：本研究量化了整个瑞典人口中符合阿姆斯特丹或贝塞斯达遗传性非息肉病性结直肠癌 (HNPCC) 标准的家庭的患病率，并调查了分类家庭中肿瘤与 HNPCC 相关的程度。方法：根据阿姆斯特丹和贝塞斯达标准对瑞典家族癌症数据库中至少有 4 代的家族（N = 566,877）进行分类。生存方法用于评估分类家族中癌症的风险、癌症患者的预后以及结直肠腺瘤和结直肠/子宫内膜腺癌后后续恶性肿瘤的风险。结果：贝塞斯达标准确定了 0.9% 的瑞典家庭和 11.2% 的结直肠癌患者。符合贝塞斯达标准的家庭显示结直肠癌、子宫内膜、小肠、卵巢、胃、胆管、肾盂和输尿管癌症的风险增加；贝塞斯达标准家庭成员患肺癌和宫颈癌的风险较低。根据贝塞斯达标准，输尿管、肾盂、胃癌、卵巢和结直肠癌（但子宫内膜癌除外）的预后优于非分类家族。结论：分类家族中的大多数恶性肿瘤反映了 HNPCC 的典型特征（与后续恶性肿瘤的相关性、加速的腺瘤-癌序列和更好的生存率）。本研究中提供的数据应有助于为符合 HNPCC 检测标准的家庭成员制定监测策略。
Background & Aims: The present study quantified the prevalence of families that fulfill the Amsterdam or Bethesda criteria for hereditary nonpolyposis colorectal cancer (HNPCC) in the whole Swedish population and investigated the extent to which tumors in the classified families are HNPCC-related. Methods: The families of the Swedish Family-Cancer Database with at least 4 generations (N = 566,877) were classified according to the Amsterdam and the Bethesda criteria. Survival methods were used to assess the risk of cancer in the classified families, the prognosis of cancer patients, and the risk of subsequent malignancies after colorectal adenomas and after colorectal/endometrial adenocarcinomas. Results: The Bethesda criteria identified 0.9% of all Swedish families and 11.2% of patients with colorectal cancer. Families that fulfilled the Bethesda criteria showed increased risks of cancer in the colorectum, endometrium, small bowel, ovary, stomach, bile ducts, renal pelvis, and ureter; members of Bethesda criteria families were at decreased risks of lung and cervical cancers. The prognosis of cancer in the ureter, renal pelvis, stomach, ovary, and colorectum, but not in the endometrium, was better in Bethesda criteria than in nonclassified families. Conclusions: Most malignancies in the classified families reflect typical features of HNPCC (association with subsequent malignancies, accelerated adenoma-carcinoma sequence, and better survival). The data presented in this study should help to define surveillance strategies for members of families that fulfill the criteria for HNPCC testing.