Vasen H F, den Hartog Jager F C, Menko F H, Nagengast F M
Foundation for the Detection of Hereditary Tumors, University Hospital, Leiden, The Netherlands.
Am J Med. 1989 Mar;86(3):278-81. doi: 10.1016/0002-9343(89)90296-9.
Identification of the hereditary non-polyposis colorectal carcinoma syndrome (HNPCC) is a basis for secondary prevention. The objectives of this study are to investigate the natural history of HNPCC and to assess the effect of screening.
Screening for colorectal carcinoma was performed in 22 HNPCC families (colonoscopy or double-contrast barium enema and sigmoidoscopy). The patients were subdivided into two groups. Group A comprised patients with colorectal cancer who were referred because they were symptomatic. Group B included family members of these patients who were found to have a colorectal lesion by screening. We compared these groups with respect to the stage of tumor growth.
Histologic examination of the tumors in Group A (87 patients) revealed Dukes A carcinomas in six patients, Dukes B carcinomas in 37, Dukes C carcinomas in 21, and Dukes D carcinomas in 10 patients (classification unknown in 13 patients). In Group B (20 patients), adenoma was found in 14 and carcinoma in six patients (Dukes A in two and Dukes B in four patients). A total of 93 patients, including those whose tumors were detected by screening, had a colorectal carcinoma. The age at diagnosis ranged from 24 to 81 years (mean age: 46 years). The location of the colonic tumors was proximal in 60 percent. Multiple primary tumors were found in 26 percent.
These results suggest that screening leads to the early detection of colorectal carcinomas and adenomas in asymptomatic members of HNPCC families. Screening should be initiated at the age of 20 and continued during the life of the individual. Careful examination of the right colon is indicated because of the frequent occurrence of tumors in the proximal colon. A subtotal colectomy is indicated at the time of diagnosis of the initial colon cancer because of the risk of multiple primary tumors.
遗传性非息肉病性结直肠癌综合征（HNPCC）的识别是二级预防的基础。本研究的目的是调查HNPCC的自然病史并评估筛查效果。
对22个HNPCC家族进行了大肠癌筛查（结肠镜检查或双重对比钡灌肠及乙状结肠镜检查）。患者被分为两组。A组包括因出现症状而转诊的大肠癌患者。B组包括这些患者的家庭成员，他们通过筛查发现有大肠病变。我们比较了两组肿瘤生长阶段。
对A组87例患者的肿瘤进行组织学检查，发现6例为Dukes A期癌，37例为Dukes B期癌，21例为Dukes C期癌，10例为Dukes D期癌（13例患者分类不明）。B组20例患者中，14例发现腺瘤，6例发现癌（2例为Dukes A期，4例为Dukes B期）。包括通过筛查发现肿瘤的患者在内，共有93例患者患有大肠癌。诊断年龄为24至81岁（平均年龄：46岁）。结肠肿瘤位于近端的占60%。26%的患者发现有多个原发性肿瘤。
这些结果表明，筛查可使HNPCC家族无症状成员的大肠癌和腺瘤得以早期发现。筛查应在20岁开始，并在个体一生中持续进行。由于近端结肠肿瘤的频繁发生，建议仔细检查右半结肠。由于存在多个原发性肿瘤的风险，在诊断出原发性结肠癌时建议行次全结肠切除术。
