Penna C P, Tiret E, Kartheuser A H, Olschwang S, Parc R
Service de Chirurgie Digestive, Hôpital Saint-Antoine, Paris.
Gastroenterol Clin Biol. 1992;16(3):210-4.
Familial adenomatous polyposis coli is a hereditary autosomal dominant disease which spontaneously and inevitably leads to degeneration of colorectal adenomas and requires preventive surgical treatment. The aim of this study was to evaluate the age of colorectal degeneration and the need for a screening technique in family members. Between 1983 and 1989, 141 patients were treated for familial adenomatous polyposis in our surgical center. Mean age at surgery was 32 years and 64 patients (45.4 percent) had a colorectal carcinoma. Thirty had an in situ tumor (mean age: 30 years) and 34 had an invasive adenocarcinoma (mean age: 45 years), 7 of whom died of their cancer. No colonic cancer was found in patients younger than 20. Thirty-eight percent of the patients under 40 years of age, 73 percent of the patients older than 40 years and 81 percent of those older than 50 had an adenocarcinoma. Fifty percent of the patients with carcinoma were younger than 40 years and 7 percent were less than 25 years old. Seventy-one patients were symptomatic at the time of operation (mean age: 40 years), 32 (45 percent) had a colonic cancer. In 70 patients, familial adenomatous polyposis was detected by screening (mean age: 24) and 2.8 percent had a colonic carcinoma. We conclude that the age-related risk of developing colonic carcinoma requires prophylactic surgery in asymptomatic patients before 20 years of age, and that routine familial screening would be of some benefit.
家族性腺瘤性息肉病是一种遗传性常染色体显性疾病,会自发且不可避免地导致结直肠腺瘤恶变,需要进行预防性手术治疗。本研究的目的是评估结直肠恶变的年龄以及家族成员中筛查技术的必要性。1983年至1989年间,我们外科中心对141例家族性腺瘤性息肉病患者进行了治疗。手术时的平均年龄为32岁,64例患者(45.4%)患有结直肠癌。30例为原位肿瘤(平均年龄:30岁),34例为浸润性腺癌(平均年龄:45岁),其中7例死于癌症。20岁以下患者未发现结肠癌。40岁以下患者中38%、40岁以上患者中73%以及50岁以上患者中81%患有腺癌。患癌患者中有50%年龄小于40岁,7%年龄小于25岁。71例患者在手术时有症状(平均年龄:40岁),32例(45%)患有结肠癌。70例患者通过筛查发现家族性腺瘤性息肉病(平均年龄:24岁),2.8%患有结肠癌。我们得出结论,与年龄相关的患结肠癌风险要求在20岁之前对无症状患者进行预防性手术,并且常规家族筛查会有一定益处。