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目的总结家族性腺瘤性息肉病(FAP)的临床病理特点,探讨合理的诊断和治疗方案。方法回顾性分析总结了从1980年9月至2005年12月在中国协和医科大学肿瘤医院住院治疗的51例FAP患者的临床病理资料,其中男29例,女22例,年龄在17~67岁之间,平均36·6岁。结果本组51例患者中有家族史29例,可疑家族史7例,主要的临床表现为便血、腹泻、腹部疼痛或不适。首次入院时伴有同时性癌变的28例,癌变率54·9%(28/51),平均40·5岁;无癌变者23例,平均31·9岁,其中直肠和乙状结肠癌变占81·8%。全组病例除1例因直肠癌变固定无法切除行放射治疗外,其余50例均接受了不同形式的结直肠切除术。全组无手术死亡,并发症发生率8%。38例患者获得随访,随访率74·5%(38/51),随访时间6个月至25年。第1次手术后1次没复查的21例(41·2%),规律性复查的14例(27·5%)。术后8例发现残余结直肠癌变。目前全组已有17例死亡。结论FAP缺乏特异的临床表现,易癌变,对可疑病例应行结肠镜检查,确诊后应尽快治疗,手术是最主要的治疗手段,术式应根据具体病情个体化对待,术后应严密监测和随访。
Objective To summarize the clinicopathological features of familial adenomatous polyposis (FAP) and to explore the reasonable diagnosis and treatment. Methods The clinical data of 51 FAP patients hospitalized at Peking Union Medical College Cancer Hospital from September 1980 to December 2005 were retrospectively analyzed. There were 29 males and 22 females, aged 17-67 years Between an average of 36.6 years old. Results The group of 51 patients had a family history of 29 cases, 7 cases of suspected family history, the main clinical manifestations of blood in the stool, diarrhea, abdominal pain or discomfort. The first admission was accompanied by simultaneous canceration of 28 cases, the rate of canceration was 54.9% (28/51), average 40.5 years; 23 cases without canceration, an average of 31.9 years, of which rectal and sigmoid colon cancer accounted for 81 8%. All the patients in addition to a case of colorectal cancer due to unresectable radiotherapy, the remaining 50 patients underwent different forms of colorectal resection. The whole group died without surgery, the complication rate was 8%. Thirty-eight patients were followed up for 74.5% (38/51), followed up for 6 months to 25 years. There were 21 cases (41.2%) who did not reexamine after the first operation and 14 cases (27.5%) who regularly reviewed. Postoperative 8 cases found residual colorectal cancer. At present, 17 cases have been fatal in the whole group. Conclusion FAP lacks specific clinical manifestations and is susceptible to canceration. Colonoscopy should be performed on suspicious cases and treated as soon as possible after diagnosis. Surgery is the most important treatment. FAP should be treated according to the specific conditions and should be closely monitored after surgery Follow-up.