摘要
目的总结家族性腺瘤性息肉病(FAP)诊断治疗经验。方法回顾分析12例 FAP 的临床病理资料。结果男5例,女7例,平均32岁。良性5例,平均25岁。恶性7例,平均37岁。8例有家族史,其中4个家系中有7人死于大肠癌。病史2～20年,息肉数均>100个,最多达3～7个/cm^2,7例癌变。全部病例均施行了全结肠切除,并分别采用回肠造口、回肠储袋及保肛手术。未癌变者均存活。癌变者有3例术后半年内出现脑、肝或腹腔转移。结论 FAP 易癌变,且癌变发生早,应争取在癌变前行结直肠全切除。手术以直肠粘膜剥脱,回肠储袋,回肠肛管吻合术为首选。
Objective To sum up experience with diagnosis and therapy of familial adenomatous polyposis (FAP).Methods Twelve patients with FAP who had undergone total colectomy were analyzed retrospective- ly.Results This series comprises five benign cases and seven malignant female eases.Average age at opera- don was 32 (range 10～57)years,25 years in benign cases and 37 years in malignant cases.Familial in- herited histories were found in 8 cases.History last 2～20 years.The number of adenomatous polyposis was more than 100.Seven cases were eanceration.All patients underwent total colectomy,ileostomy or anus- preserving surgery with or without ileal pouch.Patients without eaneeration are survived,but 3 patients with canceration developed metastasis of liver,brain and abdomen during six months after surgery.Conclusion FAP will progress to cancer if not removed.Total colectomy or proctoclectomy should be performed before cancelation did not occurred.Total colectomy,preserving muscular sheath of rectum following mucosecto- my,ileal pouch-anal anastomosis may be the operation of choice for FAP.
