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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 summarize the experience of diagnosis and treatment of familial adenomatous polyposis (FAP). Methods The clinical and pathological data of 12 cases of FAP were retrospectively analyzed. The results were 5 males and 7 females, with an average age of 32 years. Benign in 5 cases, an average of 25 years old. Malignant 7 cases, an average of 37 years old. Eight cases had a family history, and 7 of the 4 families died of colorectal cancer. In the 2 to 20 years of history, the number of polyps was >100, with a maximum of 3 to 7/cm~2, and 7 cases of canceration. All the patients underwent total colectomy, and ileostomy, ileal pouch and sphincter preservation were used. All those who did not have cancer survived. There were 3 cases of cancerous brain, liver or celiac metastases within half a year after surgery. Conclusions FAP is susceptible to carcinogenesis and early onset of cancer, and it should strive for total colorectal resection before carcinogenesis. Surgery to rectal mucosa stripping, ileal storage bag, ileal anal anastomosis is preferred.