家族性腺瘤性息肉病的诊断与治疗

目的 总结家族性腺瘤性息肉病（ＦＡＰ）的诊断和治疗经验。方法 回顾性分析１３例ＦＡＰ的临床病理资料。结果 本组１３例患者,男５例,女８例,平均年龄３１．５岁。其中良性５例,平均年龄２５岁;癌变８例,平均年龄３６岁。８例有家族史者源于６个家系,３代中共发现１９例患者其中４个家系中有７例２死于大肠癌。本组患者病史２－２０年,每例息肉数均超过１００个。最多达３－９个／ｃｍ＾２。全部病例均施行了全结肠切除

Diagnosis and treatment of familial adenomatous polyposis

Objective To summarize the experience with diagnosis and therapy of familial adenomatous polyposis(FAP).Methods Thirteen patients with FAP who had undergone total colectomy were analyzed retrospectively. ResultsBZIn 5 patients with benign and 8 patients with malignant adenomatous polyposis,their average age at operation was 315 years (range 1057), 25 years in benign patients and 36 years in malignant patients. Familial inherited histories were found in 8 patients in 6 families. Nineteen patients were determined in 6 families in 3 generations. Seven familial members died of colorectal cancer in 4 families. History lasted 220 years. The number of adenomatous polyposis was more than 100. All patients underwent total colectomy, ileostomy or anuspreserving surgery with or without ileal pouch. All patients without canceration survived. The longest one exceeded 25 years, but 3/8 patients with canceration developed metastasis of liver, brain and abdomen within 6 months after surgery. Conclusions FAP will turn to be cancer if not removed. Total colectomy or proctocolectomy should be performed before canceration. Total colectomy, preserving muscular sheath of the rectum after mucosectomy,ileal pouchanal anastomosis may be the operative choice for FAP.