家族性腺瘤性息肉病47例临床分析  

Clinical analysis of 47 cases of familial adenomatous polyposis

作　　者：胡平[1] 汤琪云 Ping Hu;Qi-Yun Tang(Department of Gastroenterology,the First Affiliated Hospital of Nanjing Medical University;Department of General Medicine,the First Affiliated Hospital of Nanjing Medical University Nanjing 210029,Jiangsu Province,China)

机构地区：[1]南京医科大学第一附属医院消化内科,江苏省南京市210029 [2]南京医科大学第一附属医院全科医学科,江苏省南京市210029

出　　处：《世界华人消化杂志》2018年第21期1313-1319,共7页World Chinese Journal of Digestology

摘　　要：目的探讨家族性腺瘤性息肉病(familial adenomatous polyposis,FAP)的临床表现、病理特征、癌变危险因素及治疗方法 .方法收集江苏省人民医院47例家族性腺瘤性息肉病患者的临床资料,回顾分析其临床表现、病理特征、癌变情况及治疗方法 .结果 47例患者中29例患者表现为黑便或便血,22例发生大便性状改变,腹痛16例,腹胀11例,9例患者伴发胃息肉,4例伴发十二指肠息肉,2例术后发生硬纤维瘤.病理结果以管状腺瘤及绒毛管状腺瘤为主,少数表现为增生性息肉或幼年性息肉.16例患者发生癌变,发现年龄≥40岁及腺瘤直径>2 cm者与40岁以下及腺瘤小于2 cm的患者癌变差异有统计学意义(P<0.05),年龄(OR=1.084,95%CI:1.016-1.157)及腺瘤大小(OR=10.264,95%CI:1.526-69.035)为影响FAP癌变的危险因素.所有患者均行结直肠切除或内镜下治疗,4例癌变患者术后辅以化疗.结论家族性腺瘤性息肉病以结直肠生长大量息肉为主要特征,可伴有一系列肠外表现.病理表现以管状腺瘤及绒毛管状腺瘤为主.发现年龄及腺瘤大小为影响FAP癌变的危险因素.预防性结直肠切除和内镜下监测仍然是目前治疗FAP的主要措施.AIM To investigate the clinical manifestations, pathologicalfeatures, potential risk factors for cancerization, and therapeutics of familial adenomatous polyposis（FAP）. METHODS Forty-seven cases of FAP treated at Jiangsu Provincial Hospital were collected. The clinical data including clinical symptoms, pathological features and therapies of the 47 patients were retrospectively analyzed. RESULTS Of the 47 patients, 29 complained of melena or bloody stools and 22 had changes of character of stool; 16 had abdominal pain, 11 had abdominal bloating, 9 had gastric polyps, and 4 had duodenal polyps; 2 suffered from desmoid tumors after proctocolectomy. Main pathological features included tubular adenoma and tubulovillous adenoma, and a few manifested as hyperplastic polyps or juvenile polyps. Sixteen patients were diagnosed with colorectal cancer and there was a significantly higher canceration rate in patients who were older than 40 years or whose polyps were larger than 2 cm in diameter（P〈0.05）. Age（OR = 1.084, 95%CI： 1.016-1.157） and the size of adenoma（OR = 10.264, 95%CI： 1.526-69.035） were potential risk factors for FAP. All patients underwent colorectal resection or endoscopic treatment, and four patients with colorectal cancer received adjuvant chemotherapy after surgical operation.CONCLUSION FAP is characterized by the formation of hundreds of colorectal polyps, which is accompanied by a series of extra-intestinal manifestations. Tubular adenoma and tubulovillous adenoma are the main pathological types. Age and the size of adenoma are potential risk factors for adenoma canceration. Prophylactic colorectal resection and endoscopic surveillance remain the main therapies for FAP at present.

关 键 词：家族性腺瘤性息肉病 结直肠癌 临床表现 治疗 

分 类 号：R730.5[医药卫生—肿瘤]