黑斑息肉综合征继发恶变五例诊疗研究  

Clinical diagnosis and treatment of five cases with malignant tumors associated with Peutz-Jeghers syndrome

作　　者：王国军[1] 郑智[1] 李培鑫[1] 秦胜旗[1] 王今[1] 李建设[1] 张忠涛[1] Wang Guojun;Zheng Zhi;Li Peixin;Qin Shengqi;Wang Jin;Li Jianshe;Zhang Zhongtao(Department of General Surgery,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China)

机构地区：[1]首都医科大学附属北京友谊医院普外科,100050

出　　处：《国际外科学杂志》2018年第10期669-673,共5页International Journal of Surgery

基　　金：国家科技支撑计划课题（2015BAII3B09）;首都医科大学基础-临床科研合作基金（17JL18）

摘　　要：目的探讨黑斑息肉综合征（PJS）继发恶变后的临床特点及诊治方法。方法回顾性分析2014年6月—2017年1月首都医科大学附属北京友谊医院诊治的5例PJS继发恶变患者的临床资料，采用电话、门诊及住院复查方式进行随访，随访以就诊日期为起点，以患者死亡为终点。观察其临床特点、治疗方式、术后生存情况，随访时间截止至2018年5月。结果PJS继发恶变患者临床表现缺乏特异性。5例患者中2例施行内镜切除，3例施行外科手术，5例患者术后均接受联合化疗，其中1例在外科手术后6个月因肿瘤进展死亡，其余4例患者截至2018年5月未见复发。结论PJS患者恶变息肉，可行内镜下切除；当息肉恶变至内镜下无法切除时，可行手术治疗，术后结合辅助化疗可改善远期预后。Objective To explore clinical characteristics, diagnosis and treatment method after Peutz-Jeghers Syndrome （PJS） secondary malignant.Methods The clinical date of five cases with malignant tumors associated with Peutz-Jeghers syndrome from June 2014 to January 2017 were analyzed retrospectively in Beijing Friendship Hospital, Capital Medical University. The patients were followed up by phone, outpatient service, and hospitalization. The starting point of the follow-up was the visit date. The patient′s death was the end point. The clinical and pathological features, therapy, and postoperative survival were observed. The follow-up deadline was May 2018.Results PJS secondary malignant patients lack clinical specificity. Two cases of five patients accepted endoscopic resection, three cases accepted surgery, and were treated with chemotherapy postoperatively, including 1 case died from tumor progression of 6 months after operation. Tumor recurrence was not found in the rest 4 cases till May 2018. Conclusions Part of the malignant polyp, endoscopic resection is feasible. When endoscopic resection is not feasible, operation treatment is needed; and postoperative adjuvant chemotherapy is needed to improve the long-term prognosis.

关 键 词：肿瘤 外科手术 诊断 治疗结果 黑斑息肉综合征 

分 类 号：R656[医药卫生—外科学]