阑尾源性腹膜假黏液瘤单中心诊疗经验

Management of pseudomyxoma peritonei originating from the appendix:a single-center experience

目的:总结细胞减灭术加腹腔热灌注化疗(cytoreductive surgery&hyperthermic intraperitoneal chemotherapy,CRS+HIPEC)治疗阑尾源性腹膜假黏液瘤(pseudomyxoma peritonei,PMP)的单中心诊疗经验。方法:回顾性分析2012年1月至2018年12月于航天中心医院收治,病理证实为阑尾源性PMP并经CRS+HIPEC治疗604例患者的临床数据,进行统计学分析。结果:604例患者经历621次CRS+HIPEC治疗,平均年龄56.7岁,其中女性364例(60.3%),男性240例(39.7%),平均腹膜癌指数(peritoneal cancer index,PCI)为25.7。28.5%(172/604)的患者完全减瘤(CCR 0/1)。3~4级不良事件发生率为21.7%(131/604),围手术期死亡率为0.7%(4/604),术后5年生存率为53.6%。高级别病理类型、不完全减瘤(CCR 2/3)、PCI>20、3~4级不良事件是PMP患者预后不良的独立危险因素。结论:阑尾源性PMP临床罕见,治疗方法特殊,对于怀疑或确诊PMP的患者,尽早行规范CRS+HIPEC治疗,有望取得良好的预后。

Objective:To analyze the clinical outcomes of pseudomyxoma peritonei(PMP)originating from the appendix following cytoreductive surgery(CRS)and hyperthermic intraperitoneal chemotherapy(HIPEC).Methods:A retrospective study was carried out by analyzing clinical data of patients who diagnosed with PMP originating from appendix and had undergone CRS and HIPEC between January 2012 and December 2018.Results:A total of 604 patients had undergone 621 CRS and HIPEC procedures.The average age of the patients was 56.7 years.Of the patients,364(60.3%)were female and 240(39.7%)were male.The median peritoneal carcinomatosis index(PCI)was 25.7.Of the patients,28.5%(172/604)had undergone complete cytoreduction(CCR 0/1).In this study,the incidence of grade 3-4 adverse events was 21.7%(131/604),and the perioperative mortality rate was 0.7%(4/604).For the entire cohort,the 5-year survival rate was 53.6%.High-grade pathological subtype CCR 2/3,PCI>20,and grade 3-4 adverse events were independent predictors of a poor overall survival.Conclusions:Appendix-derived PMP is rare,and its treatment should be considered carefully.For patients who are suspected or are confirmed as PMP,early standardized CRS and HIPEC are related to a better prognosis.