阑尾源性腹膜假黏液瘤单中心诊疗经验

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。

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

  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.