34例非阑尾来源腹膜假黏液瘤诊治分析

Diagnosis and treatment of pseudomyxoma peritonei of extra-appendiceal origin:analysis of 34 cases

目的:探讨非阑尾来源腹膜假黏液瘤(pseudomyxoma peritonei,PMP)的临床病理特征和诊疗经验。方法:回顾性分析2011年9月至2019年2月于航天中心医院采用肿瘤细胞减灭术(cytoreduction surgery,CRS)联合腹腔热灌注化疗(hyperthermic inter-peritoneal chemotherapy,HIPEC)治疗的34例非阑尾来源PMP患者的临床资料,总结其临床表现及影像学特征,同时利用Log-rank检验对相关因素进行生存分析。结果:本研究34例患者临床表现主要为腹胀(58.8%)和腹盆腔包块(52.9%);CRS+HIPEC治疗后主要并发症发生率为14.7%,在随访中9例患者死亡,1、3年生存率分别为69.6%、53.5%;单因素分析中,腹膜癌指数(peritonealcancer index,PCI)>20、未行灌注化疗以及非根治性手术是预后不良的显著危险因素,而性别、年龄、组织来源、病理类型等未体现出显著相关性。结论:非阑尾来源PMP无特异性临床表现,术前较难判断原发病灶,确诊需要依靠术后病理及免疫组织化学检测。但无论来源如何,均以腹腔内广泛肿瘤种植和局部浸润为主要临床表现,CRS+HIPEC是安全有效的治疗手段。

Objective:To investigate the clinicopathological features and treatment strategy of pseudomyxoma peritonei(PMP)of extra-appendiceal origin.Methods:Clinical data of 34 patients diagnosed with PMP of extra-appendiceal origin who were treated by cytoreduction surgery(CRS)and hyperthermic intraperitoneal chemotherapy(HIPEC)in the Aerospace Center Hospital from September 2011 to February 2019 were retrospectively analyzed.Clinical and imaging features were summarized and the Log-rank test was used for survival analysis.Results:The clinical manifestations of the 34 patients with PMP of extra-appendiceal origin were mainly abdominal distension(58.8%)and abdominal pelvic mass(52.9%),which are very similar to those of appendiceal PMP.The incidence of main complications after CRS and HIPEC was 14.7%.During the follow-up period of a median of 12 months(range 1-46 months),9 patients died,and the 1-and 3-year overall survival rates were 69.6%and 53.5%,respectively.In the univariate analysis,peritoneal cancer index(PCI)>20,no HIPEC,and non-radical surgery were significant risk factors for poor prognosis,while gender,age,origin,and pathological type did not show significant correlations.Conclusions:The clinical features of PMP of extra-appendiceal origin are not different to those of PMP originating from the appendix.It is difficult to ascertain the primary lesion before the operation;however,regardless of the origin,CRS combined with HIPEC is always a safe and effective treatment choice.