腹膜假黏液瘤的临床病理特征和预后分析

The clinical and pathological characteristics and prognosis of pseudomyxoma peritonei

目的探讨腹膜假黏液瘤的临床病理特点和预后。方法回顾我院33例腹膜假黏液瘤患者的临床资料,对病理组织重新阅片和分型,对随访资料进行生存分析。结果33例患者中男女之比为1∶2,平均年龄50岁,发病至确诊中位间期为12个月,误诊率高达84.8%。腹胀、腹部包块、腹围增大为主要临床表现。肠道肿瘤标志物、影像学和腹腔穿刺等辅助检查常有阳性发现。减瘤手术为主要的治疗方法,化疗是主要的辅助疗法。病理分型中良性66.7%,交界性21.2%,恶性12.1%。中位生存期为70个月。病理类型和化疗对生存时间具有影响。结论腹膜假黏液瘤具有独特的临床病理特点,肠道肿瘤标志物、影像学和腹腔穿刺对诊断具有提示意义,目前的治疗方法有待于完善,病理类型和化疗是预后的影响因素,应对其加强认识以降低误诊率。

Objective To investigate the clinical and pathological characteristics, treatment measures and prognosis of pseudomyxoma peritonei （PMP）. Methods The clinical records and follow-up data of the patients with PMP were retrospectively analyzed. Survival analysis （ Kaplan-Meier method and Cox regression） was used to investigate the prognosis. Results Thirty-three patients （twenty-two women, eleven men） have a median interval between onset and definite diagnosis of 12 months with an average age of 50. The main clinical presentation includes bloating, abdominal mass and abdominal distention. A delay in diagnosis is common and many patients are labeled as other diseases （84. 8% ）. Positive results were often found in the assistant examinations such as erythrocyte sedimentation rate, gastrointestinal tumor markers （carcinoembryonic antigen, carcinomatous antigen 19-9 and so on）, imaging（ B ultrasound and computer tomography） and abdominal paracentesis which was usually unsuccessful. The most frequently practiced approach is debulking surgery with the main assistant means of chemotherapy which was lack of the uniform rationale. Benign type of pathological samples accounts for 66. 7% of all, while mid-type 21.2% and malignant 12. 1%. The general median survival time is 70 months with a follow-up rate of 79%. The pathological type and chemotherapy are main factors which influence the cumulative survival rate （P 〈 0. 05 ）. Up to the end of 2004, sixteen patients died of various complications, multiple organ failure and severe infection, while ten patients survival and seven patients lost. Conclusions PMP is a rare condition of borderline malignancy with its unique clinical and pathological characteristics. Gastrointestinal tumor markers, imaging and abdominal paracentesis are very important to the diagnosis. The existing treatment measures need to be improved further. The pathological type and chemotherapy are main factors which influence the prognosis.