Objectives: To explore the clinical feathers and prognosis of primary malignant pericardial mesothelioma (PPM) reported in inland China. Methods: We searched Chinese data bases and collected medical records of PPM from 1981 to 2015 for patients' clinical, imaging, pathological, diagnostic and therapeutic information to explore the possible impact factors for prognosis. Results: A total of 186 patients were enrolled including 122 (65.6％) male, the mean age was (40.9±14.5) years at the range of (2-79) years. There were 108/166 (65.1％) patients suffered from dyspnea which was the most common symptom, 17 (10.2％) were complicated with cardiac tamponade and 48 (28.9％) complicated with constrictive pericarditis. 65 patients were misdiagnosed and 40 (61.5％) of them were diagnosed for tubercular pericarditis. 94 patients died and 30 (31.9％) of them died during hospitalization. 86 patients were discharged and received the follow-up study at the median of 6 months, 64 (74.4％) of them died. Multivariate Cox regression analysis did not find the meaningful clinical, imaging or pathological parameters to predict patients' mortality. Conclusion: PPM is a rare and highly aggressive pericardial malignant tumor, mainly involving middle-age males. Pathological combining immunohistochemical examinations are helpful for PPM diagnosis. Effective therapy has been lacking and comprehensive individualized therapy may improve the patients' prognosis.%目的:探讨原发性恶性心包间皮瘤(PPM)的临床特点及转归.方法:检索中文数据库收集1981～2015年中国内地报道的PPM病例,收集患者的临床、影像、病理及诊断和治疗信息,探讨可能影响患者预后的指标.结果:共检索到186例患者,男性122例(65.6％),年龄2～79(40.9±14.5)岁.在有记录的患者中,最常见的症状是呼吸困难(108/166,65.1％),17例(10.2％)患者并发缩窄性心包炎,48例(28.9％)合并心脏填塞.65例误诊病例中,40例(61.5％)误诊为结核性心包炎.94例死亡病例中,30例(31.9％)为住院期间死亡,对86例出院患者中位随访6个月,64例(74.4％)患者死亡.以出院后死亡为终点事件,多因素Cox回归分析未发现对病死率有预测价值的临床、影像或病理学指标.结论:PPM是一种高度侵袭性心包原发恶性肿瘤,主要累及中壮年男性.病理学结合免疫组化分析有助于确定诊断.该病目前尚缺乏有效的治疗手段,个体化、综合治疗有可能改善预后.
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