腹膜恶性间皮瘤7例临床病理分析

Peritoneal malignant mesothelioma:a clinicopathologic analysis of 7 cases

目的探讨腹膜恶性间皮瘤(PMM)的临床病理特征、免疫表型、鉴别诊断、治疗及预后。方法对7例PMM的临床资料、病理特点及免疫组化标记进行研究,并复习相关文献。结果 PMM好发于中老年人,平均年龄为61.5岁,临床主要表现为腹胀、腹痛、腹水、腹部包块等。镜下肿瘤组织形态多样,由上皮样细胞和梭形细胞2种类型组成,具有向上皮和肉瘤样双向分化的特点。免疫组化示calretinin(6例)、AE1/AE3(7例)、EMA(5例)、CK5/6(5例)、HBME-1(6例)、vimentin(7例)均(+),CEA均(-)。随访6～30个月,2例死亡,5例存活。结论 PMM对其确诊需结合临床特征、病理形态及免疫表型,根据肿瘤细胞异型程度、核分裂象多少和核的多形性,恶性间皮瘤可分为低度恶性和高度恶性。且需与腹腔原发或转移的其他肿瘤相鉴别。

Objective To investigate the clinicopathologic features, immunophenotype, differential diagnosis, therapy and prognosis of peritoneal malignant mesothelioma （PMM）. Methods Clinical data, pathological features and immunohistochemical markers were analyzed in seven patients with PMM with review of the related literatures. Results PMM occurred predominantly in middle-aged persons, with a median age of 61.5 years. Major clinical manifestations consisted of abdominal distention, abdominal pain, ascites and abdomen swelling. Microscopically, tumor had morphological diversity, which was composed of epithelioid cell and spindle cell with characteristics of epithelioid or sarcomatoid differentiation. Immunohistochemically, the tumor cells were positive for calretinin（6/7 ）, AE1/AE3 （7/7）, EMA （5/7）, CK5/6 （5/7）, HBME-1 （6/7）, vimentin （7/7）, but negative for CEA of all cases. 2 patients had died, and 5 patients survived by follow-up. Conclusions PMM is a peritoneal primary and rare tumor. Its diagnosis should depend on clinical features, pathological and immunohistochemistry. According to the cellulariry of neoplasm, the number of mitotic figures and nuclear heteromorphism, PMM can be divided into low grade and high grade malignancy. It needs to be distinguished from celiac primary or metastatic malignant tumors.