【摘要】 目的探讨10例腹膜原发间皮源性恶性肿瘤的临床病理特点、免疫表型、诊断及鉴别诊断,提高对腹膜间皮源性恶性肿瘤的认识。方法对腹膜间皮源性恶性肿瘤的临床资料、病理形态、免疫组化特点进行回顾性分析,并复习相关文献。结果 6例为腹膜恶性间皮瘤,4例为第二苗勒系统浆液性癌。大部分病例均为为腹膜弥漫、多发病变;1例第二苗勒系统浆液性癌累及双侧卵巢及输卵管表面。两者各有1例罕见生长方式即局限于肠壁呈内生性生长并阻塞肠腔,肿瘤主要位于浆膜层,累及肌层、黏膜下层及黏膜层。镜下恶性间皮瘤为上皮样细胞及肉瘤样细胞混合型者2例,2例为上皮样细胞为主型,2例为肉瘤样细胞为主型;4例第二苗勒系统浆液性癌均为高级别。恶性腹膜间皮瘤中上皮样型细胞及肉瘤样型细胞均不同程度的表达Calretinin、AE1/AE3、vimentin、CK5/6、WT-1、HBME-1,两种类型细胞均不表达CD34、Ber-EP4、CEA、CD117、desmin、SMA、DOG1。第二苗勒系统浆液性癌细胞表达ER、P16、PAX-8、P53、WT-1,灶状表达CK7,不表达CK20、Desmin、CK5/6、Calretinin、HBME-1、D2-40、CEA、PR、S100、SMA等。结论腹膜恶性间皮瘤、第二苗勒系统浆液性癌均起源于腹膜间皮,二者有相似的生长方式及组织形态学;免疫组化复杂多样且有一定程度的相似,临床容易误诊。确诊主要依靠病理形态及免疫表型,且需与有类似生长方式或组织学形态的其他肿瘤相鉴别。更多还原

【Abstract】 Objective To investigate the clinicopathologic features, immune phenotype, diagnosis, differential diagnosis of 10 cases with primary peritoneal malignant tumor originated from mesothelioma. Methods Clinical data, pathological morphology and immune phenotype were summarized and analyzed in 10 cases with review of the related literatures. Results There were 6 cases of peritoneal malignant mesothelioma and 4 cases of serous carcinoma originated from Müllerian type. Most lesions of peritoneal were multifocal and diffuse. Lesions involved surface of bilateral ovaries and fallopian tube in one case of serous carcinoma originated from Müllerian type. Both of them had one case with rare growth way, which were limited to the intestinal wall to form endogenous tumor and block part of the intestinal lumen. The tumor were both mainly in the serous membrane layer involving the muscle layer, submucosa and mucous membrane layer. Microscopically, there were 2 cases of epithelioid cell type, 2 of sarcomatoid cell type and 2 mixed type in peritoneal malignant mesothelioma. 4 cases of serous carcinoma originated from Müllerian type were all high grade. The cases with the peritoneal malignant mesothelioma showed immunohistochemistry positive Calretinin, AE1/AE3, vimentin CK5/6 and WT-1, while CD34, Ber-EP4, CEA, CD117, desmin, SMA, DOG1 were negative. The cases with serous carcinoma originated from Müllerian type showed immunohistochemistry positive ER, PAX, P16, P53 and WT-1, while CK20, Desmin, CK5/6, Calretinin, HBME-1, D2-40, CEA, PR, S100 and SMA were negative. Conclusions Peritoneal malignant mesothelioma and serous carcinoma originated from Müllerian type are both originated from peritoneal mesothelioma. They have the similar growth way, histomorphology, immunohistochemical expression in some degree. They are easily misdiagnosed. The diagnosis of them depends on the combination of pathology morphology and immunohistochemistry. At the same time which should be distinguished with other malignant tumor with same growth way.更多还原