摘要目的 探讨弥漫性肺脑膜上皮瘤病的临床病理学特征、免疫表型、诊断及鉴别诊断.方法 回顾性分析广东省人民医院2016年1月至2018年5月收集的3例肺弥漫性肺脑膜上皮瘤病的临床资料、病理形态学特征及免疫表型特点,并复习相关文献.结果 3例弥漫性肺脑膜上皮瘤病患者均为老年女性,年龄分别为53、69、74岁.病变弥漫累及双肺,形态学表现为多发的脑膜上皮样微结节,细胞形态温和,类似于脑膜上皮,大小一致,核卵圆形,可见核沟,胞质丰富、嗜酸性,呈旋涡状或巢状排列;核分裂象罕见.免疫组织化学显示3例波形蛋白、上皮细胞膜抗原、孕激素受体均弥漫强阳性;3例CAM5.2、嗜铬粒素A、突触素、细胞角蛋白7和甲状腺转录因子1均阴性.结论 弥漫性肺脑膜上皮瘤病非常罕见,病程缓慢,预后好,应避免误诊为其他肺原发性疾病或转移性的恶性肿瘤,防止造成不适当的治疗.

abstractsObjective To study the clinicopathological characteristics, immunophenotype, pathologic diagnosis and differential diagnosis of diffuse pulmonary meningotheliomatosis (DPM). Methods The clinical data, histological features and immunohistochemical results of three cases of DPM collected from January 2016 to May 2018 at Guangdong Provincial People′s Hospital were analyzed, and the relevant literature reviewed. Results The three DPM patients age was 53, 69 and 74 years. The lesions consisted of multiple minute pulmonary meningothelial?like nodules profusely involving the lungs. Microscopically, they showed typical morphology, characterized by medium?sized elongated tumor cells resembling meningothelial cells with whorled/nested clusters. The cells were uniform and had abundant eosinophilic cytoplasm with oval nuclei with delicate chromatin. Immunohistochemical staining showed tumor cells were diffusely and strongly positive for EMA, vimentin and PR, but were negative for CAM5.2, CgA, Syn, CK7 and TTF1. Conclusions DPM is extremely rare, and may be confused with primary interstitial pulmonary processes and metastatic malignant tumors of lung. Recognition of this entity is essential for pathologists to avoid misdiagnosis and unnecessary treatment.