婴儿胸膜肺母细胞瘤临床病理观察  

Clinicopatholoɡic features of infant pleuropulmonary blastoma

作　　者：徐升[1,2] 刘光[1] 王瑾[1] 李琳[1] 

机构地区：[1]北京军区总医院病理科,北京100700 [2]安徽医科大学

出　　处：《诊断病理学杂志》2012年第1期19-22,25,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨胸膜肺母细胞瘤临床病理学特点及鉴别诊断。方法对1例胸膜肺母细胞瘤进行组织病理学观察并行免疫组化标记,同时复习相关文献。结果患儿男性,4个月。咳喘伴紫绀3天。巨检:右肺肿物,组织大小9 cm×6 cm×4 cm,切面灰白色,实性、质软;肿物与肺界限尚清,并占据大部分肺。镜检:肿瘤主要由原始胚胎性的圆形或卵圆形细胞组成,细胞中等大小,胞质少,核仁清,核分裂易见;部分区域主要由梭形及短梭形细胞构成的梭形细胞肉瘤或恶性纤维组织细胞瘤结构区,其间可见分化尚好的软骨岛或软骨结节。免疫组化:瘤细胞vimentin(+),软骨结节S-100(+),残留上皮成分CK(AE1/AE3)、EMA和TTF-1(+),NSE局灶(+),间质细胞SMA局灶少许(+),CD99和bcl-2弱(+),desmin、myoglobin、calretinin、calponin、FLI、MyoD-1和CD34(-)。结论胸膜肺母细胞瘤较为罕见,但恶性程度相对较高,组织病理学最典型表现为原始恶性小细胞聚集。临床上需要与肺母细胞瘤、肉瘤样间皮瘤、胚胎性横纹肌肉瘤、滑膜肉瘤、原始神经外胚叶肿瘤等相鉴别。Objective To explore the significance of clinicopathological features and differential diagnosis of pleuropulmonary blastoma.Methods We observed 1 case of pleuralpulmonary blastoma on the histopathological and immunohistochemical features,with review of the literature.Results The patient was a 4-month-old boy with cough and cyanosis for 3 days.Gross examination showed that the size of the right lung and tumor tissue was 9 cm×6 cm×4 cm with gray and soft solid of cutting surface.Tumor and its boundaries were clear and occupied most of the lung.Microscopically tumors mainly composed of embryonal primitive round or oval cells.These cells were medium in size with less cytoplasm,clear nucleolus,and mitosis was easily seen.Some areas had characteristics of spindle cell sarcoma or malignant fibrous histiocytoma that was mainly composed of spindle and short spindle cells.Immunohistochemical studies showed that tumor cells were positive for vimentin and local positive for SMA,the nodule of cartilage were positive for S-100.The tumor cells were negative for CK（AE1/AE3）,EMA,CD99,desmin,calretinin,calponin,FLI and Myo-D1.Conclusion Pleuralpulmonary blastoma is a relatively rare but highly malignant tumor.The most typical manifestation of pathology is the original malignant small cell aggregates.For early diagnosis and treatment,pleuralpulmonary blastoma needs to be differentiated from pulmonary blastoma,sarcomatoid mesothelioma,embryonal rhabdomyosarcoma,synovial fibroids,and primitive neuroectodermal tumor and so on.

关 键 词：胸膜肺母细胞瘤 病理诊断 鉴别诊断 免疫组化 

分 类 号：R734.3[医药卫生—肿瘤]