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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 investigate the clinicopathological features and differential diagnosis of pleuropulmonary blastoma. Methods One case of pleuropulmonary blastoma was observed by histopathology and immunohistochemically, and the related literatures were reviewed. Results children with male, 4 months. Cough and cyanosis with 3 days. The huge examination: the right lung tumor, tissue size 9 cm × 6 cm × 4 cm, cut gray, solid, soft; tumor and lung boundaries are clear, and occupy most of the lungs. Microscopic examination: The tumor mainly consists of primitive embryonic round or oval cells, cells of medium size, less cytoplasm, nucleolus clear, mitotic easy to see; part of the area mainly by spindle and short spindle cells composed of spindle cells Sarcoma or malignant fibrous histiocytoma structural area, during which differentiation is still good cartilage island or cartilage nodules. Immunohistochemistry showed that vimentin (+), cartilaginous nodule S-100 (+), residual epithelial component CK (AE1 / AE3), EMA and TTF- SMA focal a little (+), CD99 and bcl-2 weak (+), desmin, myoglobin, calretinin, calponin, FLI, MyoD-1 and CD34 (-). Conclusions Pleuropulmonary blastoma is relatively rare, but its malignancy is relatively high. The most typical histopathological manifestations are primitive malignant small cell aggregates. Clinical needs and pulmonary blastoma, sarcomatoid mesothelioma, embryonal rhabdomyosarcoma, synovial sarcoma, primitive neuroectodermal tumors, phase identification.