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目的探讨小儿胸膜肺母细胞瘤(pleuro_pulmonaryblastoma,PPB)临床及病理学特征和早期诊治的可行性。方法对1例长期反复呼吸道感染、X线片呈肺野巨大圆形软组织肿块的患儿行肺叶切除术、病理学常规检查和免疫组织化学检查等,明确诊断为PPB,结合文献复习进行分析讨论。结果2岁女孩肺内病变抗炎治疗无效,无呼吸困难、贫血、消瘦等表现,手术见左上肺尖后段囊实性孤立性、境界清楚肿块,组织结构见分化较差的小圆细胞、梭形细胞,间质成分含横纹肌母细胞、成熟和/或不成熟软骨小岛,腺管分化良好。免疫组化标记检查确诊为PPB。结论小儿肺部肿瘤病理学检查出现间胚叶组织和疑似上皮细胞且免疫组化标记呈阳性表达时,可诊断为胸膜肺母细胞瘤。
Objective To investigate the clinical and pathological features of pleuro_pulmonary blastoma (PPB) in children and the feasibility of early diagnosis and treatment. Methods A case of long-term recurrent respiratory tract infection, X-ray showed a large pulmonary soft tissue mass lung lobectomy, routine examination and immunohistochemical examination of pathology, a clear diagnosis of PPB, combined with the literature review to discuss the discussion . Results The 2-year-old girl had no pulmonary dysfunction, no dyspnea, anemia, weight loss and so on. The surgery showed the solitary cystic solitary superiority of the left upper extremity apex and the clear boundary of the body. The poorly differentiated small round cells were found in the tissue structure, Spindle cells, stromal components with striated muscle cells, mature and / or immature cartilaginous islands, well differentiated duct. Immunohistochemical markers confirmed PPB. Conclusion Pulmonary pneumocytoma can be diagnosed when pathological examination of the lung tumor shows mesodermal tissue and suspected epithelial cells with positive immunohistochemical staining.