目的 探讨小儿胸膜肺母细胞瘤临床、病理学特征及与肺母细胞瘤的鉴别要点.方法 对4例小儿胸膜肺母细胞瘤进行组织形态学和免疫组织化学检查,结合文献复习进行分析并随访.结果 其中2例首发症状均为咳嗽,呼吸困难.年龄分别为8个月、4岁,胸片均为肺旁囊实性肿块.1例3岁因感冒摄片发现左上肺囊性占位.另1例出生后即出现气促,胸片见右肺占位.镜下可见原始的恶性小细胞表面被覆呼吸道上皮呈多囊性或实性结构,另一种成分为梭形细胞,除纤维肉瘤特点外,还向横纹肌肉瘤等分化.免疫组化:恶性小细胞未见上皮抗体表达;梭形细胞desmin、vimentin、MSA呈阳性表达.结论 小儿胸膜肺母细胞瘤是一种极为罕见的恶性肿瘤应与肺母细胞瘤等鉴别.%Purpose To explore the clinicopathologic features and differential diagnosis of pleuropulmonary blastoma( PPB ). Methods Four cases of PPB in children were reported with light microscopic observation and immunohistochemical study. The literature was reviewed and follow-up conducted. Results The age of two cases was 8 months and 4 years. The first symptom was cough and dyspnea. Chest radiogram showed cyst and solid formation in the lung. One case was 3 years old, to see the doctor for cold, and chest radiogram showed a cystic lesion in the left upper lobe. The other case was dyspnea after born. Chest radiogram showed a solid mass in the right lung. The tumor consisted of primitive small cells, and a variety of differentiated and/or anaplastic spindle and sarcomatous elements including fibrosarcoma and embryonal rhabdomyosarcoma. Immunohistochemically, epithelial markers are negative in malignant small cells, and the spindle cells were positive for desmin,vimentin and MSA. Conclusions PPB is a rare malignant tumor in children and should be distinguished from pulmonary blastoma.
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