Ⅰ型胸膜肺母细胞瘤的临床病理观察

目的探讨Ⅰ型胸膜肺母细胞瘤的临床病理特征、诊断和鉴别诊断，以期提高对该肿瘤的诊断水平。方法复习1例Ⅰ型胸膜肺母细胞瘤临床资料，并观察其组织学特征和免疫组化标记。结果临床以自发性气胸为首发症状。组织学特征为原始胚胎性的圆形或短梭形细胞，并向横纹肌肉瘤分化。结论Ⅰ型胸膜肺母细胞瘤是一种罕见的肿瘤，诊断主要依靠病理形态学及免疫组化，并应与先天性囊腺瘤、原始神经外胚叶肿瘤及胚胎性横纹肌肉瘤等鉴别。

Clinical Pathological Observation of Type Ⅰ Pleuropulmonary Blastoma

Objective To explore the clinical pathological features, diagnosis and differential diagnosis of type I pleuropulmonary blastoma （PPB）. Methods one case of type I PPB was reviewed and the histological and immunohistochemical features was examined. Results A girl of 26 months old presented with pneumothorax. The tumor consisted of primitive small cells,variety of differentiated embryonal rhabdomyosarcoma. Conclusion Type Ⅰ PPB is a rare neoplasm in children. Diagnosis is based on its morphology and immunohistochemistry. Differential diagnosis includes cystic adenomatoid malformation, pulmonary blastoma, primitive neuroectoural tumor（PNET）, embryonal rhabdomyosarcoma and other sarcomas.