儿童胸膜肺母细胞瘤一例诊治分析

摘要

目的 探讨胸膜肺母细胞瘤(pleuropulmonary blastoma,PPB)的临床特点及诊断要点.方法 回顾分析1例PPB的临床资料.结果 患儿因肠套叠入院常规行胸部X线检查发现右下肺类圆形气性密度影,其边缘清晰,内未见液平面.本例临床无明显呼吸道症状,CT检查见右下肺透亮度增加,有一薄壁气囊,囊内见薄分隔,诊断先天性孤立性肺囊肿.2年后复查病灶增大,行手术治疗,术中见右肺下叶囊实性肿物,行右肺下叶切除术,术后病理诊断为PPB.结论 PPB临床罕见,无症状者需密切随访,确诊后应及时手术并辅以化疗、放疗.%Objective To investigate the clinical features and main points of diagnosis of pleuropumonary blastoma (PPB).Methods The clinical data of a child with PPB were retrospectively analyzed.Results The patient was admitted to hospital for intussusception.Routine chest X-ray examination showed round shadow as gas density in the right lower lung.Shadow edge was clear with no liquid surface inside.No obvious respiratory symptoms were found in the patient.Further CT examination showed increased brightness in the right lower lung.A thin-walled balloon separated by a thin capsule was found in the right lower lung, which was called congenital solitary pulmonary cyst.Enlarged lesions were found 2 years later.The patient accepted right lower lobectomy, and a cystoid and parenchymatous goiter was found in the right lower lung.Postoperatively pathological diagnosis was PPB.Conclusion PPB was rare in clinical practice and asymptomatic patient should be closely followed up.Surgery combined with chemotherapy and radiotherapy should be performed immediately after diagnosis.