小儿胸膜肺母细胞瘤的诊断与治疗分析

目的初步探讨小儿胸膜肺母细胞瘤的临床特征、诊断与治疗方法。方法回顾性分析上海市儿童医院2013年10月至2017年6月收治的6例胸膜肺母细胞瘤患儿临床资料,其中男3例,女3例;年龄范围1岁2个月至10岁。5例患儿行肿瘤大部分切除(其中部分肺叶切除术1例,肺叶修补术4例),1例行肿瘤完整切除。6例均获得有效随访,随访时间1~34个月。结果 6例患儿中5例首诊被误诊,其中3例首诊为肺炎,1例首诊为胸腔积液,1例首诊为先天性肺囊性腺瘤样畸形。6例患儿随访结果分别为:临床痊愈1例;带瘤生存1例(目前正规接受化疗中);术后1个月死亡3例;术后2个月死亡1例。结论小儿胸膜肺母细胞瘤临床表现无特异性,Ⅰ型胸膜肺母细胞瘤易误诊为先天性肺囊性腺瘤样畸形,术后预后相对理想;早期发现、早期手术、后续正规化疗是治疗的主要方法。

Diagnosis and treatment of pleuropulmonary blastoma in children

Objective To explore the clinical characteristics,diagnosis and treatment of pleuropulmonary blastoma in children.Methods The clinical data were retrospectively analyzed for 6 hospitalized children with pleuropulmonary blastoma from October 2013 to June 2017.There were 3 boys and 3 girls with an age range of 14-120 months.There were partial lobectomy(n=1) and lobectomy(n=4).The follow-up period was 1 to 34 months.Results Five of them were initially misdiagnosed.The preliminary diagnosis was pneumonia(n=3),pleural effusion(n=1) and congenital cystic adenomatoid malformation(n=1).The follow-up outcomes were clinical recovery(n=1),survived with tumor & currently on regular chemotherapy(n=1) and death within 1 month post-operation(n=3).One patient relapsed after 1 month,was re-operated and died on the surgical day.Another child died within 2 month post-operation.Conclusion Due to non-specific clinical manifestations,pediatric pleuropulmonary blastoma is often misdiagnosed as congenital cystic adenomatoid malformation.Its postoperative prognosis is relatively decent.The treatment mainstays are an early discovery,an early operation and subsequent formal chemotherapy.