Li Xiaobing,Zhang Rufang,Shen Li,et al.Diagnosis and treatment of pleuropulmonary blastoma in children[J].Journal of Clinical Pediatric Surgery,2020,19(02):166-170.[doi:10.3969/j.issn.1671-6353.2020.02.015]
小儿胸膜肺母细胞瘤的诊断与治疗分析
- Title:
- Diagnosis and treatment of pleuropulmonary blastoma in children
- 关键词:
- 胸膜肺母细胞瘤/诊断; 胸膜肺母细胞瘤/外科学; 囊腺瘤样畸形; 肺; 先天性/诊断; 囊腺瘤样畸形; 肺; 先天性/外科学
- Keywords:
- Pleuropulmonary Blastoma/DI; Pleuropulmonary Blastoma/SU; Cystic Adenomatoid Malformation of Lung; Congenital/DI; Cystic Adenomatoid Malformation of Lung; Congenital/SU
- 分类号:
- R734.1;R734.3;R734.2
- 摘要:
- 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。
- Abstract:
- 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.
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备注/Memo
收稿日期:2018-06-25。
通讯作者:张儒舫,Email:zhangrf@shchildren.com.cn