儿童胸膜肺母细胞瘤临床分析  

Clinical analysis of pleuropulmonary blastoma in children

作　　者：徐淑梅 王磊 安琪 王楠 王媛 韩蕊 孔庆玲 刘璐 XU Shumei;WANG Lei;AN Qi;WANG Nan;WANG Yuan;HAN Rui;KONG Qingling;LIU Lu(Hematology and Oncology Department of Xuzhou children′s hospital,Xuzhou Medical University,Xuzhou 221006,China)

机构地区：[1]徐州医科大学附属徐州儿童医院血液肿瘤内科,徐州221006

出　　处：《中国小儿血液与肿瘤杂志》2022年第6期390-394,共5页Journal of China Pediatric Blood and Cancer

摘　　要：目的通过1例儿童胸膜肺母细胞瘤(PPB)的诊治经过,结合相关文献复习,加强对该病的认识。方法对1例诊断为PPB I型的患儿临床资料进行分析,并结合文献报道的19例儿童PPB I型进行分析。结果患儿,女婴,9个月,首发表现为咳嗽伴喘息,胸部CT示右肺囊性占位病变,手术完整切除肿瘤,病理报告为胸膜肺母细胞瘤(Ⅰ型)。术后化疗4疗程后获得完全缓解,随访12个月,患儿无病生存。近10年国内文献报道PPB I型患儿19例,左右肺均有发病,临床症状基本为咳嗽、气促、气喘、发热、胸痛等,其中1例有家族史,4例手术切除后接受化疗。结论PPB是儿童时期罕见的发生于肺部的恶性肿瘤,一般症状出现较晚,以呼吸道症状为主,预后与病理类型、肿瘤能否完全切除、是否转移有关。Objective Through the diagnosis and treatment of 1 case of child pleuropulmonary blastoma,review the relevant literatures,to intensify the knowledge.Methods A case diagnosed with pleuropulmonary blastoma type I was analyzed,and the literature was reviewed to summarized the characteristics of such disease.Results The patient was a 9 months old girl,presented with cough accompanied by wheezing.Chest CT scan showed cystic lesions in the right lung.The tumor was completely resected and pathology diagnosed was pleuropulmonary blastoma type I.After 4 courses of chemotherapy,the patient were followed up for 12 months without disease.In recent 10 years,19 cases of pleuropulmonary blastoma type I have been reported in domestic literatures.Both the left and right lungs were affected,the clinical symptoms were cough,shortness of breath,asthma,fever,chest pain,etc.1 patient had a family history,and 4 patients received chemotherapy after surgical resection.Conclusions Pleuropulmonary blastoma is a rare lung malignant tumor in children.The clinical symptoms appear late,and the clinical manifestations are cough,wheezing,fever,dyspnea,etc.The prognosis is related to pathological type,whether the tumor can be completely resected and whether the tumor can metastasize.

关 键 词：儿童 胸膜肺母细胞瘤 预后 

分 类 号：R734.2[医药卫生—肿瘤]