新生儿胎儿肺间质瘤一例并文献复习  

Neonatal fetal lung interstitial tumor:a case report and literature review

作　　者：赵军锋[1] 董晟[1] 席大伟[1] 俞承忠[1] 诸建明[1] 刘云凤 曾骐[3] Zhao Junfeng;Dong Sheng;Xi Dawei;Yu Chengzhong;Zhu Jianming;Liu Yunfeng;Zeng Qi(Department of Pediatric Surgery,Municipal Women&Children's Hospital,Ningbo 315012,China;Department of Pediatrics,Municipal People's Hospital,Ningbo 310000,China;Department of Thoracic Surgery,Beijing Children's Hospital,Beijing 100045,China)

机构地区：[1]宁波市妇女儿童医院小儿外科,315012 [2]宁海人民医院儿科,310000 [3]北京儿童医院胸外科,100045

出　　处：《中华小儿外科杂志》2020年第4期330-334,共5页Chinese Journal of Pediatric Surgery

基　　金：宁波市科技计划项目(2014B82003)。

摘　　要：目的 通过新生儿胎儿肺间质瘤(fetal lung interstitial tumor,FLIT)1例并文献复习,探讨小儿胸外科医生对FLIT的认识和诊治方法。方法 回顾性分析2018年8月宁波市妇女儿童医院收治的1例FLIT患儿的临床资料,同时检索Medline、Embase、Web of Science等外文数据库和中国知网、万方、维普网等中文数据库的相关文献,分析该疾病的病理组织学特点、临床表现、诊疗和预后情况。结果 本例患儿因出生后呼吸急促2 d就诊于当地医院,胸部CT发现右肺上叶巨大占位,转诊至我院,入院考虑为"先天性囊性腺瘤样畸形(congenital cystic adenomatoid malformation,CCAM)"。因生命体征不稳定,急诊行"右上肺叶切除术"。术后病理组织学结合免疫组织化学考虑为胸膜肺母细胞瘤(Ⅰ型);外院病理学检查会诊结果:间叶源性肿瘤,可见核分裂,低度恶性,难以进一步分类。Dicer1的外显子exon24和exon25未见突变。查阅文献资料,复核病理组织学特点,最后诊断为FLIT,未辅助化疗。术后患儿恢复良好,体格生长同正常儿,随访至今无复发。检索文献至2018年11月,总共报道10例,全部经手术治疗(7例肺叶切除+3例肺楔形切除),其中1例辅以长春新碱为基础的化疗。全部病例随访至今无复发。结论 FLIT是一种极其罕见的小婴儿肺部肿瘤,需要小儿胸外科医生充分认识并合理选择诊治方案:在诊断上,需要联合影像学、病理组织学和免疫组织化学加以明确;在治疗上,以手术切除为主,不建议辅助化疗。Objective To explore the recognition and management of neonatal fetal lung interstitial tumor(FLIT)among pediatric thoracic surgeons along with a literature review.Methods Clinical data were collected from a case of neonatal FLIT admitted in August 2018 and retrieved from the databases of MEDLINE,EMBASE,Web of Science,CNKI,WANFANG and CQVIP until November 2018.The histopathological features,clinical manifestations,treatment and prognosis were recorded.Results This infant was referred to a local hospital with dyspnea at 2 days after birth.Chest computed tomography(CT)imaging revealed a large mass in upper lobe of right lung.He was transferred to our hospital for an initial diagnosis of congenital cystic adenomatoid malformation(CCAM).Right upper lobectomy was performed for emergency due to unstable vital signs and the mass was provisionally diagnosed as typeⅠ(cystic)pleuropulmonary blastoma(cPPB)by pathological histology plus immunohistochemistry.However,the pathological results were recommended for consultations at a superior hospital where mesenchymal tumor was diagnosed with visible mitosis and low malignancy and further classification remained difficult.The exons 24&25 of DICER1 were not mutated.Therefore the authors reviewed the literature and histopathological features again and finally confirmed the presence of FLIT without adjuvant postoperative chemotherapy.The patient recovered well with comparable physical growth as normal children and no recurrence has been observed during follow-ups.Literature retrieval was performed up to November 2018.A total of 10 cases were reported and the specific procedures included lobectomy(n=7)and pulmonary wedge resection(n=3).One case was supplemented with vincristine-based chemotherapy.No recurrence was found during follow-ups.Conclusions As an extremely rare lung tumor in infants,FLIT requires full understandings and selecting proper diagnostic and therapeutic measures for pediatric thoracic surgeons to gain.A definite diagnosis is based upon imaging,histopathology and

关 键 词：肺肿瘤 新生儿 胎儿肺间质瘤 

分 类 号：R734.2[医药卫生—肿瘤]