儿童多发性神经母细胞瘤7例临床分析  

Clinical analysis of seven cases of children with multifocal neuroblastoma

作　　者：靳燕[1] 赵强[1] 闫杰[1] 王景福[1] 曹嫣娜[1] 李璋琳[1] 李杰[1] 李忠元[1] 王会娟[1] 

机构地区：[1]天津医科大学肿瘤医院儿童肿瘤科,国家肿瘤临床医学研究中心,天津市肿瘤防治重点实验室,天津市恶性肿瘤临床医学研究中心,天津市300060

出　　处：《中国肿瘤临床》2016年第18期804-807,共4页Chinese Journal of Clinical Oncology

基　　金：国家临床重点专科建设项目(编号:2013-544)资助~~

摘　　要：目的:总结儿童多发性神经母细胞瘤(neuroblastoma,NB)的临床特征及生物学行为,以期对这类肿瘤的治疗及预后判断提供依据。方法:回顾性分析天津医科大学肿瘤医院儿童肿瘤科1995年1月至2015年12月收治的7例多发性NB患儿的临床资料并对其随访。结果:共收治360例NB患儿,其中多发NB仅7例(1.9%)。患儿平均年龄(19.4±11.9)个月;原发部位胸腹多发3例,双肾上腺多发4例;临床分期4S期4例(57.1%),3期2例(28.6%),4期1例(14.3%)。7例共15个原发灶:14个手术完整切除;组织学预后良好型(favorable histology,FH)12个(85.7%),组织学预后不良型(unfavorable histology,UH)2个(14.3%);13个(92.9%)MYCN基因无扩增。随访满3年的5例患儿,3年总生存率(overall survival rate,OS)为100%。结论:多发性NB极其罕见,具有初次诊断年龄较低、以4S期为主、手术易完整切除的临床特征,肿瘤细胞分化较成熟、FH居多、少见MYCN基因扩增等生物学行为。该病多数预后较好,应避免过度治疗。Objective： To summarize the clinical features and biological behavior of multifocal neuroblastoma and provide evidence for its treatment and prognosis. Methods： Seven cases of multifocal neuroblastoma treated between January 1995 and December 2015 were retrospectively identified and reviewed. Follow-ups were conducted to monitor the postoperative conditions of the patients. Results： Among the 360 neuroblastoma cases treated at the authors＇ hospital in the past 20 years, 7 were multifocal neuroblastoma cases. The average age of the 7 patients was 29.4 ＋ 11.9 months. Thoracoabdominal neuroblastomas and bilateral adrenal neuroblastomas were present in 3 and 4 cases, respectively. Four of the cases were classified as stage 4S according to the International Neuroblastoma Staging System. All of the 7 patients were treated successfully with radical surgery, and 14 tumor samples were obtained. However, a tumor at the posterior mediastinum was not treated. Twelve of the tumors obtained （85.7%） were classified into the favorable histology group. Moreover, 13 tumors （92.9%） had no MYCN amplification. The estimated 3-year overall survival was 100%. Conclusion： Most multifocal neuroblastoma cases are commonly diagnosed in young patients （mostly classified as 4S） and can be easily treated by radical surgery. Multifocal neuroblastomas have been observed to demonstrate favorable histopathology and low MYCN expression. These findings suggest that most multifocal neuroblastoma cases have favorable prosnosis; therefore, excessive treatment must be avoided.

关 键 词：神经母细胞瘤 肿瘤 多原发 治疗 预后 儿童 

分 类 号：R739.4[医药卫生—肿瘤]