摘要
目的:提高对纵隔神经母细胞瘤临床特征及预后的认识。方法:对2008年3月至2012年3月中国医科大学附属第四医院儿科收治的24例纵隔神经母细胞瘤患儿的临床表现、组织分型、治疗及预后进行回顾性分析。结果:男16例,女8例,发病年龄1-60月(平均25月),原发肿瘤均位于后纵隔。21例经肿瘤组织病理学确诊,其中15例神经母细胞瘤,6例神经节母细胞瘤,3例经骨髓细胞学确诊。临床分期:Ⅰ期4.2%,Ⅱ期29.2%,III期33.3%,Ⅳ期29.1%,Ⅳs期4.2%,III、Ⅳ期病例以大于18月多见,占86.7%。初诊时常见的症状依次为呼吸系统表现、游走性腿痛伴或不伴低热、偶然体检发现、神经系统表现及无痛性肿块。20例给予原发肿瘤切除和术后化疗,4例仅给予化疗。21例存活患儿,随访6-48月,17例持续完全缓解达6-48月,4例带瘤存活,死亡3例。远期并发症6例,5例是纵隔术后的霍纳综合征,1例为眼阵挛-肌阵挛综合征后遗症。结论:儿童纵隔神经母细胞瘤初诊时临床表现各异,以呼吸系统症状多见。初诊时年龄小于18月或临床分期为Ⅰ、Ⅱ、III期者可能预后较好,年龄大于18月的Ⅳ期病例,病情进展快,生存率低。
Objective：To improve the understanding of the clinical characteristics and prognosis of mediastinal neuroblastoma in childrenhood.Methods：All 24 children treated at the Fourth Affiliated Hospital of China Medical University from 2008 to 2012 with diagnoses of mediastinal neuroblastoma were reviewed for clinical characteristics,histotypes,treatment and prognosis.Results：Of the 24 cases,16 were males and 8 females,the ages of onset ranged from 1 to 60 months（median 25 months）.Primary tumor site was the posterior mediastinum in all.21 cases were diagnosed by tumor histopathology,of which 15 cases were neuroblastoma,6 cases were ganglion neuroblastoma,Other 3 cases were diagnosed by bone marrow cytology.Stages were： I,4.2%;II,29.2%;III,33.3%;IV,29.1%;IVS,4.2%.Stages III and IV were more common in cases over 18 months of age,accounting for 86.7%.The most common symptoms at initial diagnosis were respiratory,followed by migratory pain in legs with fever or not.Occasionally medical examination found,nervous system symptoms,painless masses.20 cases treated with primary surgical resection and adjuvant chemotherapy,4 with chemotherapy.Followed up the 21 alive children for 6-48 months.17 cases went into remission continuously and completely for 6-48 months,4 cases were alive with tumors,3 children died,6 of the 24 children had long-term complications,including five cases of Horner syndrome after the operations,1 case of eye clonus-myoclonus syndrome sequelae.Conclusion：Clinical characteristics of mediastinal neuroblastoma are various,the most common symptoms are seen in the respiratory system.Chiedren ages at diagnosis younger than 18 months or clinical stages are I,II,III have better prognosises,children older than 18 months of clinical stage IV,progressed rapidly and the survival rate is low.
出处
《现代肿瘤医学》
CAS
2012年第11期2279-2282,共4页
Journal of Modern Oncology
基金
辽宁省科学技术计划项目(编号:2010225001)
关键词
神经母细胞瘤
纵隔
儿童
neuroblastoma
mediastinum
children
