探讨神经母细胞瘤(neuroblastoma，NB)患儿发病的临床特征及诊断治疗中有待提高的环节。

对2000年1月至2009年10月在本院确诊为神经母细胞瘤的36例患儿，按照美国儿童肿瘤协作组(Children's Cancer Study Group，CCSG)分期系统标准对NB患儿进行分期，对其采取影像学检查，包括超声，磁共振(magnetic resonance, MR)，CT或正电子发射断层显像(positron emission tomography, PET)-CT，骨扫描，骨髓细胞形态学检查，血清神经元特异性烯醇化酶(neuronspecific enolase，NSE)和多巴胺代谢产物尿香草扁桃酸(vanillylmandelic acid, VMA)测定，对部分患儿行肿物穿刺术或手术行活组织病理学检查。分析神经母细胞瘤患儿发病分布、临床特征、误诊常见原因及治疗后转归(本研究遵循的程序符合本院人体试验委员会所制定的伦理学标准，得到该委员会批准，并与患儿家长签署临床研究知情同意书)。

本组36例神经母细胞瘤患儿的平均发病年龄为3.6岁，其中，男孩为24例(66.7%) ；Ⅳ期为67%(24/36)。发病时，患儿临床表现多样复杂，误诊率达25%(9/36)。神经母细胞瘤的原发部位、临床表现有其特殊性，治疗率低(50%，18/36)。17例患儿放弃治疗；1例Ⅳ_s期患儿自愈；18例接受治疗患儿中，治愈为5例(3例为Ⅰ期，2例为Ⅳ期)，1例Ⅳ期患儿经治疗复发后，予维甲酸治疗痊愈。

多数神经母细胞瘤的患儿临床表现复杂，提高骨髓细胞形态学检查准确性，重视骨髓穿刺、腹部超声、神经元特异性烯醇酶、尿香草扁桃酸测定，可减少误诊率。部分Ⅰ期神经母细胞瘤患儿治疗效果不容乐观，需个体化加强治疗。目前联合化疗、手术、放疗，可延长患儿生命，但易复发，不良反应严重，需采取联合间碘苄胍(metaiodobenzyl guanidine，MIBG)放疗、3F8免疫治疗等治疗措施。

To evaluate clinical characteristics of neuroblastoma (NB)in children and identify the service gaps in the diagnosis and treatment.

Thirty-six cases who diagnosed as neuroblastoma from January 2000 to October 2009 were recurited in this study. Their stages were defined according to Children's Cancer Study Group (CCSG). Examinations included ultrasound, magnetic resonance (MR), CT or positron emission tomography(PET)-CT, bone scan, bone marrow morphology, neuronspecific enolase, urine vanillylmandelic acid, tumor aspiration or biopsy. Characterization of onset, clinical manifestations, misdiagnosis and treatment were analyzed. The study protocol was approved by the Ethical Review Board of Investigation in Human Being of SUN Yat-Sen Memorial Hospital. Informed consent was obtained from all participants.

The average age of children with neuroblastoma were 3.6 years old, including 24 boys(66.7%) and 12 girls(33.3%). Stage Ⅳ patients were 67%(24/36). Many different manifestations led to high misdiagnosis rates(25.0%, 9/36). There were specificity about primary site and clinical manifestation of neuroblastoma. Only 50.0%(18/36) received treatment. 17 cases abandoned and 1 case of stage IVs was self-cured. In the 18 cases treated, 5 were cured, including 3 stageⅠand 2 stageⅣ.1 case of stage Ⅳ had cis-platinum after relapse and cured.

Most patients have diverse manifestations , realizing that neuroblastoma may represent the cause of disease in a child will lead to earlier diagnosis by different specialists. Considered our reality, it is important to elevate the accuracy of bone marrow examination and think more of bone marrow aspiration, abdomen ultrasound, neuronspecific enolase, urine vanillylmandelic acid to decrease misdiagnosis rates. Individualized therapy is needed in some stageⅠpatients because of poor treatment effect. Combined therapy such as chemotherapy, surgery, radiotherapy could improved survival time, but had severe side effect and poor prognosis. Metaiodobenzyl guanidine (MIBG) radiotherapy, 3F8 immunotherapy are needed to be combined to the therapy.