儿童多发性神经母细胞瘤7 例临床分析*

目的:总结儿童多发性神经母细胞瘤（neurobl astoma ，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 survivalrate ，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。 

Clinical analysis of seven cases of children with multifocal neuroblastoma

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. Fol ow-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 19.4 ± 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 me-diastinum 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 prognosis;therefore, excessive treatment must be avoided.