Objective: To determine the characteristics of cervical neuroblastoma and the effect of resection extent on survival and outcomes. Methods: We performed a retrospective review of 32 children with cervical neuroblastoma treated at Beijing Children's Hospital between April 2013 and August 2020. Data were collected from the medical record. The individualized therapy was designed based on staging and risk group. Based on the extent of resection, patients were divided into incomplete and complete resection groups. Event free and overall survival rates were compared between two groups using the Kaplan-Meier method. Results: The ages of patients ranged from 1 month to 81 months, with a median age of 11 months, including 7 males and 15 females. Twenty-nine patients (90.6%) presented with cervical painless mass. The average diameter of the primary tumors was (5.12±1.43) cm. Tumors were located in the parapharyngeal space in 25 cases (78.1%) and in the root of the neck in 7 cases (21.9%). None had MYCN amplification. According to International Neuroblastoma Staging System (INSS), 15 patients (46.9%) were identified as stage 1, 11 patients (34.3%) as stage 2B, 3 patients (9.4%) as stage 3 and 3 patients (9.4%) as stage 4. There were 12 patients (37.5%) at low risk, 17 patients (53.1%) at intermediate risk and 3 patients at high risk according to Children's Oncology Group (COG) risk classification system. All patients underwent tumor resection. Postoperatively Horner's syndrome occurred in 13 patients (40.6%), pneumonia in 9 patients (28.1%), pharyngeal dysfunction in 8 patients (25.0%) and transient hoarseness in 4 patients (12.5%). At a median follow-up of 36.5 months, the overall survival rate was 96.4%, with no significant difference between incomplete and complete resection groups (100.0% vs. 96.3%, χ2=0.19, P=0.667); the event free survival rate was 78.1%, with a significant difference between the two groups (40.0% vs. 85.2%, χ²=6.71, P=0.010). Conclusions: Primary cervical neuroblastoma has a young onset age, mostly in low and medium risk groups, and represents favorable lesions with good outcomes after multidisciplinary therapy. Less aggressive surgery with preservation of important structures is recommended. Complete resection should not be attempted if it would compromise vital structures.
目的： 探索原发于颈部的神经母细胞瘤（neuroblastoma，NB）患儿的临床特征以及手术切除范围对其预后的影响。 方法： 回顾性收集2013年4月至2020年8月在首都医科大学附属北京儿童医院接受颈部NB治疗患儿的临床特征，并根据临床分期分组不同采用个体化诊疗方案，分为肿瘤部分切除组与肿瘤全部切除组，比较2组患儿的无事件生存率和总生存率，采用Kaplan-Meier法绘制生存曲线并进行组间比较。 结果： 共收集符合入组标准患者32例，其中男17例，女15例，年龄1~81个月，中位年龄11个月。29例（90.6%）患儿临床表现为颈部无痛性包块。肿瘤原发灶直径为（5.12±1.43）cm，25例（78.1%）瘤灶位于咽旁间隙，7例（21.9%）位于颈根部。所有患儿均无MYCN基因扩增。国际NB分期系统（INSS）1期15例（46.9%），2B期11例（34.3%），3期3例（9.4%），4期3例（9.4%）。儿童肿瘤协作组（COG）危险度分组低危组12例（37.5%），中危组17例（53.1%），高危组3例（9.4%）。所有患儿均接受手术治疗，术后出现Horner综合征者13例次（40.6%），肺炎9例次（28.1%），吞咽功能障碍8例次（25.0%），单侧声带麻痹4例次（12.5%）。本研究中位随访时间36.5个月（0.5~75个月），总生存率为96.4%，无事件生存率为78.1%。肿瘤部分切除组与肿瘤全部切除组之间的无事件生存率比较差异有统计学意义（40.0%比85.2%，χ²=6.71，P=0.010），总生存率比较差异无统计学意义（100.0%比96.3%，χ²=0.19，P=0.667）。 结论： 原发于颈部的NB起病年龄较小，多为低、中危组，规范治疗预后较好。推荐对儿童颈部NB采用最小破坏性的手术方法，即在保留重要结构的前提下尽可能完整切除肿瘤，但不应为了追求肿瘤完全切除而牺牲重要结构。.