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摘要: 目的 探讨儿童原发于颈部的神经母细胞瘤的临床特点、诊断、治疗、病理分型以及预后。 方法 回顾性分析2015年4月-2022年4月上海市儿童医院耳鼻咽喉头颈外科收治的11例原发于颈部神经母细胞瘤患儿的临床资料，分析其临床表现、影像学表现、病理类型、治疗方法及预后等特点，并结合文献进行讨论。 结果 11例患儿中男3例，女8例，年龄28 d~7岁4个月，中位年龄24个月。肿块位于颈动脉鞘区4例，颈侧肌间隙4例，咽旁咽后间隙3例。临床表现以无痛性颈部肿块、喉喘鸣为主。其中颈部原发瘤灶伴颈部淋巴结转移3例，伴骨髓转移1例。病理为神经母细胞瘤8例，节细胞神经母细胞瘤3例。本组患儿中7例采用手术联合化疗，2例采用手术联合放化疗，2例单采用手术治疗。所有病例中7例采用颈侧径路肿物切除术，3例采用经口径路肿物切除术，1例首次手术采用经口径路，再次手术时采用颈侧径路。患儿均完成所有治疗，中位随访时间34个月(6~79个月)，随访中9例患儿获得完全缓解，2例患儿获得部分缓解，无疾病进展者。 结论 儿童原发于颈部的神经母细胞瘤起病年龄较小，多以颈部肿物起病，具有高度异质性，内镜辅助经口内入路咽旁间隙肿瘤切除与外径路相比，具有周围组织损伤小、颜面部无瘢痕的优点，为临床提供了一种新的治疗手段。儿童神经母细胞瘤要做到定期随访，形成专病管理，全程精细化管理，以提升患儿的生存质量。Abstract: Objective To explore the clinical manifestations, diagnosis, treatment, pathological types and prognosis of primary cervical neuroblastoma in children. Methods The clinical data of 11 children with primary cervical neuroblastoma admitted to the Department of Otolaryngology and Head and Neck Surgery at Shanghai Children's Hospital from April 2015 to April 2022 were retrospectively analyzed. The clinical characteristics, imaging findings, pathological types, treatment methods, and outcomes of these 11 patients were examined in conjunction with a review of the literature. Results The cohort of 11 neuroblastoma patients ranged in age from 28 days to 88 months (median age: 24 months), including 3 males and 8 females. Among the 11 patients, 4 had tumors located in the carotid sheath area, 4 in the cervical space, and 3 in the parapharyngeal and retropharyngeal spaces. The clinical manifestations primarily included painless cervical masses and laryngeal stridor. There were 3 cases of primary cervical tumors with cervical lymph node metastasis and 1 case with bone marrow metastasis. Pathological findings revealed neuroblastoma in 8 cases and ganglionic neuroblastoma in 3 cases. In this group, 7 patients underwent surgery combined with chemotherapy, 2 patients received surgery combined with chemoradiotherapy, and 2 patients underwent surgery alone. Surgical resection was performed via a cervical approach in 7 cases, while 3 cases were treated using a transoral endoscopic approach. Additionally, one patient underwent a transoral endoscopic approach initially and a cervical approach subsequently. All patients completed their treatment and were followed up regularly, with follow-up durations ranging from 6 to 79 months (median: 34 months). Nine patients achieved complete remission, 2 patients achieved partial remission, and none experienced disease progression. Conclusion Primary cervical neuroblastoma exhibits a high degree of heterogeneity and presents at a younger age, primarily with cervical masses. Compared to external approaches, endoscopy-assisted transoral resection of parapharyngeal tumors offers advantages such as reduced damage to surrounding tissue and no visible neck scars, providing a new method for clinical treatment. Regular follow-up is essential for children with neuroblastoma, along with the establishment of specific disease management protocols and comprehensive care to improve survival quality.
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Key words:
- neck /
- neuroblastoma /
- ganglionic neuroblastoma /
- parapharyngeal space /
- retropharyngeal space /
- carotid sheath /
- children
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表 1 儿童原发于颈部神经母细胞瘤患儿的临床资料
例序 性别 年龄/月 肿块部位 肿块大小/cm 病理特点 主诉 1 男 1 右侧颈部肌间隙 2.2×2.8×3.6 神经母细胞瘤(差分化) 出生后呼吸困难，加重5 d 2 女 3 右侧咽后壁 2.0×2.0×2.4 神经母细胞瘤(差分化) 喉喘鸣半个月 3 男 10 左侧咽旁间隙 2.5×3.0×3.4 神经母细胞瘤(分化型) 睡眠打鼾伴喘憋2个月 4 女 12 右侧颈动脉鞘区 4.4×2.5×4.2 神经母细胞瘤(分化型) 发现右侧颈部肿块半个月 5 女 17 左侧颈部肌间隙 3.1×4.3×6.1 神经母细胞瘤(差分化) 发现左侧颈部肿块2个月 6 女 24 右侧胸锁乳突肌内侧 6.8×5.7×4.9 神经母细胞瘤(分化型) 发现右侧颈部肿块1年 7 女 45 右侧颈动脉鞘区 4.6×3.3×3.9 神经母细胞瘤(分化型) 发现右侧颈部肿块3个月 8 女 48 左侧颈动脉鞘区 3.0×3.3×4.7 节细胞神经母细胞瘤(混合型) 发现左侧颈部肿块3周 9 女 60 左侧颈部肌间隙 7.0×8.3×4.7 神经母细胞瘤(分化型) 发现左侧颈部肿块半年 10 男 76 左侧颈动脉鞘区 3.0×1.8×3.9 节细胞神经母细胞瘤(混合型) 发现左侧颈部肿块半个月 11 女 88 右侧咽旁间隙 3.0×6.0×7.2 节细胞神经母细胞瘤(结节型) 发现右侧颈部肿块1年 
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表 2 本组患儿的国际神经母细胞瘤病理学分类(INPC)
例序 形态学分类 预后分类 INSS分期 INRG分期 危险度分层(COG) 1 神经母细胞瘤(差分化) 良好型 Ⅰ L2 低危组 2 神经母细胞瘤(差分化) 良好型 Ⅰ L2 低危组 3 神经母细胞瘤(分化型) 良好型 Ⅰ L1 极低危组 4 神经母细胞瘤(分化型) 良好型 Ⅲ L2 中危组 5 神经母细胞瘤(差分化) 良好型 Ⅳ MS 中危组 6 神经母细胞瘤(分化型) 良好型 ⅡA L2 低危组 7 神经母细胞瘤(分化型) 良好型 ⅡB L2 低危组 8 节细胞神经母细胞瘤(混合型) 良好型 ⅡA L2 极低危组 9 神经母细胞瘤(分化型) 不良型 Ⅲ L2 高危组 10 节细胞神经母细胞瘤(混合型) 良好型 ⅡA L2 极低危组 11 节细胞神经母细胞瘤(结节型) 不良型 Ⅲ L2 中危组 注：原发于颈部的神经母细胞瘤影像学危险因子(IDRFs)：肿瘤包绕颈动脉和(或)颈内静脉；肿瘤压迫气管；肿瘤延伸到颅底或胸腔(病例5同时累及颈部与胸腔)。
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表 3 儿童原发于颈部神经母细胞瘤患儿的临床分析
临床特征 例数(%) 临床特征 例数(%) 临床特征 例数(%) 年龄 转移情况 手术方式 ≤24个月 6(54.5) 颈淋巴结转移 3(27.3) 颈侧径路 7(63.6) >24个月 5(45.5) 骨髓转移 1(9.1) 经口径路 3(27.3) 首发临床表现 预后分类 经口+颈侧 1(9.1) 颈部肿物 8(72.7) 良好型 9(81.8) 并发症 呼吸困难 3(27.3) 不良型 2(18.2) 肺炎支气管炎 8(72.7) 体征 性别 Horner征 2(18.2) 咽侧咽后壁隆起 5(45.5) 男 3(27.3) 转归 危险度分层 女 8(72.7) CR 9(81.8) 低危极低危组 7(63.6) 儿茶酚胺代谢产物 PR 2(18.2) 中高危组 4(36.4) 尿VMA增高 5(45.5) 治疗模式 尿HVA增高 8(72.7) 手术 2(18.2) 病理分型 手术+化疗 7(63.6) 神经母细胞瘤 8(72.7) 手术+化疗+放疗 2(18.2) 节细胞神经母细胞瘤 3(27.3)
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表 4 儿童原发于颈部神经母细胞瘤患儿的治疗与转归
例序 手术方式 治疗过程 并发症 化疗 放疗 随访时间/月 转归 1 颈侧入路 手术-化疗 肺炎 有 无 34 CR 2 经口径路 手术-化疗-手术 肺炎、右侧睑裂小 有 无 28 CR 3 经口径路 手术 无 无 无 6 CR 4 颈侧入路 手术-化疗-手术-放疗 颈淋巴结转移、支气管炎 有 有 9 CR 5 颈侧入路 化疗-手术-化疗 骨髓转移、支气管炎 有 无 6 PR 6 颈侧入路 手术-化疗 支气管炎 有 无 75 CR 7 颈侧入路 手术-化疗 颈淋巴结转移 有 无 79 CR 8 颈侧入路 手术-化疗 支气管炎 有 无 65 CR 9 经口+颈侧 手术-化疗-手术-放疗 肺炎、颈淋巴结转移 有 有 57 PR 10 经口径路 手术 左侧上睑下垂 无 无 10 CR 11 颈侧入路 手术-化疗 支气管炎 有 无 43 CR
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