Centre for Spinal Studies and Surgery, Queen's Medical Centre, Nottingham University Hospitals NHS Trust, Nottingham, NG7 2UH, UK.
Department of Orthopaedic Surgery, Assiut University, Assiut, Egypt.
Eur Spine J. 2023 Dec;32(12):4306-4313. doi: 10.1007/s00586-023-07820-3. Epub 2023 Jun 20.
Malignant spinal cord compression (MSCC) has been noted in 3-5% of children with primary tumours. MSCC can be associated with permanent neurological deficits and prompt treatment is necessary. Our aim was to perform a systematic review on MSCC in children < 18 years to help formulate national guidelines.
A systematic review of the English language was undertaken using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Search criteria included 'MSCC in children, paediatric and metastases' for papers published between January1999 and December 2022. Isolated case reports/case series with < 10 patients were excluded.
From a total of 17 articles identified, a final 7 were analysed (Level III/IV). Neuroblastoma constituted the most common cause for MSCC in children (62.7%) followed by sarcoma (14.2%). Soft tissue sarcomas were the most frequent cause of MSCC in children > 5 years old, while for neuroblastomas, the mean age of presentation was 20 months. The median age at time of diagnosis for the entire cohort of patients was 50.9 months (14.8-139). The median follow-up duration was 50.7 months (0.5-204). Motor deficits were the presenting symptom in 95.6% of children followed by pain in 65.4% and sphincter disturbance in 24%. There was a delay of about 26.05 days (7-600) between the onset of symptoms and diagnosis. A multimodality approach to treatment was utilised depending on the primary tumour. The prognosis for neurological recovery was found to be inversely proportional to the degree of neurological deficits and duration of symptoms in four studies.
Neuroblastoma is the most common cause for MSCC in children (62.7%) followed by sarcoma (14.2%), whilst soft tissue sarcomas constituted the most frequent cause of MSCC in children > 5 years old. The majority of patients presented with motor deficit, followed by pain. In children with neuroblastoma /lymphoma, chemotherapy was the primary treatment. Early surgery should be a consideration with rapid deterioration of neurology despite chemotherapy. A multimodality approach including chemo-radiotherapy and surgery should be the treatment of choice in metastatic sarcomas. It is worth noting that multi-level laminectomy/decompression and asymmetrical radiation to the spine can lead to spinal column deformity in the future.
原发性肿瘤患儿中有 3-5%会出现恶性脊髓压迫症（MSCC）。MSCC 可能导致永久性神经功能缺损，因此需要及时治疗。我们的目的是对 18 岁以下儿童的 MSCC 进行系统综述，以帮助制定国家指南。
我们按照系统综述和荟萃分析的首选报告项目（PRISMA）指南对英文文献进行了系统检索。检索词包括“儿童、小儿和转移”，检索时间为 1999 年 1 月至 2022 年 12 月。排除了仅有 10 例以下患者的单独病例报告/病例系列。
共确定了 17 篇文章，最终分析了 7 篇（III/IV 级）。神经母细胞瘤是儿童 MSCC 的最常见原因（62.7%），其次是肉瘤（14.2%）。儿童软组织肉瘤是儿童中最常见的 MSCC 原因，而神经母细胞瘤的平均发病年龄为 20 个月。整个患者队列的中位诊断年龄为 50.9 个月（14.8-139）。中位随访时间为 50.7 个月（0.5-204）。95.6%的患儿以运动障碍为首发症状，65.4%以疼痛为首发症状，24%以括约肌功能障碍为首发症状。症状出现至诊断的中位时间延迟约为 26.05 天（7-600）。根据原发肿瘤的不同，采用了多种方法进行治疗。四项研究表明，神经功能恢复的预后与神经功能缺损的程度和症状持续时间呈反比。
神经母细胞瘤是儿童 MSCC 的最常见原因（62.7%），其次是肉瘤（14.2%），而儿童软组织肉瘤是 5 岁以上儿童 MSCC 的最常见原因。大多数患者以运动障碍为首发症状，其次是疼痛。对于神经母细胞瘤/淋巴瘤患儿，化疗是主要治疗方法。对于神经功能迅速恶化的患者，即使接受化疗，也应考虑早期手术。对于转移性肉瘤，应采用包括化疗、放疗和手术的多模式治疗方法。需要注意的是，多节段椎板切除术/减压术和脊柱不对称放疗可能导致脊柱畸形。
