O'Halloran Philip J, Farrell Michael, Caird John, Capra Michael, O'Brien David
National Neurosurgical Centre, Beaumont Hospital, Dublin 9, Ireland.
Childs Nerv Syst. 2013 Mar;29(3):367-74. doi: 10.1007/s00381-013-2023-x. Epub 2013 Jan 15.
Although uncommon, there is significant morbidity and mortality associated with paediatric spinal glioblastoma. The paucity of cases makes treatment options difficult. The current recommended standard of care is biopsy followed by adjuvant chemo-radiotherapy, with emerging data supporting the role of safe gross total resection.
The purpose of this paper is to provide a single-institution case study and to discuss current and future therapeutic treatment strategies.
A 14-year-old boy presented with a 2-year history of intermittent back pain with recent progressively worsening motor and sensory deficits of the right side. Pre-operative MRI revealed an enhancing intra-medullary tumour extending from C2 to C7. During the operative case, no tumour-cord margin could be identified, and the patient underwent a subtotal excision. Histopathology confirmed glioblastoma. In the subsequent weeks, the patient's clinical condition deteriorated. Adjuvant therapy was declined by the family, and the patient died 9 weeks after initial presentation.
Despite major advances in surgical techniques, peri-operative neuro-imaging as well as chemo-radiotherapy, the prognosis of a paediatric intra-medullary high-grade spinal tumour remains poor. Detailed analysis of our understanding of tumour dynamics in this patient group is important in establishing future therapeutic strategies.
尽管小儿脊髓胶质母细胞瘤并不常见，但与之相关的发病率和死亡率却很高。病例稀少使得治疗选择变得困难。目前推荐的标准治疗方案是活检后进行辅助放化疗，新出现的数据支持安全的全切除的作用。
本文旨在提供一个单机构病例研究，并讨论当前和未来的治疗策略。
一名14岁男孩有2年间歇性背痛病史，近期右侧运动和感觉功能障碍逐渐加重。术前磁共振成像（MRI）显示一个强化的髓内肿瘤，从C2延伸至C7。手术过程中，无法确定肿瘤与脊髓的边界，患者接受了次全切除。组织病理学证实为胶质母细胞瘤。在随后的几周里，患者的临床状况恶化。家属拒绝辅助治疗，患者在首次就诊9周后死亡。
尽管手术技术、围手术期神经影像学以及放化疗取得了重大进展，但小儿髓内高级别脊髓肿瘤的预后仍然很差。详细分析我们对该患者群体肿瘤动力学的理解对于制定未来的治疗策略很重要。
