Department of Neurosurgery, Heidelberg University Hospital, Heidelberg, Germany.
Department of Neurosurgery, University of Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Germany.
Neurosurg Rev. 2024 Sep 6;47(1):557. doi: 10.1007/s10143-024-02770-w.
Spinal tumors (ST) often result in dire prognosis, carrying risks such as permanent paralysis, sensory loss, and sphincter dysfunction. Data on their incidence and etiology in pediatric populations are markedly scant. Our study investigates the etiology, clinical manifestation, treatment, and outcomes of pediatric ST.
We conducted a retrospective review of our institutional pediatric oncology and neurosurgery database, examining 14 patients under 18 years admitted with ST due to oncological diseases since 2005. We analyzed the clinical presentations, evaluations, molecular diagnostics and treatments for these patients.
The study spanned 15 years and included 14 pediatric patients, each diagnosed with distinct spinal tumor entity. The mean patient age was approximately 19.6 ± 10.1 months. Severe axial pain along the vertebral column was observed in 13 patients, while acute neurological deterioration manifested in 7 patients. As a first-line intervention, 13 patients underwent decompressive surgery through laminectomy and tumor resection, and only one patient received chemotherapy solely. Before surgery, seven patients were unable to walk; post-surgery, six of them regained their ability to ambulate. The diagnosis encompassed a range of neoplasms: two instances of Ewing sarcoma, 3 instances of teratoma, one case presenting an atypical teratoid Rhabdoid tumor, two instances each of low-grade astrocytoma and neuroblastoma, and single instances of ependymoma, meningioma, rhabdomyosarcoma, and embryonal tumors with multilayered rosettes (ETMRs). Three patients succumbed two years after initiating therapy.
Despite their rarity, intraspinal tumors in pediatric patients pose substantial therapeutic challenges. The intertwined complexities of the disease entity and the patient's neurological status demand swift initiation of an individualized therapeutic strategy. This crucial step helps optimize outcomes for this patient cohort, who frequently grapple with debilitating health conditions. Inclusion of these patients within a registry is mandatory to optimize treatment outcomes due to their rarity in pediatric population.
脊髓肿瘤（ST）常导致预后不良，存在永久性瘫痪、感觉丧失和括约肌功能障碍等风险。关于儿科人群中 ST 的发病率和病因的数据明显很少。我们的研究调查了儿科 ST 的病因、临床表现、治疗和结果。
我们对我们机构的儿科肿瘤学和神经外科学数据库进行了回顾性分析，研究了自 2005 年以来因肿瘤疾病而患有 ST 的 14 名 18 岁以下的患者。我们分析了这些患者的临床表现、评估、分子诊断和治疗。
这项研究跨越了 15 年，包括 14 名儿科患者，每个患者都被诊断为不同的脊髓肿瘤实体。患者的平均年龄约为 19.6±10.1 个月。13 名患者出现严重的脊柱轴向疼痛，7 名患者出现急性神经功能恶化。作为一线干预措施，13 名患者通过椎板切除术和肿瘤切除术进行了减压手术，只有一名患者仅接受了化疗。手术前，7 名患者无法行走；手术后，其中 6 名恢复了行走能力。诊断包括一系列肿瘤：2 例尤文肉瘤，3 例畸胎瘤，1 例非典型性畸胎瘤横纹肌样瘤，2 例低级别星形细胞瘤和神经母细胞瘤各 1 例，1 例室管膜瘤、脑膜瘤、横纹肌肉瘤和胚胎性肿瘤伴多层玫瑰花结（ETMRs）各 1 例。3 名患者在开始治疗后两年内死亡。
尽管罕见，儿童脊髓肿瘤仍对治疗构成重大挑战。疾病实体的复杂性和患者的神经状态交织在一起，需要迅速启动个体化的治疗策略。这一关键步骤有助于优化这群经常患有衰弱性疾病的患者的结果。由于儿科人群中发病率较低，将这些患者纳入登记册对于优化治疗结果是强制性的。
