背景:脊柱肿瘤(ST)通常会导致预后不良,有永久性瘫痪等风险,感官损失,括约肌功能障碍.关于其在儿科人群中的发病率和病因的数据明显不足。我们的研究调查了病因,临床表现,治疗,和小儿ST的结果。
方法:我们对我们的机构儿科肿瘤学和神经外科数据库进行了回顾性审查,检查自2005年以来因肿瘤疾病而入院的18岁以下的14例ST患者。我们分析了临床表现,评估,这些患者的分子诊断和治疗。
结果:该研究历时15年,包括14名儿科患者,每个诊断为不同的脊柱肿瘤实体。患者平均年龄约为19.6±10.1个月。在13例患者中观察到严重的沿脊柱的轴向疼痛,而急性神经功能恶化表现在7例。作为一线干预,13例患者通过椎板切除术和肿瘤切除术进行了减压手术,只有一名患者单独接受化疗。手术前,七名患者无法行走;手术后,其中六人恢复了走动的能力。诊断包括一系列肿瘤:尤文肉瘤的两个实例,3例畸胎瘤,一例表现为非典型畸胎瘤样横纹肌样瘤,低级别星形细胞瘤和神经母细胞瘤各2例,室管膜瘤的单个实例,脑膜瘤,横纹肌肉瘤,和具有多层玫瑰花结(ETMR)的胚胎性肿瘤。三名患者在开始治疗两年后死亡。
结论:尽管它们很少,儿科患者的椎管内肿瘤构成了巨大的治疗挑战。疾病实体和患者的神经状态交织在一起的复杂性要求迅速启动个性化治疗策略。这一关键步骤有助于优化该患者队列的结果,他们经常与衰弱的健康状况作斗争。由于这些患者在儿科人群中的稀有性,因此必须将其纳入注册表以优化治疗结果。
BACKGROUND: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.