Decoding pediatric spinal tumors: a single-center retrospective case series on etiology, presentation, therapeutic strategies, and outcomes

Abstract

Introduction: 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.

Conclusion: 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.

Keywords: Neurosurgical oncology; Pediatric spinal tumor; Surgical decompression.

Fig. 1

Sagittal (A) and Axial (B) MRI T2 Sequences Displaying Anaplastic Ependymoma in the Thoracic Spine of a 6-months-Old (ID_10). The pronounced compression of the cauda equina is evident. Remarkably, the child exhibited severe spinal pain without accompanying neurological deficits. Surgical intervention involved a laminectomy for tumor resection

Fig. 2

Preoperative (A, D), postoperative (B, E), and one-year follow-up (C, F) MRI images, both sagittal T2 and with contrast, show a spinal ETMR located in the lumbar spine of a 28-months-old child (ID_04). This child experienced severe pain, controllable only with morphine, and acute paraplegia. An urgent laminotomy from L1 to L5 and tumor resection were performed, resulting in total removal of the tumor. This was followed by multimodal adjuvant therapy