Spinal ependymoma: a comprehensive review of molecular classification, management guidelines, and clinical outcomes with a focus on the pediatric population (Part III of ependymomas across compartments).
This narrative review synthesizes molecular classification, surgical and adjuvant management, outcomes, and surveillance considerations for spinal ependymoma, emphasizing the scarcity of pediatric-specific evidence.
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This narrative review synthesizes molecular classification, surgical and adjuvant management, outcomes, and surveillance considerations for spinal ependymoma, emphasizing the scarcity of pediatric-specific evidence.
Research significance
The reviewed evidence supports maximal safe gross total resection and risk-adapted radiotherapy as central management strategies; it further suggests—but does not establish prospectively—that molecular classification, particularly identification of MYCN-amplified tumors, could improve pediatric risk stratification and treatment selection.
Source abstract
PURPOSE: Spinal ependymomas are rare tumors of the central nervous system characterized by distinct anatomical, histological, and molecular subtypes. This review aims to synthesize current evidence regarding the presentation, diagnosis, surgical management, adjuvant therapy, and outcomes of spinal ependymomas, with a focus on the pediatric population. METHODS: We conducted a narrative review of contemporary literature, consensus recommendations, and relevant treatment-related molecular classifications. Key topics included preoperative evaluation, neuraxis imaging, intraoperative strategies, extent of resection, histopathologic and molecular risk stratification, adjuvant radiotherapy and chemotherapy, recurrence patterns, and long-term surveillance. Representative clinical cases illustrating the surgical management of intramedullary spinal ependymomas and myxopapillary ependymomas are provided in the supplementary material. RESULTS: Spinal ependymomas encompass spinal ependymoma, spinal ependymoma with MYCN amplification, spinal subependymoma, and spinal myxopapillary ependymoma. The primary therapeutic goal is maximal safe gross total resection, which is the most consistent modifiable predictor of progression-free survival. Molecular classification enhances risk stratification, especially for the aggressive MYCN-amplified subgroup. However, histologic grade and the extent of resection remain critical to management. Adjuvant radiotherapy is generally recommended for cases involving subtotal resection, grade 3 disease, tumor dissemination, capsular violation in myxopapillary tumors, or high-risk biology. Chemotherapy does not have a well-established role as a first-line treatment. Much of this evidence is extrapolated from adult or mixed-age cohorts, however, and pediatric-specific data remain limited across nearly every management domain, a gap we characterize systematically. CONCLUSION: Management of spinal ependymomas is centered around surgery and adapted to individual risk factors. Overall survival is generally favorable for spinal ependymomas, but recurrence risk remains substantial and varies by extent of resection and molecular subtype. Lifelong surveillance is crucial, as both local recurrence and craniospinal dissemination may occur long after treatment.