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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).

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Τίτλος: 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).
Συγγραφείς: Price AM; Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, TN, USA.; Division of Pediatric Neurosurgery, Monroe Carell Jr Children's Hospital at Vanderbilt University Medical Center, Nashville, TN, USA., Fredricks NS; John Sealy School of Medicine, The University of Texas Medical Branch at Galveston, Galveston, TX, USA.; Department of Neurosurgery, The University of Texas Medical Branch at Galveston, Galveston, TX, USA., Koutsouras G; Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, TN, USA.; Division of Pediatric Neurosurgery, Monroe Carell Jr Children's Hospital at Vanderbilt University Medical Center, Nashville, TN, USA., Vallejo FA; Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, TN, USA.; Division of Pediatric Neurosurgery, Monroe Carell Jr Children's Hospital at Vanderbilt University Medical Center, Nashville, TN, USA., Bonfield C; Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, TN, USA.; Division of Pediatric Neurosurgery, Monroe Carell Jr Children's Hospital at Vanderbilt University Medical Center, Nashville, TN, USA., Pastakia DJ; Department of Pediatrics, Vanderbilt University Medical Center, Nashville, TN, USA.; Division of Pediatric Hematology/Oncology, Monroe Carell Jr Children's Hospital at Vanderbilt University Medical Center, Nashville, TN, USA., Luo LY; Department of Radiation Oncology, Vanderbilt University Medical Center, Nashville, TN, USA., Boop F; Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, TN, USA.; St. Jude Children's Research Hospital, Memphis, TN, USA., Ramaswamy V; Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, ON, Canada., Dewan MC; Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, TN, USA. michael.dewan@vumc.org.; Division of Pediatric Neurosurgery, Monroe Carell Jr Children's Hospital at Vanderbilt University Medical Center, Nashville, TN, USA. michael.dewan@vumc.org.
Πηγή: Journal of neuro-oncology [J Neurooncol] 2026 Oct 06; Vol. 180 (1). Date of Electronic Publication: 2026 Oct 06.
Τύπος έκδοσης: Journal Article; Review
Γλώσσα: English
Στοιχεία περιοδικού: Publisher: Springer Country of Publication: United States NLM ID: 8309335 Publication Model: Electronic Cited Medium: Internet ISSN: 1573-7373 (Electronic) Linking ISSN: 0167594X NLM ISO Abbreviation: J Neurooncol Subsets: MEDLINE
Imprint Name(s): Publication: 2005- : New York : Springer
Original Publication: Boston : M. Nijhoff, 1983-
Ιατρικοί όροι (MeSH): Ependymoma*/therapy , Ependymoma*/classification , Ependymoma*/diagnosis , Ependymoma*/pathology , Ependymoma*/genetics , Spinal Cord Neoplasms*/therapy , Spinal Cord Neoplasms*/classification , Spinal Cord Neoplasms*/diagnosis , Spinal Cord Neoplasms*/pathology , Spinal Cord Neoplasms*/genetics , Practice Guidelines as Topic*/standards, Humans ; Child
Περίληψη: 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.
(© 2026. The Author(s).)
Competing Interests: Declarations. Competing interests: The authors declare no competing interests.
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Contributed Indexing: Keywords: Ependymoma; Intramedullary tumor; Laminoplasty; MYCN amplification; Myxopapillary ependymoma; Pediatric spinal ependymoma; Spinal ependymoma
Entry Date(s): Date Created: 20261006 Date Completed: 20261006 Latest Revision: 20261009
Update Code: 20261009
PubMed Central ID: PMC13642508
DOI: 10.1007/s11060-026-05816-9
PMID: 42837045
Βάση Δεδομένων: MEDLINE
Περιγραφή
ISSN:1573-7373
DOI:10.1007/s11060-026-05816-9