ArticleNeuro-oncology2023
Surgery and proton radiation therapy for pediatric base of skull chordomas: Long-term clinical outcomes for 204 patients.
Article in Neuro-oncology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers, 1 of them a synthesis that pooled it.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
7 citing papers in PubMed, 1 synthesis or guideline pooled it.
- The efficacy and safety of tyrosine kinase inhibitors in the treatment of advanced or metastatic chordoma: a single-arm meta-analysis.Neurosurgical review · 2025Pooled it
- Development and validation of MRI-based radiomics models for predicting recurrence in patients with spine and pelvis chordomas.European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society · 2026Article
- Institutional experience and pooled survival analysis of chemotherapy based multimodal management in poorly differentiated chordoma.Discover oncology · 2026Article
- Proton-Based Re-Irradiation of Locally Recurrent Pediatric Brain Tumors in Close Proximity to the Brainstem.International journal of particle therapy · 2026Article
- Characteristics and survival outcomes in pediatric patients with spinal chordomas: insights from the National Cancer Database and review of the literature.Journal of neuro-oncology · 2025Review
- Proton beam therapy for clival chordoma: Optimising rare cancer treatments in Australia.Journal of medical radiation sciences · 2024Review
- How proton therapy fits into the management of adult intracranial tumors.Neuro-oncology · 2024Review
Corrections and comments
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Authors and funding
25 authors.
Funding
Abstract
backgroundData on clinical outcomes for base of skull (BOS) chordomas in the pediatric population is limited. We report patient outcomes after surgery and proton radiotherapy (PRT).
methodsPediatric patients with BOS chordomas were treated with PRT or combined proton/photon approach (proton-based; for most, 80% proton/20% photon) at the Massachusetts General Hospital from 1981 to 2021. Endpoints of interest were overall survival (OS), disease-specific survival, progression-free survival (PFS), freedom from local recurrence (LC), and freedom from distant failure (DC).
resultsOf 204 patients, median age at diagnosis was 11.1 years (range, 1-21). Chordoma location included 59% upper and/or middle clivus, 36% lower clivus, 4% craniocervical junction, and 1% nasal cavity. Fifteen (7%) received pre-RT chemotherapy. Forty-seven (23%) received PRT, and 157 (77%) received comboRT. Median total dose was 76.7 Gy (RBE) (range, 59.3-83.3). At a median follow-up of 10 years (interquartile range, 5-16 years), 56 recurred. Median OS and PFS were 26 and 25 years, with 5-, 10-, and 20-year OS and PFS rates of 84% and 74%, 78% and 69%, and 64% and 64%, respectively. Multivariable actuarial analyses showed poorly differentiated subtype, radiographical progression prior to RT, larger treatment volume, and lower clivus location to be prognostic factors for worse OS, PFS, and LC. RT was well tolerated at a median follow-up of 9 years (interquartile range, 4-16 years). Side effects included 166 patients (80%) with mild/moderate acute toxicities, 24 (12%) patients with late toxicities, and 4 (2%) who developed secondary radiation-related malignancies.
conclusionThis is the largest cohort of BOS chordomas in the literature, pediatric and/or adult. High-dose PRT following surgical resection is effective with low rates of late toxicity.
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