Surgery and proton radiation therapy for pediatric base of skull chordomas: Long-term clinical outcomes for 204 patients
Author:
Ioakeim-Ioannidou Myrsini1, Niemierko Andrzej1, Kim Daniel W1, Tejada Athena1, Urell Tobias1, Leahy Shannon1, Adams Judy1, Fullerton Barbara1, Nielsen G Petur2, Hung Yin P2ORCID, Shih Angela R2, Patino Manuel3, Buch Karen3, Rincon Sandra3, Kelly Hilary3, Cunnane Mary Beth3, Tolia Maria4, Widemann Brigitte C5, Wedekind Mary F5, John Liny5, Ebb David6, Shin John H7, Cote Gregory8, Curry William7, MacDonald Shannon M1
Affiliation:
1. Department of Radiation Oncology, Massachusetts General Hospital , Boston, Massachusetts , USA 2. Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston , Massachusetts , USA 3. Department of Radiology, Massachusetts General Hospital , Boston, Massachusetts , USA 4. Department of Radiotherapy, School of Medicine, University of Crete , Heraklion , Greece 5. Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda , MD , USA 6. Department of Pediatric Hematology-Oncology, Massachusetts General Hospital , Boston, Massachusetts , USA 7. Department of Neurosurgery, Massachusetts General Hospital , Boston, Massachusetts , USA 8. Department of Hematology-Oncology, Massachusetts General Hospital , Boston, Massachusetts , USA
Abstract
Abstract
Background
Data 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).
Methods
Pediatric 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).
Results
Of 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.
Conclusion
This 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.
Funder
American Radium Society American Society for Radiation Oncology Harvard Medical School
Publisher
Oxford University Press (OUP)
Subject
Cancer Research,Neurology (clinical),Oncology
|
|