Abstract

Objective: Proton beam therapy (PBT) may provide good local control for skull base chordoma and reduced toxicities, especially for pediatric patients. Methods: We evaluated the efficacy and safety of hyperfractionated high-dose PBT in6 pediatric patients with newly-diagnosed skull basechordoma who were treated with PBT at our institute from 2011 to 2015. The patients were 5 males and one female, and the median age was 9 years old (range: 5 - 13). All patients received surgery before PBT. The median period between surgery and PBT was 57 days (range: 34 - 129 days). The treatment dose was 78.4 GyE in 56 fractions (twice per day). Results: All patients received PBT without severe acute toxicity. The median follow-up period was 27 months (range: 21 - 71 months). At the last follow-up, all patients were alive and all tumors were well controlled. Acute and late toxicities were generally acceptable, with only grade 1 and 2 events. Late toxicities included growth hormone abnormality and cortical hormone abnormality. One patient needed growth hormone and cortical hormone replacement therapy. Conclusion: Although the number of pediatric patients was small, our overall findings in the 6 cases indicate that hyperfractionated high-dose PBT is safe and effective for pediatric patients with skull base chordoma.

Highlights

  • Chordomas are rare primary bone tumors that originate from remnants of the notochord

  • We evaluated the efficacy and safety of hyperfractionated high-dose Proton beam therapy (PBT) in6 pediatric patients with newly-diagnosed skull basechordoma who were treated with PBT at our institute from 2011 to 2015

  • We found that hyperfractionated high-dose PBT (78.4 GyE in 56 fractions, 2 fractions per day) for skull base chordoma gave a 5-year local control rate of 70% to 80% in adults [10]

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Summary

Introduction

Chordomas are rare primary bone tumors that originate from remnants of the notochord. Postoperative radiotherapy is standard therapy, but the radiation dose for a skull base chordomais limited by normal tissue around the tumor, such as the brainstem, optic chiasm and temporal lobe. This limits of conventional radiotherapy considered about 50 Gy in 25 fractions, which is insufficient and results in a poor local control rate of 50% or less [3]. We found that hyperfractionated high-dose PBT (78.4 GyE in 56 fractions, 2 fractions per day) for skull base chordoma gave a 5-year local control rate of 70% to 80% in adults [10]. The information of safety and effectiveness of PBT for pediatric skull base chordoma is still insufficient

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