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Base of skull and cervical spine chordomas in children treated by high-dose irradiation
V Benk1, N J Liebsch, J E Munzenrider
1Department of Radiation Oncology, Massachusetts General Hospital, Boston 02114.
Insights
High-dose irradiation for pediatric chordomas, including base of skull and cervical spine tumors, shows acceptable outcomes. Children with cervical spine chordomas had worse survival, but overall treatment-related morbidity was manageable.
Area of Science:
- Pediatric Oncology
- Radiation Oncology
- Neurosurgery
Background:
- Chordomas are rare bone tumors that can occur at the base of the skull or in the cervical spine.
- Treatment of pediatric chordomas is challenging due to their location and potential for long-term sequelae.
Purpose of the Study:
- To evaluate the efficacy and safety of high-dose irradiation in children with base of skull or cervical spine chordomas.
- To compare outcomes between different tumor locations.
Main Methods:
- Eighteen pediatric patients (4-18 years) with chordomas received fractionated high-dose postoperative radiation.
- Treatment utilized mixed photon and 160 MeV proton beams, with a median tumor dose of 69 Cobalt Gray-equivalent (CGE).
Main Results:
- The 5-year actuarial survival was 68%, and disease-free survival was 63% after a median follow-up of 72 months.
- Cervical spine chordomas were associated with significantly worse survival compared to base of skull lesions (p = 0.008).
- Treatment-related morbidities were acceptable, including two cases of growth hormone deficit, one temporal lobe necrosis, and one case of temporalis muscle fibrosis.
Conclusions:
- Pediatric chordomas exhibit similar behavior to adult chordomas regarding treatment response and survival.
- High-dose irradiation is a viable treatment option for pediatric chordomas, with acceptable toxicity profiles.
- Tumor location is a critical prognostic factor in pediatric chordoma treatment outcomes.
Purpose:
To evaluate the outcome of children with base of skull or cervical spine chordomas treated by high dose irradiation.
Methods And Materials:
Eighteen children, 4 to 18 years of age, with base of skull or cervical spine chordomas, received fractionated high-dose postoperative radiation using mixed photon and 160 MeV proton beams. The median tumor dose was 69 Cobalt Gray-equivalent (CGE) with a 1.8 CGE daily fraction.
Results:
The median follow-up was 72 months. The 5-year actuarial survival was 68% and the 5-year disease-free survival (DFS) was 63%. The only significant prognostic factor was the location: patients with cervical spine chordomas had a worse survival than those with base of skull lesions (p = 0.008). The incidence of treatment-related morbidity was acceptable: two patients developed a growth hormone deficit corrected by hormone replacement, one temporal lobe necrosis, and one fibrosis of the temporalis muscle, improved by surgery.
Conclusion:
Chordomas in children behave similarly to those in adults: children can receive the same high-dose irradiation as adults with acceptable morbidity.