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Management of optic pathway and chiasmatic-hypothalamic gliomas in children with radiation therapy
1Department of Radiation Oncology, Ankara University Faculty of Medicine, Turkey.
Insights
Radiation therapy effectively manages childhood optic pathway and chiasmatic-hypothalamic gliomas, improving vision and preventing tumor progression. Absence of neurofibromatosis correlated with better survival outcomes in these rare brain tumors.
Area of Science:
- Pediatric Oncology
- Radiation Oncology
- Neuro-oncology
Background:
- Optic pathway gliomas and chiasmatic-hypothalamic gliomas are rare pediatric brain tumors.
- Management of these tumors presents unique challenges in pediatric neuro-oncology.
Purpose of the Study:
- To present the management experience of radiation therapy for childhood optic pathway and chiasmatic-hypothalamic gliomas.
- To evaluate the efficacy of radiation therapy in this patient cohort.
Main Methods:
- Retrospective analysis of 33 children treated with radiation therapy between 1973-1994.
- Tumor types included optic pathway gliomas (n=24) and chiasmatic-hypothalamic gliomas (n=9).
- Radiation doses typically involved 50 Gy in 2 Gy daily fractions, with follow-up up to 16.1 years.
Main Results:
- Overall survival at 5 and 10 years was 93% and 79%, respectively.
- Progression-free survival at 5 and 10 years was 82% and 77%, respectively.
- Absence of neurofibromatosis was associated with significantly better progression-free and cause-specific survival.
Conclusions:
- Radiation therapy is an effective treatment for optic pathway and chiasmatic-hypothalamic gliomas in children.
- It aids in vision stabilization/improvement and prevents tumor progression.
- Patient outcomes are influenced by the presence or absence of neurofibromatosis.
Background And Purpose:
Optic pathway and chiasmatic-hypothalamic gliomas are rare childhood tumors. This study presents the experience in management of these tumors with radiation therapy.
Materials And Methods:
Thirty-three children with the diagnosis of optic pathway and chiasmatic-hypothalamic gliomas were treated with radiation therapy from 1973 through 1994 in the Department of Radiation Oncology at Ankara University Faculty of Medicine. Twenty-four children had optic pathway gliomas and nine had chiasmatic-hypothalamic gliomas. Evidence of neurofibromatosis was present in six children. Subtotal resection was performed in 22 children and a biopsy in seven. The most common prescription for total tumor dose was 50 Gy, delivered in 2 Gy daily fractions. Follow-up ranged from 0.5 to 16.1 years (mean, 13.6 years).
Results:
Overall, progression-free and cause-specific survival probabilities for the entire group were 93%, 82% and 93%, respectively, at 5 years and 79%, 77% and 88%, respectively, at 10 years. Differences in overall, progression-free and cause-specific survival probabilities between optic pathway and chiasmatic-hypothalamic gliomas were not statistically significant. Absence of evidence of neurofibromatosis correlated with significantly better progression-free and cause-specific survival probabilities.
Conclusion:
Radiation therapy is effective in stabilization or improvement of vision and prevention of tumor progression in both optic pathway and chiasmatic-hypothalamic gliomas.