Optic pathway and hypothalamic glioma, old problems, new paradigms
Optic pathway/hypothalamic gliomas (OP/HGs) are low-grade tumours that account for 3-5% of all pediatric CNS tumours. The majority of cases are diagnosed before the age of 20, and 75% before the age of 10 years. Presenting symptoms are essentially visual. Treatment is recommended when the tumour thr...
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Main Authors: | , |
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Format: | Book |
Published: |
Elsevier,
2023-06-01T00:00:00Z.
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Online Access: | Connect to this object online. |
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Summary: | Optic pathway/hypothalamic gliomas (OP/HGs) are low-grade tumours that account for 3-5% of all pediatric CNS tumours. The majority of cases are diagnosed before the age of 20, and 75% before the age of 10 years. Presenting symptoms are essentially visual. Treatment is recommended when the tumour threatens the patient's vision. Even though they are low-grade tumours, their course can be unpredictable and their management is often challenging. As OP/HGs are generally not amenable to complete resection, surgery has a limited role in the management. Chemotherapy was essentially the treatment of choice in the past but in most series, the 5-year event-free survival was between 30 and 50%. Targeted therapies are starting to gain ground over recent years with a better understanding of the underlying molecular alterations. |
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Item Description: | 2468-1245 10.1016/j.phoj.2023.04.003 |