Optic glioma in children: clinical presentation, diagnosis, and management

Optic gliomas are slow-growing astrocytic tumors that frequently occur in children. The general treatment approach for optic gliomas should be based on tumor location, patient age, and NF1 status. Optic gliomas respond well to chemotherapy; it is generally preferred over radiotherapy, especially for children under five years and those with NF1. Chemotherapy primarily results in stabilization of tumor volume, and this effect is often temporary. In some cases, a second line treatment may be required. Radiotherapy may be an option for treating patients with sporadic optic gliomas. Targeted therapy is a promising strategy.

This review provides an overview of clinical findings, diagnosis, current standard practices, emerging targeted treatments, and ongoing clinical trials for optic gliomas in children. The literature reviewed herein was identified through a non-systematic search of published articles, clinical trial registries, and authoritative guidelines. Selection was based on relevance, clinical significance, and contribution to understanding current and emerging management strategies.

Because BRAF alterations are observed in most pilocytic astrocytomas, treatments targeting the MAPK pathway play an important role in the management of optic gliomas. Inhibition of the MAPK signaling pathway represents a potential advance in the treatment of optic gliomas.