Optic pathway tumors in children.

Tumors of the optic pathways are sub-divided in this review into those that arise in one or both optic nerves anterior to the chiasm (optic nerve tumors); those that arise within the chiasm and do not extend significantly into the hypothalamus (optic chiasmatic tumors) and the large exophytic tumors that involve both the optic chiasm and the hypothalamus to a lesser or greater degree (optic chiasmatic/hypothalamic tumors). The management of optic chiasmatic gliomas is controversial, partly related to failure to separate out chiasmatic tumors from the chiasmatic/hypothalamic tumors. The optic nerve tumors are reviewed briefly, since they rarely extend intracranially. Chiasmatic tumors tend to be associated with NF1 and to behave almost like hamartomas. Close observation is usually the most appropriate management. On the other hand, chiasmatic/hypothalamic tumors grow like typical neoplasms. The tumors are almost uniformly low grade astrocytomas, but growth rates may berapid, especially in infants. Modern management has trended away from radical surgical resection, which has significant morbidity, to chemotherapy as the first line of treatment. In this review, the clinical presentation and management of different types of optic pathway tumors are discussed.