Management of optic-hypothalamic gliomas in children: still a challenging problem

Optic pathway-hypothalamic gliomas (OPHGs) are rare, often unresectable tumors that mostly occur in childhood. Their biological behavior is unpredictable, although they tend to follow an aggressive clinical course in infants and a benign course in children with neurofibromatosis type 1. Optimal management is still controversial. Nonprogressing OPHGs are usually followed by surveillance alone. Surgery is advocated for progressing tumors to decompress the optic pathways, obtain a quick relief from intracranial hypertension and allow histologic examination (when needed). The current trend is in favor of conservative surgical behavior, except for resectable tumors. Chemotherapy is increasingly used in the management of OPHGs, especially in infants, to delay radiotherapy. Carboplatin and vincristine are the most frequently used drugs, although several chemotherapeutic agents in different combinations are currently employed with good results. Radiotherapy is utilized in children over 5 years of age as an adjuvant or as an alternative to surgery. The prognosis of OPHGs is quite good, with regard to the overall survival rate (70-100% at 5 years), but less favorable in terms of late morbidity.