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

Abstract

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.

The role of surgery in optic pathway/hypothalamic gliomas in children

“…They represent 2%-7% of all pediatric intracranial tumors, with 65% of these lesions presenting in children younger than 5 years of age. 2,17,21,26,31,38 Tumor presentation is often due Object. Optic pathway/hypothalamic gliomas (OPHGs) are generally benign tumors situated in an exquisitely sensitive brain region.…”

The role of early intra-operative MRI in partial resection of optic pathway/hypothalamic gliomas in children

“…However, it was acknowledged that selected cases were amenable to attempted resection or debulking with low risk" [35]. It is clear that there is a role for surgery for the initial diagnosis, for gaining control of tumour progression, especially in those aged less than 5 years where secondary chemotherapy can avoid the need for radiation exposure [32], and for the relief of mass effect [24] . Additionally, it was stated that those tumours that are large, exophytic, cystic, or hypothalamic would be particularly amenable to surgical management [35].…”

“…Optic pathway/hypothalamic gliomas (OPHG) account for approximately 2-7% of intracranial tumours in the paediatric population, with 65% of these tumours found in children less than 5 years of age [2,11,16,24,28,33]. They comprise a broad spectrum of tumour sizes and localisations, and typically follow an unpredictable course.…”

“…They comprise a broad spectrum of tumour sizes and localisations, and typically follow an unpredictable course. Presentation tends to be due to compression of the optic pathways and diencephalon, but obstructive hydrocephalus is also a common feature [2,24]. Histologically, OPHGs are nearly always low-grade tumours, with pilocystic astrocytomas (WHO grade I) accounting for the majority, along with a proportion of pilomyxoid astrocytomas (WHO grade II) [5,7,9,11,17,22,30].…”

“…Histologically, OPHGs are nearly always low-grade tumours, with pilocystic astrocytomas (WHO grade I) accounting for the majority, along with a proportion of pilomyxoid astrocytomas (WHO grade II) [5,7,9,11,17,22,30]. A strong association with Neurofibromatosis Type 1 (NF1) is observed, which tends to correlate with a more benign course [6,10,11,13,17,[24][25]28,34].…”