OPTIC PATHWAY GLIOMA TREATMENT CHALLENGES OVER THE TIME – ONE INSTITUTION EXPERIENCE: : CASE SERIES

Objectives. To understand better the natural history of optic pathway glioma (OPG) in patients with
neurofibromatosis type 1 (NF-1), and to evaluate the current recommended guidelines for monitoring and
follow-up of OPG and the impact of chemotherapy in this population.
Materials and Methods. This retrospective case series included patient records of ophthalmological
symptoms, MRI data, and chosen treatment method of OPG from the 2002–2022 year. Children younger
than 18 years diagnosed with OPG were included in the study.
Results. In total, 56 patient medical records were conducted. OPG was diagnosed in 22 patients. Out
of 22 patients with OPG 20 underwent the ‘’watch and wait” strategy. 2 of the OPG patients underwent
chemotherapy. In one of the cases, chemotherapy included vincristine alone, and in the second case initial
cycle also included one-time administration of actinomycin D. Both patients presented with large spreading
OPG. After chemotherapy tumor growth has significantly decreased allowing us to save the globe and not
initiate surgical treatment or radiotherapy of the tumor
Conclusions. OPG is rare and difficult to treat tumor. Early recognition of present OPG and visual
deterioration is crucial for the initiation of chemotherapy. Historically, radiation therapy was the treatment of
choice for OPG, but it brought up severe complications including secondary tumors and Moya-Moya disease
in patients with NF-1
Children with radiologically notable OPG must be evaluated by a multidisciplinary
team – oncologist, ophthalmologist, and radiologist. In Latvia to achieve this kind of evaluation all children
with NF-1 should be observed at Children’s Clinical University Hospital.