Abstract

IntroductionOptic pathway gliomas (OPGs), also known as Visual Pathway Gliomas, are insidious, debilitating tumours. They are most commonly WHO grade 1 pilocytic astrocytomas and frequently occur in patients with neurofibromatosis type 1. The location of OPGs within the optic pathway typically precludes complete resection or optimal radiation dosing, hence outcomes remain poor compared to many other low-grade gliomas. The aim of this systematic review was to formulate a comprehensive list of all current ongoing clinical trials that are specifically looking at clinical care of OPGs in order to identify trends in current research and provide an overview to guide future research efforts.MethodsThis systematic review was conducted in line with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. The Cochrane Controlled Register of Trials (CENTRAL) and ClinicalTrials.gov were searched. Inclusion and exclusion criteria were applied and final results were reviewed.Results501 clinical trials were identified with the search strategy. All were screened and eligible studies extracted and reviewed. This yielded 36 ongoing clinical trials, 27 of which were pharmacological agents in phase I-III. The remaining trials were a mixture of biological agents, radiation optimisation, diagnostic imaging, surgical intervention, and a social function analysis.ConclusionOPG is a complex multifaceted disease, and advances in care require ongoing research efforts across a spectrum of different research fields. This review provides an update on the current state of research in OPG and summarises ongoing trials.

Highlights

  • Optic pathway gliomas (OPGs), known as Visual Pathway Gliomas, are insidious, debilitating tumours

  • The aim of this systematic review was to formulate a comprehensive list of all current ongoing clinical trials that were looking at a clinical care of OPGs in order to identify trends in current research and provide an overview of the field to guide future research efforts

  • Five hundred one clinical trials were identified with the search strategy described

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Summary

Introduction

Optic pathway gliomas (OPGs), known as Visual Pathway Gliomas, are insidious, debilitating tumours They are most commonly WHO grade 1 pilocytic astrocytomas and frequently occur in patients with neurofibromatosis type 1. OPGs most commonly arise at the chiasmatic-hypothalamic region but can arise anywhere along the optic pathway [3] Their intimate relationship to the optic apparatus, hypothalamus, ventricular system and brain parenchyma can result in a variety of clinical sequelae including visual loss, endocrinopathies and hypothalamic dysfunction, developmental/neuropsychological disorders, hydrocephalus and focal neurological deficits. This complexity of symptomatology, and the close relationship to key structures make the treatment of OPG challenging. A summary of each of the ongoing clinical trials will be presented to facilitate a rapid review of the field

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