A systematic review of ongoing clinical trials in optic pathway gliomas

Childs Nerv Syst. 2020 Sep;36(9):1869-1886. doi: 10.1007/s00381-020-04724-1. Epub 2020 Jun 18.


Introduction: Optic 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.

Methods: This 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.

Results: 501 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.

Conclusion: OPG 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.

Keywords: Glioma; Optic; Pathway; Systematic review; Trial; Visual.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Astrocytoma*
  • Humans
  • Neurofibromatosis 1* / therapy
  • Optic Nerve Glioma* / diagnostic imaging
  • Optic Nerve Glioma* / therapy