Radiological classification of optic pathway gliomas: experience of a modified functional classification system

Abstract

Optic pathway gliomas (OPGs) in childhood are associated with neurofibromatosis type 1 (NF1) and since 1958 have been classified anatomically using the Dodge classification (DC). MR scanning permits a more detailed anatomical description than can be classified by this historical system. A modified Dodge classification (MDC) has been applied to MRI scans from a cohort of 72 patients (36.1% NF1-positive) from 4 centres participating in an international clinical trial. The MDC was feasible, applicable and more detailed than the original DC. NF1-positive cases more commonly involved both optic nerves (p = 0.021) and other multiple locations (p = 0.001). NF1-negative tumours more commonly involved the central chiasm (p = 0.005) and hypothalamus (p = 0.003). Fewer hypothalamus-positive tumours were associated with optic nerve involvement (p = 0.009), whereas more were associated with central chiasm involvement (p<0.001). From diagnosis to follow-up, there was concordance between DC and MDC in 51/72 cases (70.8%). The MDC is therefore proposed for use in clinical trials of new treatments for OPGs.