Regression and pseudoprogression of pediatric optic pathway glioma in patients treated with proton beam therapy

We examined regression patterns in pediatric optic pathway gliomas (OPGs) after proton beam therapy (PBT) and evaluated local control and visual outcomes.

A total of 42 brain magnetic resonance imaging (MRI) scans from seven consecutive sporadic OPGs that were initially treated with chemotherapy and received PBT between June 2007 and September 2016 at the National Cancer Center, Korea were analyzed. Patients underwent brain MRI regularly before and after PBT. Total tumor, cystic lesion, and solid enhancing lesion area delineation and volume calculations were performed on gadolinium-enhanced T1-weighted MRI using Eclipse version 13, Varian.

The median follow-up period after PBT was 70 months (range 47-88). The median age at the time of PBT was 7 years (range 4-16) and the median duration of chemotherapy before referral to PBT center was 25 months (range 3-70). The median time to the greatest increase in cystic volume was 32 months (range 12-43) after PBT. Solid enhancing lesion volume gradually decreased throughout the follow-up period. On an individual basis, total volume change was varied. However, on average, it regressed, although at a slower rate than solid enhancing lesion volume did. The local control rate was 85.7% (5-year progression-free survival rate, 80%; 5-year overall survival rate, 100%) and the rate of vision preservation was 71.4% (five of seven patients).

The regression patterns in pediatric OPGs after PBT involve significant cystic change. Therefore, total volume is not appropriate for evaluating response. Care by a multidisciplinary team is necessary to manage clinical symptoms related to radiologic changes.