PAEDIATRIC-12 MANAGEMENT OF PAEDIATRIC LOW-GRADE GLIOMA IN CAPE TOWN, SOUTH AFRICA

Abstract

Abstract OBJECTIVE To assess the management of paediatric low-grade gliomas by the combined neuro-oncology services of the University of Cape Town. METHODS A retrospective analysis was performed on the folders of patients diagnosed at the Red Cross War Memorial Children’s Hospital (RCWMCH) and Groote Schuur Hospital (GSH) between 2000 and 2022. The data includes 2 patient cohorts, the first cohort being patient data collected from 2000-2016 and the second from 2017-2022. RESULTS A total of 127 patients, aged 0.38-13.76 years (mean 5.71) were treated at RCWMCH and GSH. Predisposition syndromes observed included Neurofibromatosis 1 11, Tuberous sclerosis 6, and Trisomy 21 1. The most common sites were the cerebellum 27%, hypothalamus 17%, cerebrum 12%, and optic pathway 13%. Metastatic disease was noted in 12 patients (9%). Gross total resection (or debulking) was performed in 67 (53%) of patients, 40 had biopsy only, and 20 had no surgery (mostly OPGs). Histology was predominantly Juvenile Pilocytic Astrocytoma 70%, Diffuse Astrocytoma 9%, Low-Grade Glioma Nos 8%,5% Subependymal Giant cell Astrocytoma, and 5% Pilomyxoid Astrocytoma. BRAF v600E mutation was tested in 17 patients: all were negative. Forty-four (35%) received chemotherapy and 36 (82%) as first-line therapy with vincristine and carboplatin as the most used agents. Newer therapies Bevacizumab/irinotecan and Trametinib were recently used in 10 patients as third or fourth-line regimens. There was an increase in chemotherapy usage for patients with irresectable tumours between the 2 cohorts, from 27% in the first cohort to 49% in the second. Thirty (23.6%) patients received radiotherapy and of those 8 were in an upfront setting. There was no difference in the 5-year OS between the 2 cohorts, 84% in the first and 85.7% in the second. CONCLUSION Multi-disciplinary treatment of paediatric LGG in LMIC is essential. Patterns of management have changed without affecting outcomes.