LGG-39. EPIDEMIOLOGY, MANAGEMENT AND OUTCOME OF PAEDIATRIC LOW GRADE GLIOMA IN HONG KONG

Abstract

METHOD: Retrospective cohort for patients age 0 to 18 years old from January 1999 to Aug 2016 diagnosed to have low grade glioma. RESULT: Fifty-five patients diagnosed with pLGG with n=50 (91%) got a tissue diagnosis all showing astrocytoma WHO grade 1. Sites included cerebellum (n=29, 53%), cerebrum (n=10, 18%), brainstem (n=7, 13%), optic pathway (n=8, 15%) and spinal (n=1, 2%). Four patients (7%) have multi-focal tumours. Thirty-three (60%) patients received surgery alone and remained alive. Chemotherapy adopted including CCNU/CDDP/VCR, VCR/CPM/CDDP/VP-16, BLE/VP-16/CDDP, PCZ/CCNU/VCR, TMZ, CBDCA/VCR. Six (11%) received focal radiotherapy. One had NF-1 with thalamic lesion treated by surgery alone. One had TSC treated with mTOR inhibitor. Seven (13%) had progressive disease after chemotherapy and six remained alive. One had refractory brainstem tumour despite varies chemotherapy. The tumour had BRAF-KIAA1549 fusion and patient achieved stable disease after MEK-inhibitor (currently only 3 months). Three patients (5%) died. The first had parietal tumour at 12 years old received CCV and focal radiotherapy 54Gy, died from disease progression 5.8 years later. The second presented with diencephalic syndrome at 8 months old, received VCR/CPM/CDDP/VP-16 with stable disease, then died from status epilepticus 1.7 years later. The third had optic pathway glioma diagnosed at 2 months old, died from pneumonia 5.6 years from diagnosis. Median follow-up was 7.2 years (0.4-18). OS at 5 years was 94.1%. CONCLUSION: pLGG had excellent prognosis. Many patients treated successfully with surgery alone. New novel therapy from advanced molecular study might provide better prognosis and spare some patients from radiotherapy.