Epidemiology Study of Uncommon Childhood Brain Tumors in Asian Children

Abstract

Our local registry identified 656 brain tumours from Jan 1999 to Dec 2018, (incidence: 29.8/yr/million). Other from Glioma, Medulloblastoma/PNET, Germ Cell tumours, Ependymoma, the remaining rarer tumours accounted for 18% (n=118). The 7 more common groups are: craniopharyngioma(n=28); ATRT(n=18); choroid plexus papilloma/CA(n=12); Ganglioglioma(n=11); ETMR(n=7); DNET(n=7); meningioma(n=6). Their respective incidences are 1.27; 0.81; 0.55; 0.5; 0.32; 0.32 0.27/yr/million. For craniopharyngioma, M:F=15:13 and median age was 7.4yrs (2mons-16.5yrs). 12/28 children had surgery alone and 13/28 had focal RT post-surgery with better outcome. 3 underwent intra-cystic interferon-beta also stable. For ATRT, M:F=7:8 and median age was 2.3yrs (4mos-14.2yrs). 2 had metastatic disease and 7/18 patients remained alive. For choroid plexus tumours, there were 7 papilloma, 2 atypia and 3 carcinoma. M:F=5:6 and median age was 1.5yrs (4mos-14yrs). All papilloma, 1/2 atypia and 1/3 carcinoma survived. For ganglioglioma, M:F=7:4 with median age of 5.5yrs (5mos-13.2yrs). They commonly presented with seizure and only one died (brainstem primary). The ETMR includes ependymoblastoma and medulloepithelioma, they had quite different clinical characteristics and outcome. 6/7 DNET had convulsion and M:F=6:1. Median age was 11.5yrs (2.66-14yrs). They all survived even if incompletely resected. For meningioma, 1/6 had germline mutation of NF-2 gene. M:F=3:3 and onset was >8yrs except the NF-2 patient. All survived but the NF-2 had multiple recurrences. 4 patients developed secondary meningioma due to irradiation but they were >18yrs so excluded. In summary, rarer forms of childhood brain tumours only accounted for <20% of all brain tumours and they had diverse presenting features and outcome.