Central nervous system tumors in Chinese children under the age of three: a population study by the Hong Kong Paediatric Haematology/Oncology Study Group

Abstract

The management of CNS tumors in children below the age of three represents special challenge to pediatric oncologists with distinctive epidemiology, treatment considerations and prognosis. Population-based epidemiological data on this particular patient group is lacking in Chinese. We reviewed the population-based pediatric tumor registry of the Hong Kong Paediatric Haematology/Oncology Study Group between 1999 and 2011. Eighty-one children with primary CNS tumors from 0-3 years were identified (annual incidence: 4.16 cases per 100,000). Forty-one (50.6%) were male and mean duration of FU was 94 months (±8.1). Primary tumors were infratentorial in 43 (53.1%). The tumor types in decreasing frequency were astrocytoma (n = 17), medulloblastoma (n = 16), ependymoma (n = 13), CPT (n = 7), PNET (n = 7), ATRT (n = 6), GCT (n = 5), craniopharyngioma (n = 4) and ganglioglioma (n = 3). Three patients presented antenatally. Treatment included surgery in 82.7%, chemotherapy in 50.6% and radiotherapy in 25.9%. There were 29 deaths (35.8%) and 19 relapses (23.5%) during the review period with the 1y-OS, 5y-OS, 1y-EFS and 5y-EFS being 79.4% (±4.6), 63.5% (±5.9), 68.9% (±5.3) and 52.5% (±5.9) respectively. Significantly better OS and EFS were observed in patients who received gross-total resection but those with high-grade tumors, antenatal diagnosis or ATRT/PNET had worse outcome. Survival did not differ with age. Comparison with statistics from other studies revealed higher rates of embryonal tumor, GCT and craniopharyngioma in Hong Kong Chinese. Disease outcome appeared to be better in our cohort comparing to previous reports probably due to the higher proportion of GCT locally.