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Central nervous system tumors in chinese children under the age of 3: a population study
Anthony Pak-Yin Liu1, Matthew Ming-Kong Shing, Hui-Leung Yuen
1*Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, Li Ka Shing Faculty of Medicine, The University of Hong Kong †Department of Paediatrics, Prince of Wales Hospital, The Chinese University of Hong Kong ‡Department of Paediatrics, Queen Elizabeth Hospital §Department of Paediatics and Adolescent Medicine, Tuen Mun Hospital ∥Department of Paediatrics and Adolescent Medicine, Princess Margaret Hospital, Hong Kong Special Administrative Region, China.
Insights
This study analyzes pediatric central nervous system tumors in Hong Kong, finding higher rates of embryonal tumors and germ cell tumors. Outcomes were better with gross total resection, especially for germ cell tumors.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Epidemiology
Background:
- Management of central nervous system tumors in children under 3 presents unique challenges.
- Population-based epidemiological data for this age group is scarce in Chinese populations.
Purpose of the Study:
- To investigate the epidemiology, treatment, and outcomes of primary central nervous system tumors in children aged 0-3 years in Hong Kong.
- To compare findings with international data and identify factors influencing survival.
Main Methods:
- Retrospective review of the Hong Kong pediatric tumor registry (1999-2011).
- Inclusion of 81 children aged 0-3 years with primary central nervous system tumors.
- Analysis of tumor types, treatments, survival rates (overall survival and event-free survival), and prognostic factors.
Main Results:
- An annual incidence of 4.16 cases per 100,000 was observed.
- Common tumor types included astrocytoma, medulloblastoma, and ependymoma; higher rates of embryonal tumors, germ cell tumors (GCT), and craniopharyngioma were noted compared to other studies.
- Gross total resection correlated with significantly better overall survival and event-free survival.
- High-grade tumors, antenatal diagnosis, and specific tumor types (atypical teratoid rhabdoid tumor/primitive neuroectodermal tumor) were associated with worse outcomes.
Conclusions:
- The study provides crucial epidemiological data for young children with central nervous system tumors in Hong Kong.
- Higher rates of embryonal tumors and GCTs characterize this cohort.
- Improved outcomes were linked to surgical resection, with GCTs showing a potentially better prognosis in this population.
Abstract:
The management of central nervous system tumors in children below the age of 3 years represents a 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 in Hong Kong between 1999 and 2011. Eighty-one children with primary central nervous system tumors from 0 to 3 years of age were identified (annual incidence: 4.16 cases per 100,000). Forty-one (50.6%) were male and the mean duration of follow-up 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), choroid plexus tumor (n=7), primitive neuroectodermal tumor (n=7), atypical teratoid rhabdoid tumor (n=6), germ cell tumor (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 1-year overall survival (OS), 5-year OS, 1-year event-free survival (EFS), and 5-year 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 atypical teratoid rhabdoid tumor/primitive neuroectodermal tumor 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.
