title: Central nervous system tumors in chinese children under the age of 3: a population study.
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.
authors.affiliation: *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.
authors.forename: Anthony Pak-Yin
authors.initials: AP
authors.lastname: Liu
authors.forename: Matthew Ming-Kong
authors.initials: MM
authors.lastname: Shing
authors.forename: Hui-Leung
authors.initials: HL
authors.lastname: Yuen
authors.forename: Chak-Ho
authors.initials: CH
authors.lastname: Li
authors.forename: Siu-Cheung
authors.initials: SC
authors.lastname: Ling
authors.forename: Chung-Wing
authors.initials: CW
authors.lastname: Luk
authors.forename: Shau-Yin
authors.initials: SY
authors.lastname: Ha
authors.forename: Chi-Kong
authors.initials: CK
authors.lastname: Li
authors.forename: Godfrey Chi-Fung
authors.initials: GC
authors.lastname: Chan
mesh_terms: D016543:Central Nervous System Neoplasms / Q000145:classification / Q000453:epidemiology* / Q000401:mortality / Q000628:therapy; D002675:Child, Preschool; D002681:China / Q000453:epidemiology; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology* / Q000401:mortality / Q000628:therapy; D011379:Prognosis; D011446:Prospective Studies; D015996:Survival Rate