title: Ependymoma in children under the age of 3 years: a report from the Canadian Pediatric Brain Tumour Consortium.
abstract: The determination of optimal therapy for ependymoma (EP) in infants is ongoing. We describe the incidence, management and outcomes of Canadian infants with EP to discern potential future research questions. Of 579 cases registered in a national database of children <36 months of age diagnosed with a brain tumor from 1990 to 2005, inclusive, 75 (13 %) were EP. These cases were analyzed. A mean annual age-adjusted incidence rate of 4.6 per 100,000 children years was calculated. The male:female ratio was 1.77. Of the tumors, 80 % were infratentorial in location, 67 % were WHO grade II histology, and 29 % were metastatic at diagnosis. All patients underwent a surgical procedure. A complete resection of the tumor was achieved in 56 % of the cases; 43 % of these patients survive while 36 % of the patients with tumors less than completely resected survive. Initial therapy consisted of surgery alone in 23 % of patients, or surgery plus chemotherapy (37 %), radiation therapy (RT; 19 %), or both (21 %). Any use of RT increased with patient age. The 5-year EFS rates for patients in each of the four treatment groups was 22, 11.5, 46.2 and 64.8 %, respectively. For all patients the median survival was 63 ± 6 months and 5-year overall survival was 55 ± 6 %. Patients treated with surgery and chemotherapy alone were younger and had a lower rate of survival than older patients who were more often treated with radiation therapy containing regimens. Further study is needed to determine which patients are optimally served with these treatment modalities.
authors: Pediatric Oncology and Blood and Marrow Transplant, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, T3B 6A8, Canada.
authors: Eve
authors: E
authors: Purdy
authors: Donna L
authors: DL
authors: Johnston
authors: Ute
authors: U
authors: Bartels
authors: Chris
authors: C
authors: Fryer
authors: Anne-Sophie
authors: AS
authors: Carret
authors: Bruce
authors: B
authors: Crooks
authors: David D
authors: DD
authors: Eisenstat
authors: Lucie
authors: L
authors: Lafay-Cousin
authors: Valerie
authors: V
authors: Larouche
authors: Beverly
authors: B
authors: Wilson
authors: Shayna
authors: S
authors: Zelcer
authors: Mariana
authors: M
authors: Silva
authors: Eric
authors: E
authors: Bouffet
authors: Daniel
authors: D
authors: Keene
authors: Douglas R
authors: DR
authors: Strother
mesh_terms: D001932:Brain Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy; D002170:Canada / Q000453:epidemiology; D002675:Child, Preschool; D016208:Databases, Factual; D004806:Ependymoma / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010372:Pediatrics / Q000706:statistics & numerical data