title: Survival in pediatric medulloblastoma: a population-based observational study to improve prognostication.
abstract: Medulloblastoma is the most common form of brain malignancy of childhood. The mainstay of epidemiological data regarding childhood medulloblastoma is derived from case series, hence population-based studies are warranted to improve the accuracy of survival estimates. To utilize a big-data approach to update survival estimates in a contemporary cohort of children with medulloblastoma. We performed a population-based retrospective observational cohort study utilizing the Surveillance, Epidemiology, and End Results Program database that captures all children, less than 20 years of age, between 1973 and 2012 in 18 geographical regions representing 28% of the US population. We included all participants with a presumed or histologically diagnosis of medulloblastoma. The main outcome of interest is survivors at 1, 5 and 10 years following diagnosis. A cohort of 1735 children with a median (interquartile range) age at diagnosis of 7 (4-11) years, with a diagnosis of medulloblastoma were identified. The incidence and prevalence of pediatric medulloblastoma has remained stable over the past 4 decades. There is a critical time point at 1990 when the overall survival has drastically improved. In the contemporary cohort (1990 onwards), the percentage of participants alive was 86, 70 and 63% at 1, 5 and 10 years, respectively. Multivariate Cox-Regression model demonstrated Radiation (HR 0.37; 95% CI 0.30-0.46, p < 0.001) and Surgery (HR 0.42; 95% CI 0.30-0.58, p < 0.001) independently predict survival. The probability of mortality from a neurological cause is <5% in patients who are alive 8 years following diagnosis. The SEER cohort analysis demonstrates significant improvements in pediatric medulloblastoma survival. In contrast to previous reports, the majority of patients survive in the modern era, and those alive 8 years following initial diagnosis are likely a long-term survivor. The importance of minimizing treatment-related toxicity is increasingly apparent given the likelihood of long-term survival.
authors.affiliation: Division of Pediatric Neurosurgery, Department of Surgery, Sainte Justine Hospital, University of Montreal, Montreal, QC, Canada.
authors.forename: Alexander G
authors.initials: AG
authors.lastname: Weil
authors.affiliation: Department of Neurosurgery, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, CA, USA.
authors.forename: Anthony C
authors.initials: AC
authors.lastname: Wang
authors.affiliation: Division of Pediatric Neurosurgery, Department of Surgery, Sainte Justine Hospital, University of Montreal, Montreal, QC, Canada.
authors.forename: Harrison J
authors.initials: HJ
authors.lastname: Westwick
authors.affiliation: Division of Neurosurgery, Department of Surgery, University of Toronto, Toronto, ON, Canada.
authors.forename: George M
authors.initials: GM
authors.lastname: Ibrahim
authors.affiliation: Department of Global Medicine, Keck School of Medicine at University of Southern California, Los Angeles, CA, USA.
authors.forename: Rojine T
authors.initials: RT
authors.lastname: Ariani
authors.affiliation: Division of Pediatric Neurosurgery, Department of Surgery, Sainte Justine Hospital, University of Montreal, Montreal, QC, Canada.
authors.forename: Louis
authors.initials: L
authors.lastname: Crevier
authors.affiliation: Division of Pediatric Hematology-Oncology, Department of Medicine, Sainte Justine Hospital, University of Montreal, Montreal, QC, Canada.
authors.forename: Sebastien
authors.initials: S
authors.lastname: Perreault
authors.affiliation: Division of Hematology Oncology, Department of Pediatrics, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, CA, USA.
authors.forename: Tom
authors.initials: T
authors.lastname: Davidson
authors.affiliation: Department of Medicine, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, CA, USA.
authors.forename: Chi-Hong
authors.initials: CH
authors.lastname: Tseng
authors.affiliation: Department of Neurosurgery and Pediatrics, David Geffen School of Medicine at University of California Los Angeles, Mattel Children's Hospital at UCLA300 Stein Plaza, Suite 525, Los Angeles, CA, 90095-6901, USA. afallah@mednet.ucla.edu.
authors.forename: Aria
authors.initials: A
authors.lastname: Fallah
mesh_terms: D002528:Cerebellar Neoplasms / Q000175:diagnosis* / Q000401:mortality*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008527:Medulloblastoma / Q000175:diagnosis* / Q000401:mortality*; D011379:Prognosis; D012189:Retrospective Studies; D017741:Survivors
keywords: Epidemiology; Medulloblastoma; Natural History; Population study; Prognosis; Survival; Time to event