title: Outcomes of children with central nervous system germinoma treated with multi-agent chemotherapy followed by reduced radiation.
abstract: CNS germinomas have an excellent prognosis with radiation therapy alone. However, in children, volume and dose of CNS radiation are associated with neurocognitive and neuroendocrine sequelae. Our objective was to determine long-term outcomes of our cohort who received chemotherapy and reduced radiation. This retrospective cohort study analyzed treatment and outcome of intracranial germinoma patients consecutively treated at Sick Kids, Toronto, Canada, from January 2000 to December 2013. 24 children (13 male, 11 female; median age 13.36 years) were identified. Median follow up was 61 months (range 1-144 months). Tumor location was suprasellar (n = 9), bifocal (8), pineal (6), and basal ganglia (1). Three children showed dissemination on imaging. 2/24 had only elevated serum human chorionic gonadotropin, 3/24 only elevated lumbar cerebrospinal fluid (CSF) hCG, and 2/24 had both elevated serum and lumbar CSF hCG. 23/24 children completed treatment and received multi-agent chemotherapy followed by either ventricular radiation (2340-2400 cGy) (n = 9), ventricular radiation + boost (1600 cGy) (n = 8), whole brain (2340 cGy) (n = 3), focal (4000 cGy) (n = 2) or craniospinal radiation (2340 cGy) (n = 1). Five-year progression free and overall survival was 96 and 100 % respectively. 8/24 patients with ventricular radiation ± boost (2340/4000 cGy) displayed stable full scale intelligence quotient over a mean interval of 3 years following radiation, but showed declined processing speed. In this limited experience, excellent 5-year overall survival rates were achieved with chemotherapy followed by reduced whole ventricular radiation even if ventricular radiation was delivered without boost.
authors.affiliation: Department of Paediatrics, University of British Columbia, Vancouver, BC, Canada. sylvia.cheng@cw.bcc.ca.|Department of Pediatrics, Division of Haematology/Oncology/BMT, B.C. Children's Hospital, 4480 Oak Street, B315, Vancouver, BC, V6H 3V4, Canada. sylvia.cheng@cw.bcc.ca.
authors.forename: Sylvia
authors.initials: S
authors.lastname: Cheng
authors.affiliation: Children's Brain Tumour Research Network, Royal Manchester Children's Hospital, Oxford Road, Manchester, M13 9WL, UK.|Institute of Cancer Sciences, The University of Manchester, Manchester Cancer Research Centre (MCRC), Wilmslow Road, Manchester, M20 4QL, UK.
authors.forename: John-Paul
authors.initials: JP
authors.lastname: Kilday
authors.affiliation: Department of Radiation Oncology, Princess Margaret Hospital, 610 University Avenue, Toronto, ON, M5G 2M9, Canada.
authors.forename: Normand
authors.initials: N
authors.lastname: Laperriere
authors.affiliation: Department of Psychology, The Hospital For Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
authors.forename: Laura
authors.initials: L
authors.lastname: Janzen
authors.affiliation: Division of Neurosurgery, The Hospital For Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
authors.forename: James
authors.initials: J
authors.lastname: Drake
authors.affiliation: Department of Paediatrics, University of Toronto, Toronto, ON, Canada.|Division of Haematology/Oncology, The Hospital For Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
authors.forename: Eric
authors.initials: E
authors.lastname: Bouffet
authors.affiliation: Department of Paediatrics, University of Toronto, Toronto, ON, Canada.|Division of Haematology/Oncology, The Hospital For Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
authors.forename: Ute
authors.initials: U
authors.lastname: Bartels
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016543:Central Nervous System Neoplasms / Q000473:pathology / Q000628:therapy*; D059248:Chemoradiotherapy; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D018237:Germinoma / Q000473:pathology / Q000628:therapy*; D006801:Humans; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate
keywords: CNS germinoma; Germinoma; Intracranial germ cell tumour; Neurocognitive outcome