Abstract
CNS tumors are least likely to present during adolescence, when they do, their clinical presentation is enmeshed with the young person’s physical, psychological, educational, and social development. The population incidence of the different tumor types highlights the transition from “childhood type” to “adult type” and identifies those driven by adolescent development itself – intracranial germ cell tumors. While all brain tumors in this age group are undoubtedly a product of brain development, genetic and environmental factors also play a key role and highlight the complexity of tumorigenesis in the brain. Symptomatology is determined by anatomical factors and influence pathways to diagnosis which can be prolonged and are currently the focus of population interventions, seeking to accelerate diagnosis. Awareness of genetic predisposing conditions can contribute to enhanced awareness of risk and complex genetic factors that in the future can be expected to be therapeutic targets. Once diagnosed, the AYA multidisciplinary and multiprofessional teams are in development across health systems, frequently organized differently in pediatric versus adult settings. The neurosurgical options are determined by anatomy and surgical feasibility seeking to maximize resection rates while minimizing neurosurgical toxicity. Acute neurological syndromes of raised intracranial pressure, epilepsy, and acute neurological problems associated with radiotherapy and chemotherapy require an integrated approach to care and rehabilitation. Adjuvant therapies with chemotherapy and radiotherapy are tumor-type-specific and driven by trial programs, which we anticipate to be focused increasingly upon molecularly targeted therapies as they come available, although targeting drug therapies to the brain remains an outstanding challenge to their effectiveness. Intracranial germ cell tumors peak during the AYA age groups and have reached a new level of consensus, regarding all aspects of their care as a result of global research collaboration. Late consequences of childhood brain tumors and AYA tumors are a major challenge for current health systems, as survival rates rise and the neurological, cognitive, endocrine, and psychological consequences of brain tumor color the lives of the adult survivors. Future improvements in outcome are dependent on coordinated research programs seeking to answer questions prioritized by successful public engagement.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Gogtay N, Giedd J, Lusk L et al (2004) Dynamic mapping of human cortical development during childhood through early adulthood. PNAS 101(21):8174–8179
Scotting P, Walker D, Perilongo G (2005) Childhood solid tumors: a developmental disorder. Nat Rev Cancer 5:481–488
Parkin DM, Kramárová E, Draper GJ, Masuyer E, Michaelis J, Neglia J, Qureshi S, Stiller CA, Kramárová E, Draper GJ (1998) International incidence of childhood cancer, vol 2, IARC Scientific Publications, No 144. International Agency for Research on Cancer, Lyon
World Health Organization (2007) WHO classification of tumors of the central nervous system, vol 1. 4th edn. IARC Publications. IARC WHO Classification of Tumours, Vol 1. Louis, DN, Ohgaki, H, Wiestler, OD, Cavenee, WK. ISBN-13 9789283224303, ISBN-10 9283224302
Surveillance, Epidemiology, and End Results (SEER) Program (www.seer.cancer.gov) SEER*Stat Database: incidence – SEER 9, 13 and 18 Regs Research Data + Hurricane Katrina Impacted Louisiana Cases, Nov 2014 Sub (2000–2012) <Katrina/Rita Population Adjustment> – Linked To County Attributes – Total U.S., 1969–2013 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, Surveillance Systems Branch, released April 2015, based on the November 2014 submission
Bleyer A, O’Leary M, Barr R, Ries L (2006) Cancer epidemiology in older adolescents and young adults 15–29 years of age, including SEER incidence and survival: 1975–2000. National Cancer Institute, Bethesda
Osteom QT, Gittleman H, de Blank PM et al (2016) Adolescent and young adult primary brain and central nervous system tumors diagnosed in the United States in 2008–2012, Central Brain Tumor Registry of the United States American Brain Tumor Association Adolescent and Young Adult Brain Tumor Report Statistical Supplement 2016. J Neuro-Oncol 18(1), Supplement 1–150. http://www.cbtrus.org/reports/reports.html. Accessed 28 Jan 2016
Northcott PA, Korshunov A, Witt H et al (2011) Medulloblastoma comprises four distinct molecular variants. J Clin Oncol 29(11):1408–1414
Gibson P, Tong Y, Robinson G et al (2010) Subtypes of medulloblastoma have distinct developmental origins. Nature 468(7327):1095–1099
Neglia J, Robison L, Stovall M et al (2006) New primary neoplasms of the central nervous system in survivors of childhood cancer: a report from the Childhood Cancer Survivor Study. J Natl Cancer Inst 98(21):1528–1537
Taylor A, Little M, Winter D et al (2010) Population-based risks of CNS tumors in survivors of childhood cancer: the British Childhood Cancer Survivor Study. J Clin Oncol 28(36):5287–5293
Pearce MS, Salotti JA, Little MP et al (2012) Radiation exposure from CT scans in childhood and subsequent risk of leukaemia and brain tumours: a retrospective cohort study. Lancet 380(9840):499–505
Mathews J, Forsythe A, Brady Z et al (2013) Cancer risk in 680,000 people exposed to computed tomography scans in childhood or adolescence: data linkage study of 11 million Australians. Br Med J 346:f2360
Krille L, Dreger S, Schindel R et al (2015) Risk of cancer incidence before the age of 15 years after exposure to ionising radiation from computed tomography: results from a German cohort study. Radiat Environ Biophys 54(1):1–12
Journy N, Rehel J-L, Ducou Le Pointe H et al (2015) Are the studies on cancer risk from CT scans biased by indication? Elements of answer from a large-scale cohort study in France. Br J Cancer 112:185–193
Boice J (2015) Radiation epidemiology and recent paediatric computed tomography studies. Ann ICRP 44(1 Suppl):236–248
Spycher B, Lupatsch J, Zwahlen M et al (2015) Background ionizing radiation and the risk of childhood cancer: a census-based nationwide cohort study. Environ Health Perspect 123:622–628
Del Risco Kollerud R, Blaasaas K, Claussen B (2014) Risk of leukemia or cancer in the central nervous system among children living in an area with high indoor radon concentrations: results from a cohort study in Norway. Br J Cancer 111:1413–1420
Hauri D, Spycher B, Huss A et al (2013) Domestic radon exposure and risk of childhood cancer: a prospective census-based cohort study. Environ Health Perspect 121:1239–1244
Kendall G, Little M, Wakeford R et al (2013) A record-based case-control study of natural background radiation and the incidence of childhood leukemia and other cancers in Great Britain during 1980–2006. Leukemia 27:3–9
Raaschou-Nielsen O, Andersen C, Andersen H et al (2008) Domestic radon and childhood cancer in Denmark. Epidemiology 19:536–543
Brauner E, Andersen Z, Andersen C et al (2013) Residential radon and brain tumor incidence in a Danish cohort. PLoS ONE 8(9):374435
Huncharek M, Kupelnick B (2004) A meta-analysis of maternal cured meat consumption during pregnancy and the risk of childhood brain tumors. Neuroepidemiology 23:78–84
Huncharek M, Kupelnick B, Wheeler L (2003) Dietary cured meat and the risk of adult glioma: a meta-analysis of nine observational studies. J Environ Pathol Toxicol Oncol 22:129–137
Terry M, Howe G, Pogoda J et al (2009) An international case-control study of adult diet and brain tumor risk: a histology-specific analysis by food group. Ann Epidemiol 19(3):161–171
Chen M, Chang C, Tao L, Lu C (2015) Residential exposure to pesticide during childhood and childhood cancers: a meta-analysis. Pediatrics 136:719–729
van Maele-Fabry G, Hoet P, Lison D (2013) Parental occupational exposure to pesticides as risk factor for brain tumors in children and young adults: a systematic review and meta-analysis. Environ Int 56:19–31
Huoi C, Olsson A, Lightfoot T et al (2014) Parental occupational exposure and risk of childhood central nervous system tumors: a pooled analysis of case-control studies from Germany, France and the UK. Cancer Causes Control 25(12):1603–1613
Franceschi S, Dal Maso L, La Vecchia C (1999) Advances in the epidemiology of HIV-associated non-Hodgkin’s lymphoma and other lymphoid neoplasms. Int J Cancer 83:481–485
International Collaboration on HIV & Cancer (2000) Highly active anti-retroviral therapy and incidence of cancer in human immunodeficiency virus-infected adults. J Natl Cancer Inst 92:1823–1830
O’Neill K, Murphy M, Bunch K et al (2015) Infant birthweight and risk of childhood cancer: international population-based case control studies of 40,000 cases. Int J Epidemiol 44(1):153–168
Crump C, Sundquist J, Sieh W, Winkleby M, Sundquist K (2015) Perinatal and familial risk factors for brain tumors in childhood through young adulthood. Cancer Res 75:576–583
Kitahara C, Wang S, Melin B et al (2012) Association between adult height, genetic susceptibility and risk of glioma. Int J Epidemiol 41:1075–1085
Sergentanis T, Tsivgoulis G, Perlepe C et al (2015) Obesity and risk for brain/CNS tumors, gliomas and meningiomas: a meta-analysis. PLoS ONE 10(9):e1036974
Niedermaier T, Behrens G, Schmid D, Schlecht I, Fischer B, Leitzmann M (2015) Body mass index, physical activity and risk of adult meningioma and glioma: a meta-analysis. Neurology 85:1342–1350
Johnson K, Cullen J, Barnholtz-Sloan J et al (2014) Childhood brain tumor epidemiology: a brain tumor epidemiology consortium review. Cancer Epidemiol Biomark Prev 23:2716–2736
Linos E, Raine T, Alonso A, Michaud D (2007) Atopy and risk of brain tumors: a meta-analysis. J Natl Cancer Inst 99:1544–1550
Chen C, Xu T, Chen J, Zhou J, Yan Y, Lu Y, Wu S (2011) Allergy and risk of glioma: a meta-analysis. Eur J Neurol 18(3):387–395
Krishnamachari B, Il-yasova D, Scheurer M et al (2015) A pooled multisite analysis of the effects of atopic medical conditions in glioma risk in different ethnic groups. Ann Epidemiol 25(4):270–274
McCarthy B, Rankin K, Aldape K et al (2011) Risk factors for oligodendroglial tumors: a pooled international study. Neuro-Oncology 13(2):242–250
Wang M, Chen C, Qu J et al (2011) Inverse association between eczema and meningioma: a meta-analysis. Cancer Causes Control 22:1355–1363
Claus E, Calvocoressi L, Bondy M, Schildkraut J, Wiemels J, Wrensch M (2011) Family and personal medical history and risk of meningioma. J Neurosurg 115:1072–1077
Turner M, Krewski D, Armstrong B et al (2013) Allergy and brain tumors in the INTERPHONE study: pooled results from Australia, Canada, France, Israel and New Zealand. Cancer Causes Control 24(5):949–960
Kitahara C, Linet M, Brenner A et al (2014) Personal history of diabetes, genetic susceptibility to diabetes, and risk of brain glioma: a pooled analysis of observational studies. Cancer Epidemiol Biomark Prev 23:47–54
Zhao L, Zheng Z, Huang P (2015) Diabetes mellitus and the risk of glioma: a meta-analysis. Oncotarget. doi:10.18632/oncotarget.6605, Epub ahead of print
Qi Z, Shao C, Zhang X, Hui G, Wang Z (2013) Exogenous and endogenous hormones in relation to glioma in women; a meta-analysis of 11 case-control studies. PLoS ONE 8(7):e68695
Krishnamachari B, Il-yasova D, Scheurer M, Bondy M, Wrensch M, Davis F (2014) A pooled multisite analysis of the effects of female reproductive hormones on glioma risk. Cancer Causes Control 25:1007–1013
Mezei G, Gadallah M, Kheifets L (2008) Residential magnetic field exposure and childhood brain cancer: a meta-analysis. Epidemiology 19:424–430
Kheifets L, Ahlbom A, Crespi C et al (2010) A pooled analysis of extremely low-frequency magnetic fields and childhood brain tumors. Am J Epidemiol 172(7):752–761
Aydin D, Feychting M, Schuz J et al (2011) Mobile phone use and brain tumors in children and adolescents: a multicenter case-control study. J Natl Cancer Inst 103:1264–1276
Kheifets L, Monroe J, Vergara X, Mezei G, Afifi A (2008) Occupational electromagnetic fields and leukemia and brain cancer: an update to two meta-analyses. J Occup Environ Med 50:677–688
Turner M, Benke G, Bowman J et al (2014) Occupational exposure to extremely low-frequency magnetic fields and brain tumor risks in the INTEROCC Study. Cancer Epidemiol Biomark Prev 23:1863–1872
Lagorio S, Roosli M (2014) Mobile phone use and risk of intracranial tumors: a consistency analysis. Bioelectromagnetics 35:79–90
Ostrom Q, Bauchet L, Davis F et al (2014) The epidemiology of glioma in adults: a ‘state of the science’ review. Neuro-Oncology 16(7):896
Huang Y, Huang J, Lan H, Zhao G, Huang C-Y (2014) A meta-analysis of parental smoking and the risk of childhood brain tumors. PLoS ONE 9(7):e102910
Mandelzweig L, Novikov I, Sadetzki S (2009) Smoking and risk of glioma: a meta-analysis. Cancer Causes Control 20:1927–1938
Fan Z, Ji T, Wan S et al (2013) Smoking and risk of meningioma: a meta-analysis. Cancer Epidemiol 37(1):39–45
Galeone C, Malerba S, Rota M et al (2013) A meta-analysis of alcohol consumption and the risk of brain tumors. Ann Oncol 24(2):514–523
Malerba S, Galeone C, Pelucchi C et al (2013) A meta-analysis of coffee and tea consumption and the risk of glioma in adults. Cancer Causes Control 24(2):267–276
Liu Y, Lu Y, Wang J et al (2014) Association between non steroidal anti-inflammatory drug use and brain tumor risk: a meta-analysis. Br J Clin Pharmacol 78(1):58–68
Engels E, Hormuzd A, Nielsen N et al (2003) Cancer incidence in Denmark following exposure to poliovirus vaccine contaminated with simian virus 40. J Natl Cancer Inst 95(7):532–539
Wiemels J, Wrensch M, Claus E (2010) Epidemiology and etiology of meningioma. J Neuro Oncol 99:307–314
Schlehofer B, Blettner M, Preston-Martin S et al (1999) Role of medical history in brain tumour development. Results from the international adult brain tumour study. Int J Cancer 82:155–160
Wrensch M, Lee M, Miike R, Newman B, Barger G, Davis R, Wiencke J, Neuhaus J (1997) Familial and personal medical history of cancer and nervous system conditions among adults with glioma and controls. Am J Epidemiol 145:581–593
Pogoda J, Preston-Martin S, Howe G et al (2009) An international case-control study of maternal diet during pregnancy and childhood brain tumor risk: a histology-specific analysis by food group. Ann Epidemiol 19(3):148–160
Gurney J, Mueller B, Preston-Martin S et al (1997) a study of pediatric brain tumors and their association with epilepsy and anti-convulsant use. Neuroepidemiology 16:248–255
Inskip P, Mellemkjaer L, Gridley G, Olsen J (1998) Incidence of intracranial tumours following hospitalization for head injuries (Denmark). Cancer Causes Control 9:109–116
Preston-Martin S, Pogoda J, Schlehofer B et al (1998) An international case-control study of adult glioma and meningioma: the role of head trauma. Int J Epidemiol 27(4):579–586
Wrensch M, Miike R, Lee M, Neuhaus J (2000) Are prior head injuries or diagnostic x-rays associated with glioma in adults? The effects of control selection bias. Neuroepidemiology 19:234–244
de Kock L, Sabbaghian N, Druker H et al (2014) Germ-line and somatic DICER1 mutations in pineoblastoma. Acta Neuropathol 128(4):583–595
de Kock L, Sabbaghian N, Plourde F et al (2014) Pituitary blastoma: a pathognomonic feature of germ-line DICER1 mutations. Acta Neuropathol 128(1):111–122
Dubuc A, Northcott P, Mack S, Whitt H, Pfister S, Taylor M (2010) The genetics of pediatric brain tumors. Curr Neurol Neurosci Rep 10(3):215–223
Foulkes W, Bahubeshi A, Hamel N et al (2011) Extending the phenotypes associated with DICER1 mutations. Hum Mutat 32(12):1381–1384
Hassleblatt M, Nagel I, Oyen F et al (2014) SMARCA4-mutated atypical teratoid/rhabdoid tumors are associated with inherited germline alterations and poor prognosis. Acta Neuropathol 128(3):453–456
Hottinger A, Khakoo Y (2007) Update on the management of familial central nervous system tumor syndromes. Curr Neurol Neurosci Rep 7:200–207
Kimmelman A, Liang B (2001) Familial neurogenic tumor syndromes. Hematol Oncol Clin N Am 15(6):1073–1084
Kirschner L, Carney J, Pack S et al (2000) Mutations of the gene encoding the protein kinase A type 1-alpha regulatory subunit in patients with the carney complex. Nat Genet 26(1):89–92
Louis DN, Ohgaki H, Wiestler O et al (2007) The 2007 WHO classification of tumors of the central nervous system. Acta Neuropathol 114(2):97–109
Ohgaki H, Kim Y, Steinbach J (2010) Nervous system tumors associated with familial tumor syndromes. Curr Opin Neurol 23(6):583–591
Tabori U, Laberge A-M, Ellezam B, Carret A-S (2015) Cancer predisposition in children with brain tumors. In: Scheinemann K, Bouffet E (eds) Pediatric neuro-oncology. Springer Science + Business Media, New York, pp 69–89
Taylor M, Mainprize T, Rutka J, Becker L, Bayani J, Drake J (2001) Medulloblastoma in a child with Rubenstein-Taybi syndrome: case report and review of the literature. Pediatr Neurosurg 35(5):235–238
Villani A, Malkin D, Tabori U (2012) Syndromes predisposing to pediatric central nervous system tumors: lessons learned and new promises. Curr Neurol Neurosci Rep 12(2):153–164
Ramaswamy V, Northcott PA, Taylor MD (2011) FISH & Chips: the recipe for imporoved prognostication & outcomes for children with medulloblastoma Cancer Genetics 204:577–588
Fahmideh M, Lavebratt C, Schuz J et al (2015) CCDC26, CDKN2BAS, RTEL1 and TERT polymorphisms in pediatric brain tumor susceptibility. Carcinogenesis 36(8):876–882
Walsh K, Wiencke J, Lachance D et al (2015) Telomere maintenance and the etiology of adult glioma. Neuro-Oncology 17(11):1445–1452
Friedman J, Birch P (1997) An association between optic glioma and other tumors of the central nervous system in neurofibromatosis type 1. Neuropediatrics 28:131–132
Gutmann D, Rasmussen S, Wolkenstein P et al (2002) Gliomas presenting after age 10 in individuals with neurofibromatosis type 1 (NF1). Neurology 59:759–761
Listernick R, Charrow J, Greenwald M, Esterly N (1989) Optic gliomas in children with neurofibromatosis type 1. J Pediatr 114(5):788–792
Molloy P, Bilaniuk L, Vaughan S et al (1995) Brainstem tumors in patients with neurofibromatosis type 1: eA distinct clinical entity. Neurology 45(10):1897–1902
Riffaud L, Vinchon M, Ragragui O, Delestret I, Ruchoux M, Dhellemmes P (2002) Hemispheric cerebral gliomas in children with NF1: arguments for a long term follow up. Childs Nerv Syst 18(1–2):43–47
Listernick R, Ferner R, Liu G, Gutmann D (2007) Optic pathway gliomas in neurofibromatosis-1: controversies and recommendations. Ann Neurol 61(3):189–198
Evans DGR, Sainio M, Baser ME (2000) Neurofibromatosis type 2. J Med Genet 37:897–904
Evans D (2009) Neurofibromatosis type 2 (NF2): a clinical and molecular review. Orphanet J Rare Dis 4:16
Kotecha R, Pascoe E, Rushing E et al (2011) Meningiomas in children and adolescents: a meta-analysis of individual patient data. Lancet Oncol 12(13):1229–1239
Plotkin S, Stemmer-Rachamimov A, Barker F II et al (2009) Hearing improvement after Bevacizumab in patients with neurofibromatosis type 2. N Engl J Med 361:358–367
Maddock IR, Moran A, Maher ER, Teare MD et al (1996) A genetic register for von Hippel-Lindau disease. J Med Genet 33(2):120–127
Corradetti M, Inoki K, Bardeesy N, DePinho R, Guan K-L (2004) Regulation of the TSC pathway by LKB1: evidence of a molecular link between tuberous sclerosis complex and Peutz-Jeghers syndrome. Genes Dev 18:1533–1538
Inoki K, Zhu T, Guan K-L (2003) TSC2 mediates cellular energy response to control cell growth and survival. Cell 115:577–590
Roach E, Smith M, Huttenlocher P, Bhat M, Alcorn D, Hawley L (1992) Diagnostic criteria: tuberous sclerosis complex. Report of the Diagnostic Criteria Committee of the National Tuberous Sclerosis Association. J Child Neurol 7:221–224
Webb D, Fryer A, Osborne J (1996) Morbidity associated with tuberous sclerosis: a population study. Dev Med Child Neurol 38:146–155
Leiden open variation database: tuberous sclerosis database. http://chromium.liacs.nl/LOVD2/TSC. Accessed 28 Jan 2016
Hoogeveen-Westerveld M, Ekong R, Povey S et al (2013) Functional assessment of TSC2 variants identified in individuals with tuberous sclerosis complex. Hum Mutat 34(1):167–175
Hoogeveen-Westerveld M, Ekong R, Povey S et al (2012) Functional assessment of TSC1 missense variants identified in individuals with tuberous sclerosis complex. Hum Mutat 33(3):476–479
Northrup H, Kruger DA, International Tuberous Sclerosis Complex Consensus Group (2013) Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatr Neurol 49:243–254
Kleihues P, Schauble B, Hausen A, Esteve J, Ohgaki H (1997) Tumors associated with p53 germline mutations. A synopsis of 91 families. Am J Pathol 150:1–13
Birch J, Hartley A, Blair V et al (1990) Cancer in the families of children with soft tissue sarcoma. Cancer 66(10):2239–2248
Garber J, Goldstein A, Kantor A, Dreyfus M, Fraumeni JJ, Li F (1991) Follow up study of 24 families with Li-Fraumeni syndrome. Cancer Res 51(22):6094–6097
Li F, Fraumeni JJ, Mulvihill J et al (1988) A cancer family syndrome in 24 kindreds. Cancer Res 48(18):5358–5362
Tabori U, Shlien A, Baskin B et al (2010) TP53 alterations determine clinical sub-groups and survival of patients with choroid plexus tumors. J Clin Oncol 28(12):1995–2001
Varley J (2003) Germline TP53 mutations and Li-Fraumeni syndrome. Hum Mutat 21:313–320
Birch J, Alston R, McNally R et al (2001) Relative frequency and morphology of cancers in carriers of germline TP53 mutations. Oncogene 20(34):4621–4628
Trump D, Farren B, Wooding C et al (1996) Clinical studies of multiple endocrine neoplasia type 1 (MEN1). Q J Med 89:653–669
Robinson S, Cohen A (2000) Cowden disease and L’Hermitte-Duclos disease: characterization of a new phakomatosis. Neurosurgery 46(2):371
Kleihues P, Cavenee W (2000) Pathology & genetics. Tumors of the nervous system. IARC Press, Lyon
Paraf F, Jothy S, Van Meir E (1997) Brain tumor polyposis syndrome: two genetic diseases? J Clin Oncol 15:2744–2758
Vasen H, Sanders E, Taal B et al (1996) The risk of brain tumors in hereditary non-polyposis colorectal cancer (HNPCC). Int J Cancer 65:422–425
Aarnio M, Sankila R, Pukkala E et al (1999) Cancer risk in mutation carriers of DNA-mismatch-repair genes. Int J Cancer 81(2):214–218
Durno C, Sherman P, Aronson M et al (2015) Phenotypic and genotypic characterisation of biallelic mismatch repair deficiency (BMMR-D) syndrome. Eur J Cancer 51(8):977–983
Le D, Uram J, Wang H et al (2015) PD-1 blockade in tumors with mismatch-repair deficiency. N Engl J Med 372:2509–2520
Hamilton S, Liu B, Parsons R et al (1995) The molecular basis of Turcot’s syndrome. N Engl J Med 332(13):839–847
Hasle H (2001) Pattern of malignant disorders in individuals with Down’s syndrome. Lancet Oncol 2(7):429–436
Amlashi S, Riffaud L, Brassier G, Morandi X (2003) Nevoid basal cell carcinoma syndrome; relation with desmoplastic medulloblastoma in infancy. A population-based study and review of the literature. Cancer 98(31):618–624
Biegel J, Zhou J-Y, Rorke L, Stenstrom C, Wainwright L, Fogelgren B (1999) Germ-line and acquired mutations of IN11 in atypical teratoid and rhabdoid tumors. Cancer Res 59:74–79
Cowan R, Hoban P, Kelsey A, Birch J, Gattamaneni R, Evans D (1997) The gene for the naevoid basal cell carcinoma syndrome acts as a tumor-suppressor gene in medulloblastoma. Br J Cancer 76(2):141–145
Stiller C, Bleyer W (2004) Epidemiology. In: Walker D, Perilongo G, Punt J, Taylor R (eds) Brain & spinal tumors of childhood. Arnold, London, pp 35–49
Becker Y (1986) Cancer in ataxia-telangiectasia patients: analysis of factors leading to radiation – induced and spontaneous tumors. Anticancer Res 6:1021–1032
Chun H, Gatti R (2004) Ataxia-telangiectasia, an evolving phenotype. DNA Repair 3:1187–1196
Khanna K (2000) Cancer risk and the ATM gene: a continuing debate. J Natl Cancer Inst 92:795–802
Kuhne M, Riballo E, Rief N, Toghkamm K, Jeggo P (2004) A double-strand break repair defect in ATM-deficient cells contributes to radiosensitivity. Cancer Res 64:500–508
Hemminki K, Li X (2004) Association of brain tumors with other neoplasms in families. Eur J Cancer 40:253–259
Malmer B, Henriksson R, Gronberg H (2002) Different aetiology of familial low-grade and high-grade glioma? A nationwide cohort study of familial glioma. Neuroepidemiology 21:279–286
Malmer B, Henriksson R, Gronberg H (2003) Familial brain tumors – genetics of environment? A nationwide cohort study of cancer risk in spouses and first-degree relatives of brain tumor patients. Int J Cancer 106:260–263
Hemminki K, Li X, Collins V (2001) A population-based study of familial central nervous system hemangioblastomas. Neuroepidemiology 20:257–261
Paunu N, Syrjakoski K, Sankila R et al (2001) Analysis of p53 tumor suppressor gene in families with multiple glioma patients. J Neuro-Oncol 55(3):159–165
Chu T, Shah A, Walker D, Coleman M (2015) Pattern of symptoms and signs of primary intracranial tumors in children and young adults: a record linkage study. Arch Dis Child 12:1115–1122
Wilne S, Collier J, Kennedy C, Koller K, Grundy R, Walker D (2007) Presentation of childhood CNS tumors: a systematic review and meta-analysis. Lancet Oncol 8:685–695
HeadSmart: be brain tumor aware (2015) A new clinical guideline from the Royal College of Paediatrics & Child Health with a national awareness campaign accelerates brain tumor diagnosis in UK children – ‘HeadSmart: Be Brain Tumor Aware’. Neuro Oncol. Vol 18(3):445–454
Pathway to diagnosis: the diagnosis of brain tumors. RCPCH (2008). London
Improving outcomes in children and young people with cancer. National Institute for Health & Care Excellence (NICE) (2005). London
Improving outcomes for people with brain and other CNS tumors. London
Nicholson JC, Punt J, Hale J, Saran F, Calaminus G, Germ Cell Tumour Working Groups of the United Kingdom Children’s Cancer Study Group (UKCCSG), International Society of Paediatric Oncology (SIOP) (2002) Neurosurgical management of paediatric germ cell tumours of the central nervous system – a multi-disciplinary team approach for the new millennium. Br J Neurosurg 16:93–95
Walker D, Punt J, Sokal M (1999) Clinical management of brain stem glioma (BSG). Arch Dis Child 40:558–564
Walker D, Punt J, Sokal M (2004) Brainstem tumors. In: Walker D, Perilongo G, Punt J, Taylor R (eds) Brain & spinal tumors of childhood. Arnold, London
Walker D, Liu J-F, Kieran M et al (2013) A multi-disciplinary consensus statement concerning surgical approaches to low-grade, high-grade astrocytomas and diffuse intrinsic pontine gliomas in childhood (CPN Paris 2011) using the Delphi method. Neuro-Oncology 15(4):462–468
Westphal M, Hilt D, Bortey E et al (2003) A phase 3 trial of local chemotherapy with biodegradable carmustine (BCNU) wafers (Gliadel wafers) in patients with primary malignant glioma. Neuro-Oncology 5(2):79–88
Barnett F, Scharer-Schuksz M, Wood M, Yu X, Wagner T, Friedlander M (2004) Intra-arterial delivery of endostatin gene to brain tumors prolongs survival and alters tumor vessel ultrastructure. Gene Ther 11(16):1283–1289
Chiocca E, Abbed K, Tatter S et al (2004) A Phase 1 open-label, dose-escalation, multi-institutional trial of injection with an E1B-attenuated adenovirus, ONYX-015, into the peritumoral region of recurrent malignant gliomas in the adjuvant setting. Mol Ther 10(5):958–966
Glorioso J, Fink D (2004) Herpes vector-mediated gene transfer in treatment of diseases of the nervous system. Annu Rev Microbiol 58:253–271
Immonen A, Vapalahti M, Tyynela K et al (2004) Adv-HSV-tk gene therapy with intravenous ganciclovir improves survival in human malignant glioma: a randomised controlled study. Mol Ther 10(5):967–972
McKeown S, Ward C, Robson T (2004) Gene-directed enzyme prodrug therapy: a current assessment. Curr Opin Mol Ther 6(4):421–435
Okada H, Pollack IF (2004) Cytokine gene therapy for malignant glioma. Expert Opin Biol Ther 4(10):1609–1620
Merchant T, Kun L, Wu S, Xiong X, Sanford R, Boop F (2009) Phase II trial of conformal radiation therapy for pediatric low-grade glioma. J Clin Oncol 27(22):3598–3604
Packer R, Goldwin J, Nicholson HS et al (1999) Treatment of children with medulloblastomas with reduced-dose craniospinal radiation therapy and adjuvant chemotherapy: a Children’s Cancer Group Study. J Clin Oncol 17(7):2127–2136
Bleyer A, Choi M, Wang S, Fuller C, Raney R (2009) Increased vulnerability of the spinal cord to radiation or intrathecal chemotherapy during adolescence: a report from the Children’s Oncology Group. Pediatr Blood Cancer 53(7):1205–1210
Moskowitz C, Chou J, Wolden S et al (2014) Breast cancer after chest radiation therapy for childhood cancer. J Clin Oncol 32(21):2217–2223
Kumar R, Zhai H, Both S, Tochner Z, Lustig R, Hill-Kayser C (2013) Breast cancer screening for childhood cancer survivors after craniospinal irradiation with protons versus x-rays: a dosimetric analysis and review of the literature. J Pediatr Hematol Oncol 35(6):462–467
Balis F, Poplack D (1993) Cancer chemotherapy. In: Nathan D, Oski F (eds) Hematology of infancy and childhood. WB Saunders, Philadelphia, pp 1207–1238
Vivekanandan S, Breene R, Ramajujachar R et al (2015) The UK experience of a treatment strategy for pediatric metastatic medulloblastoma comprising intensive induction chemotherapy, hyperfractionated accelerated radiotherapy and response directed high dose myeloablative chemotherapy or maintenance chemotherapy (Milan strategy). Pediatr Blood Cancer 62(12):2132–2139
Glaser A, Buxton N, Hewitt M, Punt J, Walker D (1996) The role of steroids in paediatric central nervous system malignancies. Br J Neurosurg 10:123–124
Tabori U, Sung L, Hudin J et al (2005) Medulloblastoma in the second decade of life: a specific group with respect to toxicity and management. Cancer 103(9):1874–1880
Murray M, Bartels U, Nishikawa R, Fangusaro J, Matsutani M, Nicholson J (2015) Consensus on the management of intracranial germ-cell tumors. Lancet Oncol 16(9):e470–e477
Wara W, Jenkin R, Evans A et al (1979) Tumors of the pineal and suprasellar region: Children’s Cancer Study Group treatment results 1960–1975. Cancer 43:698–701
Jennings M, Gelman R, Hochberg F (1985) Intracranial germ-cell tumors: natural history and pathogenesis. J Neurosurg 63:155–167
Calaminus G, Kortmann R, Worch J et al (2013) SIOP CNS GCT 95: final report of outcome of a prospective, multinational non-randomized trial for children and adults with intracranial germinoma, comparing craniospinal irradiation alone with chemotherapy followed by focal primary site irradiation for patients with localized disease. Neuro-Oncology 15(6):788–796
Yang Q-Y, Chen Z-P (2012) The treatment for histologically unconfirmed intracranial germ cell tumors: experience of 38 cases. Neuro-Oncology 14(Suppl 1):i49–i55
Bates A, Bullivant B, Sheppard M, Stewart P (1999) Life expectancy following surgery for pituitary tumors. Clin Endocrinol (Oxf) 50:315–319
Bates A, Van’t Hoff W, Jones P, Clayton R (1996) The effect of hypopituitarism on life expectancy. Clin Endocrinol Metab 81:1169–1172
Bulow B, Hagmar L, Mikoczy Z, Nordstrom C, Erfurth E (1997) Increased cerebrovascular mortality in patients with hypopituitarism. Clin Endocrinol (Oxf) 46:75–81
Nilsson B, Gustavasson-Kadaka E, Bengtsson B, Jonsson B (2000) Pituitary adenomas in Sweden between 1958 and 1991: incidence, survival, and mortality. J Clin Endocrinol Metab 85:1420–1425
Rosen T, Bengtsson B (1990) Premature mortality due to cardiovascular disease in hypopituitarism. Lancet 336:285–288
Tomlinson J, Holden N, Hills R et al (2001) Association between premature mortality and hypopituitarism. West Midlands Prospective Hypopituitary Study Group. Lancet 357:425–431
Motoyama T, Watanabe H, Yamamoto T, Sekiguchi M (1987) Production of alpha-fetoprotein by human germ cell tumors in vivo and in vitro. Acta Pathol Jpn 37:1263–1277
Motoyama T, Watanabe H, Yamamoto T, Sekiguchi M (1988) Production of beta-human chorionic gonadotropin by germ cell tumors in vivo and in vitro. Acta Pathologica Japan 38:577–590
Weissman D (1988) Glucocorticoid treatment for brain metastases and epidural spinal cord compression: a review. J Clin Oncol 6:543–551
Itoyama Y, Kochi M, Yamamoto H, Kuratsu J, Uemura S, Ushio Y (1990) Clinical study of intracranial non-germinomatous germ cell tumors producing alpha-fetoprotein. Neurosurgery 27:454–460
Packer R, Sutton L, Rosenstock J et al (1984) Pineal region tumors of childhood. Pediatrics 74(1):97–102
Murray M, Nicholson J, Coleman N (2015) Biology of childhood germ cell tumors, focusing on the significance of microRNAs. Andrology 3:129–139
Murray M, Horan G, Lowis S, Nicholson J (2013) Highlights from the Third International Central Nervous System Germ Cell Tumor symposium: laying the foundations for future consensus. eCancer 7:333
Fukushima S, Otsuka A, Suzuki T et al (2014) Mutually exclusive mutations of KIT and RAS are associated with KIT mRNA expression and chromosomal instability in primary intracranial pure germinomas. Acta Neuropathol 127(6):911–925
Wang L, Yamaguchi S, Burstein M et al (2014) Novel somatic and germline mutations in intracranial germ cell tumors. Nature 511:241–245
Palmer R, Murray M, Saini H et al (2010) Malignant germ cell tumors display common microRNA profiles resulting in global changes in expression of messenger RNA targets. Cancer Res 70:2911–2923
Murray M, Coleman N (2012) Testicular cancer: a new generation of biomarkers for malignant germ cell tumors. Nat Rev Urol 9(6):298–300
Murray M, Halsall D, Hook C, Williams D, Nicholson J, Coleman N (2011) Identification of microRNAs from the miR-371-373 and miR-302 clusters as potential serum biomarkers of malignant germ cell tumors. Am J Clin Pathol 135(1):119–125
Terashima K, Shen J, Luan J et al (2013) MicroRNA 371–373 and 302a in cerebrospinal fluid are potential tumor-derived biomarkers for intracranial germ cell tumors. Br J Neurosurg 27(4):e1–e25
Eiser C (1997) Children’s quality of life measures. Arch Dis Child 77:350–354
Herrman H, Westphal M, Winkler K, Laas R, Schulte F (1994) Treatment of non-germinomatous germ-cell tumors of the pineal region. Neurosurgery 34:524–529
Glaser A, Furlong W, Walker D et al (1999) Applicability of the health utilities index to a population of childhood survivors of central nervous system tumors in the United Kingdom. Eur J Cancer 35(2):256–261
Kiltie A, Gattamaneni R (1995) Survival and quality of life of paediatric intracranial germ cell tumor patients treated at the Christie Hospital, 1972–1993. Med Paediatr Oncol 25:450–456
Merchant T, Davis B, Sheldon J, Leibel S (1998) Radiation therapy for relapsed CNS germinoma after primary chemotherapy. J Clin Oncol 16(1):204–209
Ogawa K, Toita T, Nakamura K et al (2003) Treatment and prognosis of patients with intracranial non-germinomatous malignant germ cell tumors. Cancer 98:369–376
Shibamoto Y, Mitsuyuki A, Yamashita J et al (1988) Treatment results of intracranial germinoma as a function of the irradiated volume. Int J Radiat Oncol Biol Phys 15:285–290
Aoyama H, Shirato H, Kakuto Y et al (1998) Pathologically-proven intracranial germinoma treated with radiation therapy. Radiother Oncol 47:201–205
Kennedy C, Bull K (2004) Effect of neo-adjuvant chemotherapy on long-term health state and behaviour in the PNET3 RCT of treatment for primitive neuro-ectodermal tumor (PNET). ISPNO. Neuro Oncology, Boston
Benesch M, Lackner H, Schagerl S, Gallistl S, Frey E-M, Urban C (2001) Tumor- and treatment related side effects after multimodal therapy of childhood intracranial germ cell tumors. Acta Paediatr 90:264–270
Jenkin D, Shabanah M, Shail E et al (2000) Prognostic factors for medulloblastoma. Int J Radiat Oncol Biol Phys 47(3):573–584
Sankaranarayanan R, Black R, Swaminathan R, Parkin D (1998) An overview of cancer survival in developing countries. IARC Sci Publ 145:135–173
Surveillance, Epidemiology, and End Results (SEER) Program (www.seer.cancer.gov) SEER*Stat Database: incidence – SEER 18 Regs Research Data + Hurricane Katrina Impacted Louisiana Cases, Nov 2014 Sub (1973–2012 varying) – Linked To County Attributes – Total U.S., 1969–2013 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, Surveillance Systems Branch, released April 2015, based on the November 2014 submission
MacDonald L, On behalf of the Neuro-Oncology Group (2015) Top 10 priorities for clinical research in primary brain and spinal cord tumors. Final report of the James Lind Alliance Priority Setting Partnership in Neuro-Oncology
Acknowledgments
The authors would like to acknowledge the very capable assistance of Sue Franklin in the preparation of this manuscript.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2017 Springer International Publishing
About this chapter
Cite this chapter
Walker, D. et al. (2017). Central Nervous System Tumors. In: Bleyer, A., Barr, R., Ries, L., Whelan, J., Ferrari, A. (eds) Cancer in Adolescents and Young Adults. Pediatric Oncology. Springer, Cham. https://doi.org/10.1007/978-3-319-33679-4_14
Download citation
DOI: https://doi.org/10.1007/978-3-319-33679-4_14
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-33677-0
Online ISBN: 978-3-319-33679-4
eBook Packages: MedicineMedicine (R0)