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Pediatric Radiation Oncology pp 257–275Cite as

Childhood Ependymoma

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Part of the book series: Pediatric Oncology ((PEDIATRICO))

Abstract

Childhood ependymoma (EP) is a complex and malignant tumor that often arises in difficult locations in young children who suffer from the effects of tumor and aggressive surgery prior to consideration of adjuvant therapy, which is high-dose irradiation. Radiation therapy for EP has evolved considerably during the past 25 years both in methods and indications. Highly-focused irradiation administered post-operatively with limited margins surrounding the residual tumor and/or tumor bed in children of all ages is the current standard. The past history of radiation therapy for EP includes larger treatment volumes, even craniospinal irradiation, restricted to older children and delay or omission of radiation therapy in young patients. Indeed, during an era when the use of radiation therapy has been removed as a standard primary or adjuvant therapy for many childhood tumors, the use of radiation therapy has increased in the treatment of childhood EP and the excellent results and improved functional outcomes have served as an example of the benefit of newer treatment methods and template for the use of radiation therapy in the treatment of other tumors that involved young children.

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References

  • Children’s Oncology Group (2011) ACNS0831 Protocol 2011. https://members.childrensoncologygroup.org/Prot/ACNS0831/ACNS0831DOC.pdf. Accessed 22 April 2016

  • Agbahiwe HC, Wharam M, Batra S, Cohen K, Terezakis SA (2013) Management of pediatric myxopapillary ependymoma: the role of adjuvant radiation. Int J Radiat Oncol Biol Phys 85:421–427

    Article  PubMed  Google Scholar 

  • Akyurek S, Chang EL, Yu TK, Little D, Allen PK, Mccutcheon I, Mahajan A, Maor MH, Woo SY (2006) Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at M.D. Anderson Cancer Center. J Neuro-Oncol 80:177–183

    Article  Google Scholar 

  • Al-Halabi H, Montes JL, Atkinson J, Farmer JP, Freeman CR (2010) Adjuvant radiotherapy in the treatment of pediatric myxopapillary ependymomas. Pediatr Blood Cancer 55:639–643

    Article  PubMed  Google Scholar 

  • Archer TC, Pomeroy SL (2011) Posterior fossa ependymomas: a tale of two subtypes. Cancer Cell 20:133–134

    Article  CAS  PubMed  Google Scholar 

  • Ares C, Albertini F, Frei-Welte M, Bolsi A, Grotzer MA, Goitein G, Weber DC (2016) Pencil beam scanning proton therapy for pediatric intracranial ependymoma. J Neuro-Oncol 128:137–145

    Article  CAS  Google Scholar 

  • Bagley CA, Kothbauer KF, Wilson S, Bookland MJ, Epstein FJ, Jallo GI (2007) Resection of myxopapillary ependymomas in children. J Neurosurg 106:261–267

    PubMed  Google Scholar 

  • Bouffet E, Capra M, Bartels U (2009) Salvage chemotherapy for metastatic and recurrent ependymoma of childhood. Childs Nerv Syst 25:1293–1301

    Article  PubMed  Google Scholar 

  • Bouffet E, Foreman N (1999) Chemotherapy for intracranial ependymomas. Childs Nerv Syst 15:563–570

    Article  CAS  PubMed  Google Scholar 

  • Bouffet E, Hawkins CE, Ballourah W, Taylor MD, Bartels UK, Schoenhoff N, Tsangaris E, Huang A, Kulkarni A, Mabbot DJ, Laperriere N, Tabori U (2012) Survival benefit for pediatric patients with recurrent ependymoma treated with reirradiation. Int J Radiat Oncol Biol Phys 83:1541–1548

    Article  PubMed  Google Scholar 

  • Bouffet E, Perilongo G, Canete A, Massimino M (1998) Intracranial ependymomas in children: a critical review of prognostic factors and a plea for cooperation. Med Pediatr Oncol 30:319–329; discussion 329–31

    Article  CAS  PubMed  Google Scholar 

  • Carter M, Nicholson J, Ross F, Crolla J, Allibone R, Balaji V, Perry R, Walker D, Gilbertson R, Ellison DW (2002) Genetic abnormalities detected in ependymomas by comparative genomic hybridisation. Br J Cancer 86:929–939

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Chan MD, McMullen KP (2012) Multidisciplinary management of intracranial ependymoma. Curr Probl Cancer 36:6–19

    Google Scholar 

  • Chao ST, Kobayashi T, Benzel E, Reddy CA, Stevens GH, Prayson RA, Kalfas I, Schlenk R, Krishnaney A, Steinmetz MP, Bingaman W, Hahn J, Suh JH (2011) The role of adjuvant radiation therapy in the treatment of spinal myxopapillary ependymomas. J Neurosurg Spine 14:59–64

    Article  PubMed  Google Scholar 

  • Chiu JK, Woo SY, Ater J, Connelly J, Bruner JM, Maor MH, van Eys J, Oswald MJ, Shallenberger R (1992) Intracranial ependymoma in children: analysis of prognostic factors. J Neuro-Oncol 13:283–290

    Article  CAS  Google Scholar 

  • Combs SE, Thilmann C, Debus J, Schulz-Ertner D (2006) Local radiotherapeutic management of ependymomas with fractionated stereotactic radiotherapy (FSRT). BMC Cancer 6:222

    Article  PubMed  PubMed Central  Google Scholar 

  • Conklin HM, Li C, Xiong X, Ogg RJ, Merchant TE (2008) Predicting change in academic abilities after conformal radiation therapy for localized ependymoma. J Clin Oncol 26:3965–3970

    Article  PubMed  PubMed Central  Google Scholar 

  • Conter C, Carrie C, Bernier V, Geoffray A, Pagnier A, Gentet JC, Lellouch-Tubiana A, Chabaud S, Frappaz D (2009) Intracranial ependymomas in children: society of pediatric oncology experience with postoperative hyperfractionated local radiotherapy. Int J Radiat Oncol Biol Phys 74:1536–1542

    Article  PubMed  Google Scholar 

  • Di Pinto M, Conklin HM, Li C, Xiong X, Merchant TE (2010) Investigating verbal and visual auditory learning after conformal radiation therapy for childhood ependymoma. Int J Radiat Oncol Biol Phys 77:1002–1008

    Article  PubMed  Google Scholar 

  • Ducassou A, Murraciole X, Chaltiel L, Bolle S, Claude L, Bernier V, Coche-Dequeant B, Supiot S, Huchet A, Kerr C, Nguyen TD, Alapetite C, Tensaouti F, Liceaga S, Filleron T, Laprie A (2015) OC-0309: Role of age, grade and RT dose on outcome of 177 ependymoma—13 years experience of Child’s cancer French Society. Radiother Oncol 115(Supplement 1):S155

    Article  Google Scholar 

  • Duffner PK, Horowitz ME, Krischer JP, Friedman HS, Burger PC, Cohen ME, Sanford RA, Mulhern RK, James HE, Freeman CR et al (1993) Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. N Engl J Med 328:1725–1731

    Article  CAS  PubMed  Google Scholar 

  • Duffner PK, Krischer JP, Sanford RA, Horowitz ME, Burger PC, Cohen ME, Friedman HS, Kun LE (1998) Prognostic factors in infants and very young children with intracranial ependymomas. Pediatr Neurosurg 28:215–222

    Article  CAS  PubMed  Google Scholar 

  • Dyer S, Prebble E, Davison V, Davies P, Ramani P, Ellison D, Grundy R (2002) Genomic imbalances in pediatric intracranial ependymomas define clinically relevant groups. Am J Pathol 161:2133–2141

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Ebert C, von Haken M, Meyer-Puttlitz B, Wiestler OD, Reifenberger G, Pietsch T, von Deimling A (1999) Molecular genetic analysis of ependymal tumors. NF2 mutations and chromosome 22q loss occur preferentially in intramedullary spinal ependymomas. Am J Pathol 155:627–632

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Ellison DW, Kocak M, Figarella-Branger D, Felice G, Catherine G, Pietsch T, Frappaz D, Massimino M, Grill J, Boyett JM, Grundy RG (2011) Histopathological grading of pediatric ependymoma: reproducibility and clinical relevance in European trial cohorts. J Negat Results Biomed 10:7

    Article  PubMed  PubMed Central  Google Scholar 

  • Evans AE, Anderson JR, Lefkowitz-Boudreaux IB, Finlay JL (1996) Adjuvant chemotherapy of childhood posterior fossa ependymoma: cranio-spinal irradiation with or without adjuvant Ccnu, vincristine, and prednisone: a Children’s Cancer Group study. Med Pediatr Oncol 27:8–14

    Article  CAS  PubMed  Google Scholar 

  • Ferrante L, Mastronardi L, Celli P, Lunardi P, Acqui M, Fortuna A (1992) Intramedullary spinal cord ependymomas—a study of 45 cases with long-term follow-up. Acta Neurochir 119:74–79

    Article  CAS  PubMed  Google Scholar 

  • Foreman NK, Love S, Gill SS, Coakham HB (1997) Second-look surgery for incompletely resected fourth ventricle ependymomas: technical case report. Neurosurgery 40:856–860; discussion 860

    Article  CAS  PubMed  Google Scholar 

  • Gajjar A, Packer RJ, Foreman NK, Cohen K, Haas-Kogan D, Merchant TE (2013) Children’s Oncology Group’s 2013 blueprint for research: central nervous system tumors. Pediatr Blood Cancer 60:1022–1026

    Article  PubMed  Google Scholar 

  • Gandola L, Pecori E, Scarzello G, Barra S, Mascarin M, Scoccianti S, Diletto B, Mussano A, Garré ML, Sardi I, Meroni S, Biassoni V, Pignoli E, Giangaspero F, Massimino M (2015) OC-0310: Hypofractionated radiotherapy (RT) boost for children with Ependymoma and a measurable residue after surgery. Radiother Oncol 115(Supplement 1):S155–S156

    Article  Google Scholar 

  • Garvin JH Jr, Selch MT, Holmes E, Berger MS, Finlay JL, Flannery A, Goldwein JW, Packer RJ, Rorke-Adams LB, Shiminski-Maher T, Sposto R, Stanley P, Tannous R, Pollack IF (2012) Phase II study of pre-irradiation chemotherapy for childhood intracranial ependymoma. Children’s Cancer Group protocol 9942: a report from the Children’s Oncology Group. Pediatr Blood Cancer 59:1183–1189

    Article  PubMed  Google Scholar 

  • Godfraind C, Kaczmarska JM, Kocak M, Dalton J, Wright KD, Sanford RA, Boop FA, Gajjar A, Merchant TE, Ellison DW (2012) Distinct disease-risk groups in pediatric supratentorial and posterior fossa ependymomas. Acta Neuropathol 124:247–257

    Article  PubMed  PubMed Central  Google Scholar 

  • Goldwein JW, Leahy JM, Packer RJ, Sutton LN, Curran WJ, Rorke LB, Schut L, Littman PS, D’angio GJ (1990) Intracranial ependymomas in children. Int J Radiat Oncol Biol Phys 19:1497–1502

    Article  CAS  PubMed  Google Scholar 

  • Hanbali F, Fourney DR, Marmor E, Suki D, Rhines LD, Weinberg JS, Mccutcheon IE, Suk I, Gokaslan ZL (2002) Spinal cord ependymoma: radical surgical resection and outcome. Neurosurgery 51:1162–1172; discussion 1172–4

    Article  PubMed  Google Scholar 

  • Hodgson DC, Goumnerova LC, Loeffler JS, Dutton S, Black PM, Alexander E 3rd, Xu R, Kooy H, Silver B, Tarbell NJ (2001) Radiosurgery in the management of pediatric brain tumors. Int J Radiat Oncol Biol Phys 50:929–935

    Article  CAS  PubMed  Google Scholar 

  • Hoffman LM, Plimpton SR, Foreman NK, Stence NV, Hankinson TC, Handler MH, Hemenway MS, Vibhakar R, Liu AK (2014) Fractionated stereotactic radiosurgery for recurrent ependymoma in children. J Neuro-Oncol 116:107–111

    Article  Google Scholar 

  • Hua C, Bass JK, Khan R, Kun LE, Merchant TE (2008) Hearing loss after radiotherapy for pediatric brain tumors: effect of cochlear dose. Int J Radiat Oncol Biol Phys 72:892–899

    Article  PubMed  Google Scholar 

  • Hukin J, Epstein F, Lefton D, Allen J (1998) Treatment of intracranial ependymoma by surgery alone. Pediatr Neurosurg 29:40–45

    Article  CAS  PubMed  Google Scholar 

  • Johnson RA, Wright KD, Poppleton H, Mohankumar KM, Finkelstein D, Pounds SB, Rand V, Leary SE, White E, Eden C, Hogg T, Northcott P, Mack S, Neale G, Wang YD, Coyle B, Atkinson J, Dewire M, Kranenburg TA, Gillespie Y, Allen JC, Merchant T, Boop FA, Sanford RA, Gajjar A, Ellison DW, Taylor MD, Grundy RG, Gilbertson RJ (2010) Cross-species genomics matches driver mutations and cell compartments to model ependymoma. Nature 466:632–636

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Kano H, Yang HC, Kondziolka D, Niranjan A, Arai Y, Flickinger JC, Lunsford LD (2010) Stereotactic radiosurgery for pediatric recurrent intracranial ependymomas. J Neurosurg Pediatr 6:417–423

    Article  PubMed  Google Scholar 

  • Kernohan JW (1932) Primary tumors of the spinal cord and intradural filum terminale. In: Penfield W (ed) Cytology and cellular pathology of the nervous system. Paul B. Hoeber, New York

    Google Scholar 

  • Kilday JP, Mitra B, Domerg C, Ward J, Andreiuolo F, Osteso-Ibanez T, Mauguen A, Varlet P, Le Deley MC, Lowe J, Ellison DW, Gilbertson RJ, Coyle B, Grill J, Grundy RG (2012) Copy number gain of 1q25 predicts poor progression-free survival for pediatric intracranial ependymomas and enables patient risk stratification: a prospective European clinical trial cohort analysis on behalf of the Children’s Cancer Leukaemia Group (CCLG), Societe Francaise d’Oncologie Pediatrique (SFOP), and International Society for Pediatric Oncology (SIOP). Clin Cancer Res 18:2001–2011

    Article  CAS  PubMed  Google Scholar 

  • Kool M, Korshunov A, Remke M, Jones DT, Schlanstein M, Northcott PA, Cho YJ, Koster J, Schouten-Van Meeteren A, van Vuurden D, Clifford SC, Pietsch T, von Bueren AO, Rutkowski S, Mccabe M, Collins VP, Backlund ML, Haberler C, Bourdeaut F, Delattre O, Doz F, Ellison DW, Gilbertson RJ, Pomeroy SL, Taylor MD, Lichter P, Pfister SM (2012) Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of Wnt, Shh, Group 3, and Group 4 medulloblastomas. Acta Neuropathol 123:473–484

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Korshunov A, Witt H, Hielscher T, Benner A, Remke M, Ryzhova M, Milde T, Bender S, Wittmann A, Schottler A, Kulozik AE, Witt O, von Deimling A, Lichter P, Pfister S (2010) Molecular staging of intracranial ependymoma in children and adults. J Clin Oncol 28:3182–3190

    Article  PubMed  Google Scholar 

  • Kovnar E, Curran W, Tomita T, Burger P, Langston J, Kepner J, Kun L (1998) Hyperfractionated irradiation for childhood ependymoma: improved local control in subtotally resected tumors (abstract). Childs Nerv Syst 14:489

    Google Scholar 

  • Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114:97–109

    Article  PubMed  PubMed Central  Google Scholar 

  • MacDonald SM, Yock TI (2010) Proton beam therapy following resection for childhood ependymoma. Childs Nerv Syst 26:285–291

    Google Scholar 

  • Mack SC, Witt H, Piro RM, Gu L, Zuyderduyn S, Stutz AM, Wang X, Gallo M, Garzia L, Zayne K, Zhang X, Ramaswamy V, Jager N, Jones DT, Sill M, Pugh TJ, Ryzhova M, Wani KM, Shih DJ, Head R, Remke M, Bailey SD, Zichner T, Faria CC, Barszczyk M, Stark S, Seker-Cin H, Hutter S, Johann P, Bender S, Hovestadt V, Tzaridis T, Dubuc AM, Northcott PA, Peacock J, Bertrand KC, Agnihotri S, Cavalli FM, Clarke I, Nethery-Brokx K, Creasy CL, Verma SK, Koster J, Wu X, Yao Y, Milde T, Sin-Chan P, Zuccaro J, Lau L, Pereira S, Castelo-Branco P, Hirst M, Marra MA, Roberts SS, Fults D, Massimi L, Cho YJ, van Meter T, Grajkowska W, Lach B, Kulozik AE, von Deimling A, Witt O, Scherer SW, Fan X, Muraszko KM, Kool M, Pomeroy SL, Gupta N, Phillips J, Huang A, Tabori U, Hawkins C, Malkin D, Kongkham PN, Weiss WA, Jabado N, Rutka JT, Bouffet E, Korbel JO, Lupien M, Aldape KD, Bader GD, Eils R, Lichter P, Dirks PB, Pfister SM, Korshunov A, Taylor MD (2014) Epigenomic alterations define lethal CIMP-positive ependymomas of infancy. Nature 506:445–450

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Mason WP, Goldman S, Yates AJ, Boyett J, Li H, Finlay JL (1998) Survival following intensive chemotherapy with bone marrow reconstitution for children with recurrent intracranial ependymoma—a report of the Children’s Cancer Group. J Neuro-Oncol 37:135–143

    Article  CAS  Google Scholar 

  • Massimino M, Gandola L, Giangaspero F, Sandri A, Valagussa P, Perilongo G, Garre ML, Ricardi U, Forni M, Genitori L, Scarzello G, Spreafico F, Barra S, Mascarin M, Pollo B, Gardiman M, Cama A, Navarria P, Brisigotti M, Collini P, Balter R, Fidani P, Stefanelli M, Burnelli R, Potepan P, Podda M, Sotti G, Madon E (2004) Hyperfractionated radiotherapy and chemotherapy for childhood ependymoma: final results of the first prospective AIEOP (Associazione Italiana di Ematologia-Oncologia Pediatrica) study. Int J Radiat Oncol Biol Phys 58:1336–1345

    Article  PubMed  Google Scholar 

  • Mendrzyk F, Korshunov A, Benner A, Toedt G, Pfister S, Radlwimmer B, Lichter P (2006) Identification of gains on 1q and epidermal growth factor receptor overexpression as independent prognostic markers in intracranial ependymoma. Clin Cancer Res 12:2070–2079

    Article  CAS  PubMed  Google Scholar 

  • Merchant TE (2001) Observation or radiation therapy and/or chemotherapy and second surgery in treating children who have undergone surgery for ependymoma (ClinicalTrials.gov Identified: NCT00027846)

  • Merchant TE (2009) Three-dimensional conformal radiation therapy for ependymoma. Childs Nerv Syst 25:1261–1268

    Article  PubMed  Google Scholar 

  • Merchant TE, Boop FA, Kun LE, Sanford RA (2008) A retrospective study of surgery and reirradiation for recurrent ependymoma. Int J Radiat Oncol Biol Phys 71:87–97

    Article  PubMed  Google Scholar 

  • Merchant TE, Chitti RM, Li C, Xiong X, Sanford RA, Khan RB (2010) Factors associated with neurological recovery of brainstem function following postoperative conformal radiation therapy for infratentorial ependymoma. Int J Radiat Oncol Biol Phys 76:496–503

    Article  PubMed  Google Scholar 

  • Merchant TE, Fouladi M (2005) Ependymoma: new therapeutic approaches including radiation and chemotherapy. J Neuro-Oncol 75:287–299

    Article  Google Scholar 

  • Merchant TE, Haida T, Wang MH, Finlay JL, Leibel SA (1997) Anaplastic ependymoma: treatment of pediatric patients with or without craniospinal radiation therapy. J Neurosurg 86:943–949

    Article  CAS  PubMed  Google Scholar 

  • Merchant TE, Happersett L, Finlay JL, Leibel SA (1999) Preliminary results of conformal radiation therapy for medulloblastoma. Neuro-Oncology 1:177–187

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Merchant TE, Jenkins JJ, Burger PC, Sanford RA, Sherwood SH, Jones-Wallace D, Heideman RL, Thompson SJ, Helton KJ, Kun LE (2002a) Influence of tumor grade on time to progression after irradiation for localized ependymoma in children. Int J Radiat Oncol Biol Phys 53:52–57

    Article  PubMed  Google Scholar 

  • Merchant TE, Kiehna EN, Li C, Xiong X, Mulhern RK (2005) Radiation dosimetry predicts IQ after conformal radiation therapy in pediatric patients with localized ependymoma. Int J Radiat Oncol Biol Phys 63:1546–1554

    Article  PubMed  Google Scholar 

  • Merchant TE, Kiehna EN, Thompson SJ, Heideman R, Sanford RA, Kun LE (2000) Pediatric low-grade and ependymal spinal cord tumors. Pediatr Neurosurg 32:30–36

    Article  CAS  PubMed  Google Scholar 

  • Merchant TE, Lee H, Zhu J, Xiong X, Wheeler G, Phipps S, Boop FA, Sanford RA (2004b) The effects of hydrocephalus on intelligence quotient in children with localized infratentorial ependymoma before and after focal radiation therapy. J Neurosurg 101:159–168

    Article  PubMed  Google Scholar 

  • Merchant TE, Li C, Xiong X, Kun LE, Boop FA, Sanford RA (2009) Conformal radiotherapy after surgery for paediatric ependymoma: a prospective study. Lancet Oncol 10:258–266

    Article  PubMed  PubMed Central  Google Scholar 

  • Merchant TE, Mulhern RK, Krasin MJ, Kun LE, Williams T, Li C, Xiong X, Khan RB, Lustig RH, Boop FA, Sanford RA (2004a) Preliminary results from a phase II trial of conformal radiation therapy and evaluation of radiation-related CNS effects for pediatric patients with localized ependymoma. J Clin Oncol 22:3156–3162

    Article  PubMed  Google Scholar 

  • Merchant TE, Rose SR, Bosley C, Wu S, Xiong X, Lustig RH (2011) Growth hormone secretion after conformal radiation therapy in pediatric patients with localized brain tumors. J Clin Oncol 29:4776–4780

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Merchant TE, Sharma S, Xiong X, Wu S, Conklin H (2014) Effect of cerebellum radiation dosimetry on cognitive outcomes in children with infratentorial ependymoma. Int J Radiat Oncol Biol Phys 90:547–553

    Article  PubMed  PubMed Central  Google Scholar 

  • Merchant TE, Zhu Y, Thompson SJ, Sontag MR, Heideman RL, Kun LE (2002b) Preliminary results from a Phase II trail of conformal radiation therapy for pediatric patients with localised low-grade astrocytoma and ependymoma. Int J Radiat Oncol Biol Phys 52:325–332

    Article  PubMed  Google Scholar 

  • Modena P, Buttarelli FR, Miceli R, Piccinin E, Baldi C, Antonelli M, Morra I, Lauriola L, di Rocco C, Garre ML, Sardi I, Genitori L, Maestro R, Gandola L, Facchinetti F, Collini P, Sozzi G, Giangaspero F, Massimino M (2012) Predictors of outcome in an AIEOP series of childhood ependymomas: a multifactorial analysis. Neuro-Oncology 14:1346–1356

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Morris EB, Li C, Khan RB, Sanford RA, Boop F, Pinlac R, Xiong X, Merchant TE (2009) Evolution of neurological impairment in pediatric infratentorial ependymoma patients. J Neuro-Oncol 94:391–398

    Article  Google Scholar 

  • Nazar GB, Hoffman HJ, Becker LE, Jenkin D, Humphreys RP, Hendrick EB (1990) Infratentorial ependymomas in childhood: prognostic factors and treatment. J Neurosurg 72:408–417

    Article  CAS  PubMed  Google Scholar 

  • Netson KL, Conklin HM, Wu S, Xiong X, Merchant TE (2012) A 5-year investigation of children’s adaptive functioning following conformal radiation therapy for localized ependymoma. Int J Radiat Oncol Biol Phys 84:217–223.e1

    Article  PubMed  PubMed Central  Google Scholar 

  • Pajtler KW, Witt H, Sill M, Jones DT, Hovestadt V, Kratochwil F, Wani K, Tatevossian R, Punchihewa C, Johann P, Reimand J, Warnatz HJ, Ryzhova M, Mack S, Ramaswamy V, Capper D, Schweizer L, Sieber L, Wittmann A, Huang Z, van Sluis P, Volckmann R, Koster J, Versteeg R, Fults D, Toledano H, Avigad S, Hoffman LM, Donson AM, Foreman N, Hewer E, Zitterbart K, Gilbert M, Armstrong TS, Gupta N, Allen JC, Karajannis MA, Zagzag D, Hasselblatt M, Kulozik AE, Witt O, Collins VP, von Hoff K, Rutkowski S, Pietsch T, Bader G, Yaspo ML, von Deimling A, Lichter P, Taylor MD, Gilbertson R, Ellison DW, Aldape K, Korshunov A, Kool M, Pfister SM (2015) Molecular classification of ependymal tumors across all CNS compartments, histopathological grades, and age groups. Cancer Cell 27:728–743

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Parker M, Mohankumar KM, Punchihewa C, Weinlich R, Dalton JD, Li Y, Lee R, Tatevossian RG, Phoenix TN, Thiruvenkatam R, White E, Tang B, Orisme W, Gupta K, Rusch M, Chen X, Li Y, Nagahawhatte P, Hedlund E, Finkelstein D, Wu G, Shurtleff S, Easton J, Boggs K, Yergeau D, Vadodaria B, Mulder HL, Becksfort J, Gupta P, Huether R, Ma J, Song G, Gajjar A, Merchant T, Boop F, Smith AA, Ding L, Lu C, Ochoa K, Zhao D, Fulton RS, Fulton LL, Mardis ER, Wilson RK, Downing JR, Green DR, Zhang J, Ellison DW, Gilbertson RJ (2014) C11orf95-RELA fusions drive oncogenic NF-kappaB signalling in ependymoma. Nature 506:451–455

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Paulino AC, Wen BC, Buatti JM, Hussey DH, Zhen WK, Mayr NA, Menezes AH (2002) Intracranial ependymomas: an analysis of prognostic factors and patterns of failure. Am J Clin Oncol 25:117–122

    Article  PubMed  Google Scholar 

  • Perilongo G, Massimino M, Sotti G, Belfontali T, Masiero L, Rigobello L, Garre L, Carli M, Lombardi F, Solero C, Sainati L, Canale V, Del Prever AB, Giangaspero F, Andreussi L, Mazza C, Madon E (1997) Analyses of prognostic factors in a retrospective review of 92 children with ependymoma: Italian Pediatric Neuro-oncology Group. Med Pediatr Oncol 29:79–85

    Article  CAS  PubMed  Google Scholar 

  • Pica A, Miller R, Villa S, Kadish SP, Anacak Y, Abusaris H, Ozyigit G, Baumert BG, Zaucha R, Haller G, Weber DC (2009) The results of surgery, with or without radiotherapy, for primary spinal myxopapillary ependymoma: a retrospective study from the rare cancer network. Int J Radiat Oncol Biol Phys 74:1114–1120

    Article  PubMed  Google Scholar 

  • Pollack IF, Gerszten PC, Martinez AJ, Lo KH, Shultz B, Albright AL, Janosky J, Deutsch M (1995) Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 37:655–666; discussion 666–7

    Article  CAS  PubMed  Google Scholar 

  • Reni M, Gatta G, Mazza E, Vecht C (2007) Ependymoma. Crit Rev Oncol Hematol 63:81–89

    Article  PubMed  Google Scholar 

  • Rezai AR, Woo HH, Lee M, Cohen H, Zagzag D, Epstein FJ (1996) Disseminated ependymomas of the central nervous system. J Neurosurg 85:618–624

    Article  CAS  PubMed  Google Scholar 

  • Robertson PL, Zeltzer PM, Boyett JM, Rorke LB, Allen JC, Geyer JR, Stanley P, Li H, Albright AL, Mcguire-Cullen P, Finlay JL, Stevens KR Jr, Milstein JM, Packer RJ, Wisoff J (1998) Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children’s Cancer Group. J Neurosurg 88:695–703

    Article  CAS  PubMed  Google Scholar 

  • Rousseau P, Habrand JL, Sarrazin D, Kalifa C, Terrier-Lacombe MJ, Rekacewicz C, Rey A (1994) Treatment of intracranial ependymomas of children: review of a 15-year experience. Int J Radiat Oncol Biol Phys 28:381–386

    Article  CAS  PubMed  Google Scholar 

  • Rubio MP, Correa KM, Ramesh V, Maccollin MM, Jacoby LB, von Deimling A, Gusella JF, Louis DN (1994) Analysis of the neurofibromatosis 2 gene in human ependymomas and astrocytomas. Cancer Res 54:45–47

    CAS  PubMed  Google Scholar 

  • Sabin ND, Merchant TE, Li X, Li Y, Klimo P Jr, Boop FA, Ellison DW, Ogg RJ (2016) Quantitative imaging analysis of posterior fossa ependymoma location in children. Childs Nerv Syst

    Google Scholar 

  • Salazar OM, Rubin P, Bassano D, Marcial VA (1975) Improved survival of patients with intracranial ependymomas by irradiation: doseselection and field extension. Cancer 35:1563–1573

    Article  CAS  PubMed  Google Scholar 

  • Schroeder TM, Chintagumpala M, Okcu MF, Chiu JK, Teh BS, Woo SY, Paulino AC (2008) Intensity-modulated radiation therapy in childhood ependymoma. Int J Radiat Oncol Biol Phys 71:987–993

    Article  PubMed  Google Scholar 

  • Shim KW, Kim DS, Choi JU (2009) The history of ependymoma management. Childs Nerv Syst 25:1167–1183

    Article  PubMed  Google Scholar 

  • Smith AA (2010) Maintenance chemotherapy or observation following induction chemotherapy and radiation therapy in treating younger patients with newly diagnosed ependymoma (ClinicalTrials.gov Identifier: NCT01096368)

  • Smyth MD, Horn BN, Russo C, Berger MS (2000) Intracranial ependymomas of childhood: current management strategies. Pediatr Neurosurg 33:138–150

    Article  CAS  PubMed  Google Scholar 

  • Sonneland PR, Scheithauer BW, Onofrio BM (1985) Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases. Cancer 56:883–893

    Article  CAS  PubMed  Google Scholar 

  • Stafford SL, Pollock BE, Foote RL, Gorman DA, Nelson DF, Schomberg PJ (2000) Stereotactic radiosurgery for recurrent ependymoma. Cancer 88:870–875

    Article  CAS  PubMed  Google Scholar 

  • Stauder MC, Ni Laack N, Ahmed KA, Link MJ, Schomberg PJ, Pollock BE (2012) Stereotactic radiosurgery for patients with recurrent intracranial ependymomas. J Neuro-Oncol 108:507–512

    Article  Google Scholar 

  • Stuben G, Stuschke M, Kroll M, Havers W, Sack H (1997) Postoperative radiotherapy of spinal and intracranial ependymomas: analysis of prognostic factors. Radiother Oncol 45:3–10

    Article  CAS  PubMed  Google Scholar 

  • Sutton LN, Goldwein J, Perilongo G, Lang B, Schut L, Rorke L, Packer R (1990) Prognostic factors in childhood ependymomas. Pediatr Neurosurg 16:57–65

    Article  PubMed  Google Scholar 

  • Tamburrini G, D’ercole M, Pettorini BL, Caldarelli M, Massimi L, di Rocco C (2009) Survival following treatment for intracranial ependymoma: a review. Childs Nerv Syst 25:1303–1312

    Article  CAS  PubMed  Google Scholar 

  • Taylor MD, Poppleton H, Fuller C, Su X, Liu Y, Jensen P, Magdaleno S, Dalton J, Calabrese C, Board J, Macdonald T, Rutka J, Guha A, Gajjar A, Curran T, Gilbertson RJ (2005) Radial glia cells are candidate stem cells of ependymoma. Cancer Cell 8:323–335

    Article  CAS  PubMed  Google Scholar 

  • Taylor RE (2004) Review of radiotherapy dose and volume for intracranial ependymoma. Pediatr Blood Cancer 42:457–460

    Article  PubMed  Google Scholar 

  • Teo C, Nakaji P, Symons P, Tobias V, Cohn R, Smee R (2003) Ependymoma. Childs Nerv Syst 19:270–285

    Article  PubMed  Google Scholar 

  • Timmermann B, Kortmann RD, Kuhl J, Rutkowski S, Dieckmann K, Meisner C, Bamberg M (2005) Role of radiotherapy in anaplastic ependymoma in children under age of 3 years: results of the prospective German brain tumor trials HIT-SKK 87 and 92. Radiother Oncol 77:278–285

    Article  PubMed  Google Scholar 

  • Timmermann B, Lomax AJ, Nobile L, Grotzer MA, Weiss M, Kortmann RD, Bolsi A, Goitein G (2007) Novel technique of craniospinal axis proton therapy with the spot-scanning system: avoidance of patching multiple fields and optimized ventral dose distribution. Strahlenther Onkol 183:685–688

    Article  PubMed  Google Scholar 

  • Vaidya K, Smee R, Williams JR (2012) Prognostic factors and treatment options for paediatric ependymomas. J Clin Neurosci 19:1228–1235

    Article  CAS  PubMed  Google Scholar 

  • Vanuytsel L, Brada M (1991) The role of prophylactic spinal irradiation in localized intracranial ependymoma. Int J Radiat Oncol Biol Phys 21:825–830

    Article  CAS  PubMed  Google Scholar 

  • Venkatramani R, Dhall G, Patel M, Grimm J, Hawkins C, Mccomb G, Krieger M, Wong K, O’neil S, Finlay JL (2012) Supratentorial ependymoma in children: to observe or to treat following gross total resection? Pediatr Blood Cancer 58:380–383

    Article  PubMed  Google Scholar 

  • Volpp PB, Han K, Kagan AR, Tome M (2007) Outcomes in treatment for intradural spinal cord ependymomas. Int J Radiat Oncol Biol Phys 69:1199–1204

    Article  PubMed  Google Scholar 

  • Wallner KE, Wara WM, Sheline GE, Davis RL (1986) Intracranial ependymomas: results of treatment with partial or whole brain irradiation without spinal irradiation. Int J Radiat Oncol Biol Phys 12:1937–1941

    Article  CAS  PubMed  Google Scholar 

  • Wani K, Armstrong TS, Vera-Bolanos E, Raghunathan A, Ellison D, Gilbertson R, Vaillant B, Goldman S, Packer RJ, Fouladi M, Pollack I, Mikkelsen T, Prados M, Omuro A, Soffietti R, Ledoux A, Wilson C, Long L, Gilbert MR, Aldape K (2012) A prognostic gene expression signature in infratentorial ependymoma. Acta Neuropathol 123:727–738

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Witt H, Mack SC, Ryzhova M, Bender S, Sill M, Isserlin R, Benner A, Hielscher T, Milde T, Remke M, Jones DT, Northcott PA, Garzia L, Bertrand KC, Wittmann A, Yao Y, Roberts SS, Massimi L, van Meter T, Weiss WA, Gupta N, Grajkowska W, Lach B, Cho YJ, von Deimling A, Kulozik AE, Witt O, Bader GD, Hawkins CE, Tabori U, Guha A, Rutka JT, Lichter P, Korshunov A, Taylor MD, Pfister SM (2011) Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma. Cancer Cell 20:143–157

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Zacharoulis S, Moreno L (2009) Ependymoma: an update. J Child Neurol 24:1431–1438

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Radiation Oncology Department, Centro Infantil Dr. Domingo A. Boldrini, University of Campinas, São Paulo, Brazil

    Rosangela Correa Villar M.D., Ph.D.

  2. Radiation Oncology Department, Institute of Radiology, Hospital das Clinicas, University of São Paulo, 1270, Dr. Gabriel Porto Street, Cidade Universitaria Campinas, São Paulo, 13083-210, Brazil

    Rosangela Correa Villar M.D., Ph.D.

  3. Department of Radiation Oncology, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, Memphis, TN, 38105-3678, USA

    Thomas E. Merchant D.O., Ph.D.

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  1. Rosangela Correa Villar M.D., Ph.D.
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  2. Thomas E. Merchant D.O., Ph.D.
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Correspondence to Thomas E. Merchant D.O., Ph.D. .

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Editors and Affiliations

  1. Department of Radiation Oncology, St Jude Children’s Research Hospital, Memphis, Tennessee, USA

    Thomas E. Merchant

  2. Department of Radiation Oncology, University of Leipzig, Leipzig, Germany

    Rolf-Dieter Kortmann

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Villar, R.C., Merchant, T.E. (2018). Childhood Ependymoma. In: Merchant, T., Kortmann, RD. (eds) Pediatric Radiation Oncology. Pediatric Oncology. Springer, Cham. https://doi.org/10.1007/978-3-319-43545-9_12

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