Abstract
Despite its frequency (third most common brain tumor in children), publications about ependymomas per year are three times less frequent than for medulloblastomas (120 vs. 330). The knowledge and the sophistication of the protocols are lagging behind those of medulloblastomas. Accordingly, this has limited the progress made for these tumors. The improvement of neurosurgery is probably the driving force improving the outcome of children affected with this disease in the last 20 years. The three main questions that remain for ependymomas are the following: (i) Which biomarkers are useful to understand the oncogenesis and prognosis? (ii) Is there a role for chemotherapy? (iii) What is the appropriate strategy at the time of relapse?
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References
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(3):258–266
Grill J, Chastagner P, Kalifa C (2003) Childhood ependymoma: a systematic review of treatment options and strategies. Paediatr Drugs 5(8):533–543
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) Children’s Oncology Group. 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(7):1183–1189
Palma L, Celli P, Mariottini A, Zalaffi A, Schettini G (2000) The importance of surgery in supratentorial ependymomas. Long-term survival in a series of 23 cases. The importance of surgery in supratentorial ependymomas. Long-term survival in a series of 23 cases. Childs Nerv Syst 16(3):170–175
Hukin J, Epstein F, Lefton D, Allen J (1998) Treatment of intracranial ependymoma by surgery alone. Pediatr Neurosurg 29(1):40–45
Figueiredo N, Brooks N, Resnick DK (2013) Evidence-based review and guidelines for the management of myxopapillary and intramedullary ependymoma. J Neurosurg Sci 57(4):327–341
Oh MC, Sayegh ET, Safaee M, Sun MZ, Kaur G, Kim JM, Aranda D, Molinaro AM, Gupta N, Parsa AT (2013) Prognosis by tumor location for pediatric spinal cord ependymomas. J Neurosurg Pediatr 11(3):282–288
Grill J, Le Deley MC, Gambarelli D, Raquin MA, Couanet D, Pierre-Kahn A, Habrand JL, Doz F, Frappaz D, Gentet JC, Edan C, Chastagner P, Kalifa C (2001) French Society of Pediatric Oncology. Postoperative chemotherapy without irradiation for ependymoma in children under 5 years of age: a multicenter trial of the French Society of Pediatric Oncology. J Clin Oncol 19(5):1288–1296
Grundy RG, Wilne SH, Robinson KJ, Ironside JW, Cox T, Chong WK, Michalski A, Campbell RH, Bailey CC, Thorp N, Pizer B, Punt J, Walker DA, Ellison DW, Machin D (2010) Children’s Cancer and Leukaemia Group (formerly UKCCSG) Brain Tumour Committee. primary postoperative chemotherapy without radiotherapy for treatment of brain tumours other than ependymoma in children under 3 years: results of the first UKCCSG/SIOP CNS 9204 trial. Eur J Cancer 46(1):120–133
von Hoff K, Kieffer V, Habrand JL, Kalifa C, Dellatolas G, Grill J (2008) Impairment of intellectual functions after surgery and posterior fossa irradiation in children with ependymoma is related to age and neurologic complications. BMC Cancer 8:15
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(24):3965–3970
Landau E, Boop FA, Conklin HM, Wu S, Xiong X, Merchant TE (2013) Supratentorial ependymoma: disease control, complications, and functional outcomes after irradiation. Int J Radiat Oncol Biol Phys 85(4):e193–e199
Strother DR, Lafay-Cousin L, Boyett JM, Burger P, Aronin P, Constine L, Duffner P, Kocak M, Kun LE, Horowitz ME, Gajjar A (2014) Benefit from prolonged dose-intensive chemotherapy for infants with malignant brain tumors is restricted to patients with ependymoma: a report of the Pediatric Oncology Group randomized controlled trial 9233/34. Neuro Oncol 16(3):457–465
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(2):381–386
Paulino AC (2001) The local field in infratentorial ependymoma: does the entire posterior fossa need to be treated? Int J Radiat Oncol Biol Phys 49(3):757–761
Evans AE, Anderson JR, Lefkowitz-Boudreaux IB, Finlay JL (2014) Adjuvant chemotherapy of childhood posterior fossa ependymoma: cranio-spinal irradiation with or without adjuvant CCNU, vincristine, and prednisone: a Childrens Cancer Group study. Neuro Oncol 16(3):457–465
Massimino M, Gandola L, Giangaspero F, Sandri A, Valagussa P, Perilongo G, Garrè 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) AIEOP Pediatric Neuro-Oncology Group. 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(5):1336–1345
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(1):87–97
Fouladi M, Baruchel S, Chan H, Grant R, Malkin D, Weitzman S, Greenberg ML (1998) Use of adjuvant ICE chemotherapy in the treatment of anaplastic ependymomas. Childs Nerv Syst 14(10):590–595
Grill J, Kalifa C, Doz F, Schoepfer C, Sainte-Rose C, Couanet D, Terrier-Lacombe MJ, Valteau-Couanet D, Hartmann O (1996) A high-dose busulfan-thiotepa combination followed by autologous bone marrow transplantation in childhood recurrent ependymoma. A phase-II study. Pediatr Neurosurg 25(1):7–12
Gururangan S, Fangusaro J, Young Poussaint T, Onar-Thomas A, Gilbertson RJ, Vajapeyam S, Gajjar A, Goldman S, Friedman HS, Packer RJ, Boyett JM, Kun LE, McLendon R (2012) Lack of efficacy of bevacizumab + irinotecan in cases of pediatric recurrent ependymoma–a pediatric brain tumor consortium study. Neuro Oncol 14(11):1404–1412
Robison NJ, Campigotto F, Chi SN, Manley PE, Turner CD, Zimmerman MA, Chordas CA, Werger AM, Allen JC, Goldman S, Rubin JB, Isakoff MS, Pan WJ, Khatib ZA, Comito MA, Bendel AE, Pietrantonio JB, Kondrat L, Hubbs SM, Neuberg DS, Kieran MW (2014) A phase II trial of a multi-agent oral antiangiogenic (metronomic) regimen in children with recurrent or progressive cancer. Pediatr Blood Cancer 61(4):636–642
Atkinson JM, Shelat AA, Carcaboso AM, Kranenburg TA, Arnold LA, Boulos N, Wright K, Johnson RA, Poppleton H, Mohankumar KM, Féau C, Phoenix T, Gibson P, Zhu L, Tong Y, Eden C, Ellison DW, Priebe W, Koul D, Yung WK, Gajjar A, Stewart CF, Guy RK, Gilbertson RJ (2011) An integrated in vitro and in vivo high-throughput screen identifies treatment leads for ependymoma. Cancer Cell 20(3):384–399
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 Neurooncol 116(1):107–111
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(5):417–423
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(5):1541–1548
Lo SS, Abdulrahman R, Desrosiers PM, Fakiris AJ, Witt TC, Worth RM, Dittmer PH, Desrosiers CM, Frost S, Timmerman RD (2006) The role of Gamma Knife Radiosurgery in the management of unresectable gross disease or gross residual disease after surgery in ependymoma. J Neurooncol 79(1):51–56
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
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, 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(7):2001–2011
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(2):143–157
Mack SC, Witt H, Piro RM, Gu L, Zuyderduyn S, Stütz AM, Wang X, Gallo M, Garzia L, Zayne K, Zhang X, Ramaswamy V, Jäger 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(7489):445–450
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, Becksford 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-κB signalling in ependymoma. Nature 506(7489):451–455
Andreiuolo F, Puget S, Peyre M, Dantas-Barbosa C, Boddaert N, Philippe C, Mauguen A, Grill J, Varlet P (2010) Neuronal differentiation distinguishes supratentorial and infratentorial childhood ependymomas. Neuro Oncol 12(11):1126–1134
Andreiuolo F, Ferreira C, Puget S, Grill J (2013) Current and evolving knowledge of prognostic factors for pediatric ependymomas. Future Oncol 9(2):183–191
Rogers HA, Mayne C, Chapman RJ, Kilday JP, Coyle B, Grundy RG (2013) PI3K pathway activation provides a novel therapeutic target for pediatric ependymoma and is an independent marker of progression-free survival. Clin Cancer Res 19(23):6450–6460
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Grill, J., Iglesias, MJ.L. (2015). Oncological Management of Ependymomas. In: Özek, M., Cinalli, G., Maixner, W., Sainte-Rose, C. (eds) Posterior Fossa Tumors in Children. Springer, Cham. https://doi.org/10.1007/978-3-319-11274-9_24
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DOI: https://doi.org/10.1007/978-3-319-11274-9_24
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