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Ependymoma

  • Cassie KlineEmail author
  • Craig Forester
  • Anuradha Banerjee
Chapter
Part of the Pediatric Oncology book series (PEDIATRICO)

Abstract

Ependymomas comprise approximately 10 % of all pediatric central nervous system (CNS) tumors and are the third most common brain tumor in children less than 20 years of age (Gurney et al. 1999). Ependymomas are gliomas that originate from the lineage of cells that give rise to the differentiated ependymal cell layer lining the ventricular system and central canal of the spinal cord. The majority of those arising in the brain, up to two-thirds, arise in the posterior fossa. The percentage of ependymomas arising from the posterior fossa is greatest in children less than 3 years old (Horn et al. 1999). Data from the SEER group (Surveillance, Epidemiology, and End Results) suggest that the annual incidence of ependymoma is 2.6 per million for the 0–14 age group and 2.2 per million for the 0–20 age group (Linet et al. 1999; Ries et al. 1999). Another recent SEER data review from 2012 identified that ependymomas make up about 20 % of spinal cord tumors in patients less than 20 years old. This study also revealed that spinal cord ependymoma diagnoses as a whole have increased over time since 1990, likely due to increased histological precision (Hayden Gephart et al. 2012).

Keywords

Posterior Fossa Gross Total Resection Spinal Cord Tumor Posterior Fossa Tumor Myxopapillary Ependymoma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. Agarwal S, Stevenson ME, Sughrue ME, Wartchow EP, Mierau GW, Fung KM (2014) Features of intraventricular tanycytic ependymoma: report of a case and review of literature. Int J Clin Exp Pathol 7(6):3399–3407PubMedPubMedCentralGoogle Scholar
  2. Aggarwal R, Yeung D, Kumar P, Muhlbauer M, Kun LE (1997) Efficacy and feasibility of stereotactic radiosurgery in the primary management of unfavorable pediatric ependymoma. Radiother Oncol 43:269–273PubMedCrossRefGoogle Scholar
  3. Alfaro-Cervello C, Soriano-Navarro M, Ramirez M, Bernet L, Martinez Banaclocha M, Cano R, Reyes Santias RM, Forteza-Vila J, Garcia-Verdugo JM (2015) Ultrastructural pathology of anaplastic and grade II ependymomas reveals distinctive ciliary structures—electron microscopy redux. Ultrastruct Pathol 39(1):23–29PubMedCrossRefGoogle Scholar
  4. Antony R, Wong KE, Patel M, Olch AJ, McComb G, Krieger M, Gilles F, Sposto R, Erdreich-Epstein A, Dhall G, Gardner S, Finlay J (2015) A retrospective analysis of recurrent intracranial ependymoma. Pediatr Blood Cancer 61:1195–1201CrossRefGoogle Scholar
  5. Atkinson JM, Shelat AA, Carcaboso AM, Kranenburg TA, Arnold LA et al. (2011) An integrated in vitro and in vivo high-throughput screen identifies treatment leads for ependymoma. Cancer Cell 20(3):384–399Google Scholar
  6. Awaad YM, Allen JC, Miller DC, Schneider SJ, Wisoff J, Epstein FJ (1996) Deferring adjuvant therapy for totally resected intracranial ependymoma. Pediatr Neurol 14:216–219PubMedCrossRefGoogle Scholar
  7. Balovannis SJ, Balovannis IS (2014) The fine structure of ependymomas. CNS Oncol 3(1):49–59CrossRefGoogle Scholar
  8. Bergsagel DJ, Finegold MJ, Butel JS, Kupsky WJ, Garcea RL (1992) DNA sequences similar to those of simian virus 40 in ependymomas and choroid plexus tumors of childhood. N Engl J Med 326:988–993PubMedCrossRefGoogle Scholar
  9. Bi Z, Ren X, Zhang J, Jia W (2015) Clinical, radiological, and pathological features in 43 case of intracranial subependymoma. J Neurosurg 122(1):49–60PubMedCrossRefGoogle Scholar
  10. Bijlsma EK, Voesten AMJ, Bijleveld EH, Troost D, Westerveld A, Mérel P et al (1995) Molecular analysis of genetic changes in ependymomas. Genes Chromosom Cancer 13(4):272–277PubMedCrossRefGoogle Scholar
  11. Birch BD, Johnson JP, Parsa A, Desai RD, Yoon JT, Lycette CA, Li YM, Bruce JN (1996) Frequent type 2 neurofibromatosis gene transcript mutations in sporadic intramedullary spinal cord ependymomas. Neurosurgery 39:135–140PubMedCrossRefGoogle Scholar
  12. Bird AP, Wolffe AP (1999) Methylation-induced repression—belts, braces, and chromatin. Cell 99(5):451–454PubMedCrossRefGoogle Scholar
  13. Bouffet E, Tabori U, Bartels U (2007) Paediatric ependymomas: should we avoid radiotherapy? Lancet Oncol 8:665–666PubMedCrossRefGoogle Scholar
  14. Bouffet E, Capra M, Bartels U (2009) Salvage chemotherapy for metastatic and recurrent ependymoma of childhood. Childs Nerv Syst 25:1293–1301PubMedCrossRefGoogle Scholar
  15. Brandes AA, Cavallo G, Reni M, Tosoni A, Nicolardi L, Scopece L, Franceschi E, Sotti G, Talacchi A, Turazzi S, Ermani M (2005) A multicenter retrospective study of chemotherapy for recurrent intracranial ependymal tumors in adults by the Gruppo Italiano Cooperativo di Neuro-Oncologia. Cancer 104:143–148PubMedCrossRefGoogle Scholar
  16. Brannon Morris E, 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 Neurooncol 94:391–398PubMedCrossRefGoogle Scholar
  17. Carbone M, Rizzo P, Pass HI (1997) Simian virus 40, poliovaccines and human tumors: a review of recent developments. Oncogene 15:1877–1888PubMedCrossRefGoogle Scholar
  18. Carter M, Nicholson J, Ross F, Crolla J, Allibone R, Balaji V et al (2002) Genetic abnormalities detected in ependymomas by comparative genomic hybridisation. Br J Cancer 86(6):929–939PubMedPubMedCentralCrossRefGoogle Scholar
  19. Cervoni L, Celli P, Fortuna A, Cantore G (1994) Recurrence of spinal ependymoma. Risk factors and long-term survival. Spine 19:2838–2841PubMedCrossRefGoogle Scholar
  20. Cimino PJ, Agarwal A, Dehner LP (2014) Myxopapillary ependymoma in children: a study of 11 cases and a comparison with the adult experience. Pediatr Blood Cancer 61(11):1969–1971PubMedCrossRefGoogle Scholar
  21. Ciraldo AV, Platt MS, Agamanolis DP, Boeckman CR (1986) Sacrococcygeal myxopapillary ependymomas and ependymal rests in infants and children. J Pediatr Surg 21:49–52PubMedCrossRefGoogle Scholar
  22. 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(5):1536–1542PubMedCrossRefGoogle Scholar
  23. Coulon RA, Till K (1977) Intracranial ependymomas in children: a review of 43 cases. Childs Brain 3:154–168PubMedGoogle Scholar
  24. Debiec-Rychter M, Hagemeijer A, Sciot R (2000) Cytogenetic analysis in three cerebral subependymomas: further evidence for a hamartomatous nature? Cancer Genet Cytogenet 122:63–64PubMedCrossRefGoogle Scholar
  25. DeWire M, Fouladi M, Turner DC, Wetmore C, Hawkins C, Jacobs C, Yuan Y, Liu D, Goldman S, Fisher P, Rytting M, Bouffet E, Khakoo Y, Hwang EI, Foreman N, Stewart CF, Gilbert MR, Gilbertson R, Gajjar A (2015) An open-label, two stage, phase II study of bevacizumab and lapatinib in children with recurrent or refractory ependymoma: a collaborative ependymoma research network study (CERN). J Neuro-Oncol 123:85–91CrossRefGoogle Scholar
  26. Dohrmann GJ, Farwell JR, Flannery JT (1976) Ependymomas and ependymoblastomas in children. J Neurosurg 45:273–283PubMedCrossRefGoogle Scholar
  27. 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 328:1725–1731PubMedCrossRefGoogle Scholar
  28. Evans DGR, Sainio M, Baser ME (2000) Neurofibromatosis type 2. J Med Genet 37(12):897–904PubMedPubMedCentralCrossRefGoogle Scholar
  29. 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 31;10:7Google Scholar
  30. Fassett DR, Pingree J, Kestle JR (2005) The high incidence of tumor dissemination in myxopapillary ependymoma in pediatric patients. Report of five cases and review of the literature. J Neurosurg 102;1 suppl:59–64Google Scholar
  31. Feldman WB, Clark AJ, Safaee M, Ames CP, Parsa AT (2013) Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children: a systematic review. J Neurosurg Spine 19(4):471–476PubMedCrossRefGoogle Scholar
  32. Fiskus W, Wang Y, Sreekumar A, Buckley K, Shi H, Jillella A, Ustun C, Rao R, Fernandez P, Chen J, Balusu R, Koul S, Atadja P, Marquez V, Bhalli K (2009) Combined epigenetic therapy with the histone methyltransferase EZH2 inhibitor 3-deazaneplanocin A and the histone deacetylase inhibitor panobinostat against human AML cells. Blood 114(13):2733–2743PubMedPubMedCentralCrossRefGoogle Scholar
  33. Fouladi M, Helton K, Dalton J, Gilger E, Gajjar A, Merchant T, Kun L, Newsham I, Burger P, Fuller C (2003) Clear cell ependymoma: a clinicopathologic and radiographic analysis of 10 patients. Cancer 98(10):2232–2244PubMedCrossRefGoogle Scholar
  34. Frandsen JE, Wagner A, Bollo RJ, Shrieve DC, Poppe MM (2015) Long-term life expectancy for children with ependymoma and medulloblastoma. Pediatr Blood Cancer 62(11):1986–1991, epubPubMedCrossRefGoogle Scholar
  35. Furie DM, Provenzale JM (1995) Supratentorial ependymomas and subependymomas: CT and MR appearance. J Comput Assist Tomogr 19:518–526PubMedCrossRefGoogle Scholar
  36. Gajjar A, Pfister SM, Taylor MD, Gilbertson RJ (2014) Molecular insights into pediatric brain tumors have the potential to transform therapy. Clin Cancer Res 20(22):5630–5640PubMedPubMedCentralCrossRefGoogle Scholar
  37. Garrett PG, Simpson WJ (1983) Ependymomas: results of radiation treatment. Int J Radiat Oncol Biol Phys 9:1121–1124PubMedCrossRefGoogle Scholar
  38. Garvin J, Sposto R, Stanley P, Rorke L, Packer P (2004) Childhood ependymoma: improved survival for patients with incompletely resected tumors with the use of preirradiation chemotherapy. In: 11th International Symposium on Pediatric Neuro-oncology, BostonGoogle Scholar
  39. Geyer JR, Zeltzer PM, Boyett JM, Rorke LB, Stanley P, Albright AL, Wisoff JH, Milstein JM, Allen JC, Finlay JL et al (1994) Survival of infants with primitive neuroectodermal tumors or malignant ependymomas of the CNS treated with eight drugs in 1 day: a report from the Childrens Cancer Group. J Clin Oncol 12:1607–1615PubMedGoogle Scholar
  40. Ghia AJ, Majaian A, Allen PK, Armstrong TS, Lang FF Jr, Gilbert MR, Brown PD (2013) Supratentorial gross-totally resected non-anaplastic ependymoma: population based patterns of care and outcomes analysis. J Neurooncol 115(3):513–520PubMedCrossRefGoogle Scholar
  41. Gilbertson RJ, Bentley L, Hernan R, Junttila TT, Frank AJ, Haapasalo H, Connelly M, Wetmore C, Curran T, Elenius K, Ellison DW (2002) ERBB receptor signaling promotes ependymoma cell proliferation and represents a potential novel therapeutic target for this disease. Clin Cancer Res 8:3054–3064PubMedGoogle Scholar
  42. Glazer RI, Knode MC, Tseng CK, Haines DR, Marquez VE (1986) 3-Deazaneplanocin A: a new inhibitor of S-adenosylhomocysteine synthesis and its effects in human colon carcinoma cells. Biochem Pharmacol 35(24):4523–4527PubMedCrossRefGoogle Scholar
  43. Godfraind C, Kaczmarska JM, Kocak M, Dalton J, Wright KD, Sanford RA et al (2012) Distinct disease-risk groups in pediatric supratentorial and posterior fossa ependymomas. Acta Neuropathol 124(2):247–257PubMedPubMedCentralCrossRefGoogle Scholar
  44. Gold EB, Leviton A, Lopez R, Austin DF, Gilles FH, Hedley-Whyte ET, Kolonel LN, Lyon JL, Swanson GM, Weiss NS et al (1994) The role of family history in risk of childhood brain tumors. Cancer 73:1302–1311PubMedCrossRefGoogle Scholar
  45. Goldwein JW, Glauser TA, Packer RJ, Finlay JL, Sutton LN, Curran WJ, Laehy JM, Rorke LB, Schut L, D’Angio GJ (1990) Recurrent intracranial ependymomas in children. Survival, patterns of failure, and prognostic factors. Cancer 66:557–563PubMedCrossRefGoogle Scholar
  46. Goldwein JW, Corn BW, Finlay JL, Packer RJ, Rorke LB, Schut L (1991) Is craniospinal irradiation required to cure children with malignant (anaplastic) intracranial ependymomas? Cancer 67:2766–2771PubMedCrossRefGoogle Scholar
  47. Good CD, Wade AM, Hayward RD, Phipps KP, Michalski AJ, Harkness WF, Chong WK (2001) Surveillance neuroimaging in childhood intracranial ependymoma: how effective, how often, and for how long? J Neurosurg 94:27–32PubMedCrossRefGoogle Scholar
  48. Gornet MK, Buckner JC, Marks RS, Scheithauer BW, Erickson BJ (1999) Chemotherapy for advanced CNS ependymoma. J Neurooncol 45:61–67PubMedCrossRefGoogle Scholar
  49. 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 25:7–12PubMedCrossRefGoogle Scholar
  50. Grill J, Renaux VK, Bulteau C, Viguier D, Levy-Piebois C, Sainte-Rose C, Dellatolas G, Raquin MA, Jambaque I, Kalifa C (1999) Long-term intellectual outcome in children with posterior fossa tumors according to radiation doses and volumes. Int J Radiat Oncol Biol Phys 45:137–145PubMedCrossRefGoogle Scholar
  51. 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) 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 19:1288–1296PubMedGoogle Scholar
  52. Grundy RG, Wilne SA, Weston CL, Robinson K, Lashford LS, Ironside J, Cox T, Chong WK, Campbell RH, Bailey CC, Gattamaneni R, Picton S, Thorpe N, Mallucci C, English MW, Punt JA, Walker DA, Ellison DW, Machin D (2007) Primary postoperative chemotherapy without radiotherapy for intracranial ependymoma in children: the UKCCSG/SIOP prospective study. Lancet Oncol 8:696–705PubMedCrossRefGoogle Scholar
  53. Hagel C, Treszl A, Fehlert J, Harder J, von Haxthausen F, Kern M, von Bueren AO, Kordes U (2013) Supra- and infratentorial pediatric ependymomas differ significantly in NeuN, p75, and GFAP expression. J Neurooncol 112(2):191–197PubMedCrossRefGoogle Scholar
  54. Hamilton RL, Pollack IF (1997) The molecular biology of ependymomas. Brain Pathol 7:807–822PubMedCrossRefGoogle Scholar
  55. Hayden Gephart MG, Lober RM, Arrigo RT, Zygourakis CC, Guzman R, Boakye M, Edwards MS, Fisher PG (2012) Trends in the diagnosis and treatment of pediatric primary spinal cord tumors. J Neurosurg Pediatr 10(6):555–559PubMedCrossRefGoogle Scholar
  56. Hemminki K, Li X, Vaittinen P, Dong C (2000) Cancers in the first-degree relatives of children with brain tumours. Br J Cancer 83:407–411PubMedPubMedCentralCrossRefGoogle Scholar
  57. 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 50:929–935PubMedCrossRefGoogle Scholar
  58. Horn B, Heideman R, Geyer R, Pollack I, Packer R, Goldwein J, Tomita T, Schomberg P, Ater J, Luchtman-Jones L, Rivlin K, Lamborn K, Prados M, Bollen A, Berger M, Dahl G, McNeil E, Patterson K, Shaw D, Kubalik M, Russo C (1999) A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors. J Pediatr Hematol Oncol 21:203–211PubMedCrossRefGoogle Scholar
  59. Hukin J, Epstein F, Lefton D, Allen J (1998) Treatment of intracranial ependymoma by surgery alone. Pediatr Neurosurg 29:40–45PubMedCrossRefGoogle Scholar
  60. Hulsebos TJ, Oskam NT, Bijleveld EH, Westerveld A, Hermsen MA, van den Ouweland AM, Hamel BC, Tijssen CC (1999) Evidence for an ependymoma tumour suppressor gene in chromosome region 22pter-22q11.2. Br J Cancer 81:1150–1154PubMedPubMedCentralCrossRefGoogle Scholar
  61. Ilgren EB, Stiller CA, Hughes JT, Silberman D, Steckel N, Kaye A (1984) Ependymomas: a clinical and pathologic study. Part I. Biologic features. Clin Neuropathol 3:113–121PubMedGoogle Scholar
  62. Ishizawa K, Komori T, Aihara Y, Maruyama T, Okada Y, Ikuta S, Muragaki Y, Hirose T, Homma T, Sasaki A (2012) Clear cells are associated with proliferative activity in ependymoma: a quantitative study. Clin Neuropathol 31(3):146–151PubMedCrossRefGoogle Scholar
  63. Kieran MW, Turner CD, Rubin JB, Chi SN, Zimmerman MA, Chordas C, Klement G, Laforme A, Gordon A, Thomas A, Neuberg D, Browder T, Folkman J (2005) A feasibility trial of antiangiogenic (metronomic) chemotherapy in pediatric patients with recurrent or progressive cancer. J Pediatr Hematol Oncol 27:573–581PubMedCrossRefGoogle Scholar
  64. Kikuchi J, Takashina T, Kinoshita I, Kikuchi E, Shimizu Y, Sakakibar-Konishi J et al. (2012) Epigenetic therapy with 3-deazaneplanocin A, an inhibitor of the histone methyltransferase EZH2, inhibits growth of non-small cell lung cancer cells. Lung Cancer 78;2:138–143Google Scholar
  65. Koperek O, Gelpi E, Birner P, Haberler C, Budka H, Hainfellner JA (2004) Value and limits of immunohistochemistry in differential diagnosis of clear cell primary brain tumors. Acta Neuropathol 108(1):24–30PubMedCrossRefGoogle Scholar
  66. Kukreia S, Ambekar S, Sin AH, Nanda A (2014) Cumulative survival analysis of patients with spinal myxopapillary ependymomas in the first 2 decades of life. J Neurosurg Pediatr 13(4):400–407CrossRefGoogle Scholar
  67. Lamszus K, Lachenmayer L, Heinemann U, Kluwe L, Finckh U, Hoppner W, Stavrou D, Fillbrandt R, Westphal M (2001) Molecular genetic alterations on chromosomes 11 and 22 in ependymomas. Int J Cancer 91:803–808PubMedCrossRefGoogle Scholar
  68. Lamzabi I, Arvanitis LD, Reddy VB, Bitterman P, Gattuso P (2013) Immunophenotype of myxopapillary ependymomas. Appl Immunohistochem Mol Morphol 21(6):485–489PubMedCrossRefGoogle Scholar
  69. Lee M, Rezai AR, Freed D, Epstein FJ (1996) Intramedullary spinal cord tumors in neurofibromatosis. Neurosurgery 38:32–37PubMedCrossRefGoogle Scholar
  70. Li AM, Dunham C, Tabori U, Carret AS, McNeely PD, Johnston D, Laray-Cousin L, Wilson B, Eisenstat DD, Jabado N, Zelcer S, Silva M, Scheinemann K, Fryer C, Hendson G, Fotovati A, Hawkins C, Yip S, Dunn SE, Hukin J (2015) EZH2 expression is a prognostic factor in childhood intracranial ependymoma: a Canadian pediatric brain tumor consortium study. Cancer 121(9):1499–1507PubMedCrossRefGoogle Scholar
  71. Linet MS, Gridley G, Cnattingius S, Nicholson HS, Martinsson U, Glimelius B, Adami HO, Zack M (1996) Maternal and perinatal risk factors for childhood brain tumors (Sweden). Cancer Causes Control 7(4):437–448Google Scholar
  72. Linet MS, Ries LA, Smith MA, Tarone RE, Devesa SS (1999) Cancer surveillance series: recent trends in childhood cancer incidence and mortality in the United States. J Natl Cancer Inst 91:1051–1058PubMedCrossRefGoogle Scholar
  73. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) (2007) WHO classification of tumors of the central nervous system. International Agency for Research on Cancer, LyonGoogle Scholar
  74. Mack SC, Taylor MD (2009) The genetic and epigenetic basis of ependymoma. Childs Nerv Syst 25(10):1195–1201PubMedCrossRefGoogle Scholar
  75. Mack SC, Witt H, Piro RM, Gu L, Zuyderduyn S, Stütz AM et al (2014) Epigenomic alterations define lethal CIMP-positive ependymomas of infancy. Nature 506(7489):445–450PubMedPubMedCentralCrossRefGoogle Scholar
  76. Martini F, Iaccheri L, Lazzarin L, Carinci P, Corallini A, Gerosa M, Iuzzolino P, Barbanti-Brodano G, Tognon M (1996) SV40 early region and large T antigen in human brain tumors, peripheral blood cells, and sperm fluids from healthy individuals. Cancer Res 56:4820–4825PubMedGoogle Scholar
  77. 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 Neurooncol 37:135–143PubMedCrossRefGoogle Scholar
  78. Massimino M, Solero CL, Garre ML, Biassoni V, Cama A, Genitori L et al. (2011) Second-look surgery for ependymoma: the Italian experience. J Neurosurg Pediatr 8;3:246–250Google Scholar
  79. 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 12:2070–2079PubMedCrossRefGoogle Scholar
  80. Merchant TE, Thompson S, Williams T (2000) Preliminary results from a phase II trial of conformal radiation therapy for localized pediatric brain tumors. In: Annual Meeting of the American Society of Therapeutic Radiology and Oncology, BostonGoogle Scholar
  81. Merchant TE, Mulhern RK, Krasin MJ, Kun LE, Williams T, Li C, Xiong X, Khan RB, Lustig RH, Boop FA, Sanford RA (2004) 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 22:3156–3162PubMedCrossRefGoogle Scholar
  82. 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 10:258–266PubMedPubMedCentralCrossRefGoogle Scholar
  83. Modena P, Lualdi E, Facchinetti F, Veltman J, Reid JF, Minardi S, Janssen I, Giangaspero F, Forni M, Finocchiaro G, Genitori L, Giordano F, Riccardi R, Schoenmakers EF, Massimino M, Sozzi G (2006) Identification of tumor-specific molecular signatures in intracranial ependymoma and association with clinical characteristics. J Clin Oncol 24:5223–5233PubMedCrossRefGoogle Scholar
  84. Mork SJ, Loken AC (1977) Ependymoma: a follow-up study of 101 cases. Cancer 40:907–915PubMedCrossRefGoogle Scholar
  85. Nadkarni TD, Rekate HL (1999) Pediatric intramedullary spinal cord tumors. Critical review of the literature. Childs Nerv Syst 15:17–28PubMedCrossRefGoogle Scholar
  86. Nazar GB, Hoffman HJ, Becker LE, Jenkin D, Humphreys RP, Hendrick EB (1990) Infratentorial ependymomas in childhood: prognostic factors and treatment. J Neurosurg 72:408–417PubMedCrossRefGoogle Scholar
  87. Newton HB, Henson J, Walker RW (1992) Extraneural metastases in ependymoma. J Neurooncol 14:135–142PubMedCrossRefGoogle Scholar
  88. Ohgaki H, Eibl RH, Wiestler OD, Yasargil MG, Newcomb EW, Kleihues P (1991) p53 mutations in nonastrocytic human brain tumors. Cancer Res 51(22):6202–6205PubMedGoogle Scholar
  89. Osterdock R, Sanford RA, Merchant TE (2000) Pediatric ependymoma. AANS/CNS Section on Pediatric Neurological Surgery, CoronadoGoogle Scholar
  90. Pachella LA, Kamiya-Matsuoka C, Lee EL, Olar A, Yung WK (2015) Supratentorial extraventricular anaplastic ependymoma with extracranial metastasis. J Clin Neurosci 22(3):605–607PubMedCrossRefGoogle Scholar
  91. Perez-Bovet J, Rimbau-Munoz J, Martin-Ferrer S (2013) Anaplastic ependymoma with holocordal and intracranial meningeal carcinomatosis and holospinal bone metastases. Neurosurgery 72(3):E497–E503PubMedCrossRefGoogle Scholar
  92. 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 29:79–85PubMedCrossRefGoogle Scholar
  93. Pezzolo A, Capra V, Raso A, Morandi F, Parodi F, Gambini C et al (2008) Identification of novel chromosomal abnormalities and prognostic cytogenetics markers in intracranial pediatric ependymoma. Cancer Lett 261(2):235–243PubMedCrossRefGoogle Scholar
  94. Prayson RA, Suh JH (1999) Subependymomas: clinicopathologic study of 14 tumors, including comparative MIB-1 immunohistochemical analysis with other ependymal neoplasms. Arch Pathol Lab Med/Arch Pathol Lab Med 123:306–309PubMedGoogle Scholar
  95. Ries LA, Smith MA, Gurney JG, Linet M, Tamra T, Young JL, Bunin GR (eds) (1999) Cancer incidence and survival among children and adolescents: United States SEER Program 1975–1995, National Cancer Institute, SEER Program (NIH Pub. No. 99-4649). NIH, BethesdaGoogle Scholar
  96. 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 88:695–703PubMedCrossRefGoogle Scholar
  97. Rogers HA, Kilday JP, Mayne C, Ward J, Adamowicz-Brice M, Schwalbe EC et al (2011) Supratentorial and spinal pediatric ependymomas display a hypermethylated phenotype which includes the loss of tumor suppressor genes involved in the control of cell growth and death. Acta Neuropathol 123(5):711–725PubMedPubMedCentralCrossRefGoogle Scholar
  98. Ross GW, Rubinstein LJ (1989) Lack of histopathological correlation of malignant ependymomas with postoperative survival. J Neurosurg 70:31–36PubMedCrossRefGoogle Scholar
  99. 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 28:381–386PubMedCrossRefGoogle Scholar
  100. Safaee M, Oh MC, Mummaneni PV, Weinstein PR, Ames CP, Chou D, Berger MS, Parsa AT, Gupta N (2014) Surgical outcomes in spinal cord ependymomas and the importance of extent of resection in children and young adults. J Neurosurg Pediatr 13(4):393–399PubMedCrossRefGoogle Scholar
  101. Salazar OM, Rubin P, Bassano D, Marcial VA (1975) Improved survival of patients with intracranial ependymomas by irradiation: dose selection and field extension. Cancer 35:1563–1573PubMedCrossRefGoogle Scholar
  102. Sara A, Bruner JM, Mackay B (1994) Ultrastructure of ependymoma. Ultrastruct Pathol 18:33–42PubMedCrossRefGoogle Scholar
  103. Sato K, Schäuble B, Kleihues P, Ohgaki H (1996) Infrequent alterations of the p15, p16, CDK4 and CYCLIN D1 genes in non‐astrocytic human brain tumors. Int J Cancer 66(3):305–308PubMedCrossRefGoogle Scholar
  104. Schiffer D, Chio A, Giordana MT, Migheli A, Palma L, Pollo B, Soffietti R, Tribolo A (1991) Histologic prognostic factors in ependymoma. Childs Nerv Syst 7:177–182PubMedCrossRefGoogle Scholar
  105. Shaw EG, Evans RG, Scheithauer BW, Ilstrup DM, Earle JD (1987) Postoperative radiotherapy of intracranial ependymoma in pediatric and adult patients. Int J Radiat Oncol Biol Phys 13:1457–1462PubMedCrossRefGoogle Scholar
  106. Shih CS, Hale GA, Gronewold L, Tong X, Laningham FH, Gilger EA, Srivastava DK, Kun LE, Gajjar A, Fouladi M (2008) High-dose chemotherapy with autologous stem cell rescue for children with recurrent malignant brain tumors. Cancer 112:1345–1353PubMedCrossRefGoogle Scholar
  107. Shu HK, Sall WF, Maity A, Tochner ZA, Janss AJ, Belasco JB, Rorke-Adams LB, Phillips PC, Sutton LN, Fisher MJ (2007) Childhood intracranial ependymoma: twenty-year experience from a single institution. Cancer 110:432–441PubMedCrossRefGoogle Scholar
  108. Slasky BS, Bydder GM, Niendorf HP, Young IR (1987) MR imaging with gadolinium-DTPA in the differentiation of tumor, syrinx, and cyst of the spinal cord. J Comput Assist Tomogr 11:845–850PubMedCrossRefGoogle Scholar
  109. Smyth MD, Pitts L, Jackler RK, Aldape KD (2000) Metastatic spinal ependymoma presenting as a vestibular schwannoma. Case illustration. J Neurosurg 92:247PubMedGoogle Scholar
  110. Strickler HD, Rosenberg PS, Devesa SS, Hertel J, Fraumeni JF Jr, Goedert JJ (1998) Contamination of poliovirus vaccines with simian virus 40 (1955–1963) and subsequent cancer rates. JAMA 279:292–295PubMedCrossRefGoogle Scholar
  111. Tarapore PE, Modera P, Naujokas A, Oh MC, Amin B, Tihan T, Parsa AT, Ames CP, Chou D, Mummaneni PV, Weinstein PR (2013) Pathology of spinal ependymomas: an institutional experience over 25 years in 134 patients. Neurosurgery 73(2):247–255PubMedCrossRefGoogle Scholar
  112. 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 glial cells are candidate stem cells of ependymoma. Cancer Cell 8:323–335PubMedCrossRefGoogle Scholar
  113. Thorarinsdottir HK, Rood B, Kamani N, Lafond D, Perez-Albuerne E, Loechelt B, Packer RJ, MacDonald TJ (2007) Outcome for children <4 years of age with malignant central nervous system tumors treated with high-dose chemotherapy and autologous stem cell rescue. Pediatr Blood Cancer 48:278–284PubMedCrossRefGoogle Scholar
  114. Tihan T, Zhou T, Holmes E, Burger PC, Ozuysal S, Rushing EJ (2008) The prognostic value of histological grading of posterior fossa ependymomas in children: a Children’s Oncology Group study and a review of prognostic factors. Mod Pathol 21:165–177PubMedGoogle Scholar
  115. Timmermann B, Kortmann RD, Kuhl J, Meisner C, Slavc I, Pietsch T, Bamberg M (2000) Combined postoperative irradiation and chemotherapy for anaplastic ependymomas in childhood: results of the German prospective trials HIT 88/89 and HIT 91. Int J Radiat Oncol Biol Phys 46:287–295PubMedCrossRefGoogle Scholar
  116. Tortori-Donati P, Fondelli MP, Cama A, Garre ML, Rossi A, Andreussi L (1995) Ependymomas of the posterior cranial fossa: CT and MRI findings. Neuroradiology 37:238–243PubMedCrossRefGoogle Scholar
  117. Tsai CJ, Wang Y, Allen PK, Mahajan A, McCutcheon IE, Rao G, Rhines LD, Tatsui CE, Armstrong TS, Maor MH, Chang EL, Brown PD, Li J (2014) Outcomes after surgery and radiotherapy for spinal myxopapillary ependymoma: update of the MD Anderson Cancer Center experience. Neurosurgery 75(3):204–214CrossRefGoogle Scholar
  118. Vanuytsel L, Brada M (1991) The role of prophylactic spinal irradiation in localized intracranial ependymoma. Int J Radiat Oncol Biol Phys 21:825–830PubMedCrossRefGoogle Scholar
  119. Vege KD, Giannini C, Scheithauer BW (2000) The immunophenotype of ependymomas. Appl Immunohistochem Mol Morphol 8(1):25–31PubMedGoogle Scholar
  120. Venkatramani R, Ji L, Lasky J, Haley K, Judkins A, Zhou S, Sposto R, Olshefski R, Garvin J, Tekautz T, Kennedy G, Rassekh SR, Moore T, Gardner S, Allen J, Shore R, Moertel C, Atlas M, Dhall G, Finlay J (2013) Outcome of infants and young children with newly diagnosed ependymoma treated on the “Head Start” III prospective clinical trial. J Neurooncol 113(2):285–291PubMedPubMedCentralCrossRefGoogle Scholar
  121. Villano JL, Parker CK, Dolecek TA (2013) Descriptive epidemiology of ependymal tumours in the United States. Br J Cancer 108(11):2367–2371PubMedPubMedCentralCrossRefGoogle Scholar
  122. Wager M, Lapierre F, Blanc JL, Listrat A, Bataille B (2000) Cauda equina tumors: a French multicenter retrospective review of 231 adult cases and review of the literature. Neurosurg Rev 23:119–129; discussion 130–131PubMedCrossRefGoogle Scholar
  123. Waha A, Koch A, Hartmann W, Mack H, Schramm J, Sörensen N et al (2004) Analysis of HIC-1 methylation and transcription in human ependymomas. Int J Cancer 110(4):542–549PubMedCrossRefGoogle Scholar
  124. Waldron JN, Laperriere NJ, Jaakkimainen L, Simpson WJ, Payne D, Milosevic M, Wong CS (1993) Spinal cord ependymomas: a retrospective analysis of 59 cases. Int J Radiat Oncol Biol Phys 27:223–229PubMedCrossRefGoogle Scholar
  125. Walker RW, Allen JC (1988) Cisplatin in the treatment of recurrent childhood primary brain tumors. J Clin Oncol 6:62–66PubMedGoogle Scholar
  126. Weber DC, Wang Y, Miller R, Villa S, Zaucha R, Pica A, Poortmans P, Anacak Y, Ozygit G, Baumert B, Haller G, Preusser M, Li J (2015) Long-term outcome of patients with spinal myxopapillary ependymoma: treatment results from the MD Anderson Cancer Centre and institutions from the Rare Cancer Network. Neuro Oncol 17(4):588–595PubMedCrossRefGoogle Scholar
  127. Whitaker SJ, Bessell EM, Ashley SE, Bloom HJ, Bell BA, Brada M (1991) Postoperative radiotherapy in the management of spinal cord ependymoma. J Neurosurg 74:720–728PubMedCrossRefGoogle Scholar
  128. White L, Kellie S, Gray E, Toogood I, Waters K, Lockwood L, Macfarlane S, Johnston H (1998) Postoperative chemotherapy in children less than 4 years of age with malignant brain tumors: promising initial response to a VETOPEC-based regimen. A study of the Australian and New Zealand Children’s Cancer Study Group (ANZCCSG). J Pediatr Hematol Oncol 20:125–130PubMedCrossRefGoogle Scholar
  129. Witt H, Mack SC, Ryzhova M, Bender S, Sill M, Isserlin R et al (2011) Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma. Cancer Cell 20(2):143–157PubMedPubMedCentralCrossRefGoogle Scholar
  130. Woesler B, Moskopp D, Kuchelmeister K, Schul C, Wassmann H (1998) Intracranial metastasis of a spinal myxopapillary ependymoma. A case report. Neurosurg Rev 21:62–65PubMedCrossRefGoogle Scholar
  131. Zacharoulis S, Levy A, Chi SN, Gardner S, Rosenblum M, Miller DC, Dunkel I, Diez B, Sposto R, Ji L, Asgharzadeh S, Hukin J, Belasco J, Dubowy R, Kellie S, Termuhlen A, Finlay J (2007) Outcome for young children newly diagnosed with ependymoma, treated with intensive induction chemotherapy followed by myeloablative chemotherapy and autologous stem cell rescue. Pediatr Blood Cancer 49:34–40PubMedCrossRefGoogle Scholar
  132. Zemmoura I, Vourch P, Paubel A, Parfait B, Cohen J, Bilan F, Kitzis J, Rousselot C, Parker J, Andres CR (2014) A deletion causing exon NF2 exon 9 skipping is associated with familial autosomal dominant intramedullary ependymoma. Neuro-Oncol 16:250–255PubMedCrossRefGoogle Scholar

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© Springer International Publishing 2017

Authors and Affiliations

  • Cassie Kline
    • 1
    Email author
  • Craig Forester
    • 1
  • Anuradha Banerjee
    • 1
  1. 1.Division of Hematology/Oncology, Departments of PediatricsUniversity of California San Francisco & UCSF Benioff Children’s HospitalSan FranciscoUSA

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