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Ependymoma

  • Chapter
Pediatric CNS Tumors

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

Abstract

Ependymomas are relatively rare gliomas arising from the differentiated ependymal cell layer lining the ventricular system and central canal of the spinal cord. Intracranial ependymomas account for approximately 9% of all brain tumors in the population under 20 years of age, and are the third most common primary brain tumor in children (following astrocytomas and primitive neuroectodermal tumors). According to SEER (Surveillance, Epidemiology and End Results) data from 1975 until 1998, the annual incidence of ependymoma is 2.6 per million for the 0 to 14 age group, and 2.2 per million for the 0 to 20 age group (Ries et al. 1999). Population-based measurements of the incidence of spinal cord ependymoma in children are available from the Connecticut Tumor Registry. Between 1935 and 1973, 5 spinal cord ependymomas and 44 intracranial ependymomas were identified in the Connecticut population under 20 years of age; suggesting that spinal cord ependymoma represents approximately 10% of all ependymal tumors in children and young adults (Dohrmann et al. 1976). Another large institutional series confirmed that spinal cord ependymomas are rare in children under 10 years of age, accounting for less than 1% of all spinal tumors. After age 10, the incidence of spinal cord ependymoma increases, and it represents the majority of intramedullary tumors in patients older than 20 years (Constantini et al. 1997).

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References

  1. Aggarwal R, Yeung D, Kumar P et al (1997) Efficacy and feasibility of stereotactic radiosurgery in the primary management of unfavorable pediatric ependymoma. Radiother Oncol 43:269–273

    Google Scholar 

  2. Awaad Y, Allen J, Miller D et al (1996) Deferring adjuvant therapy for totally resected intracranial ependymoma. Pediatr Neurol 14: 216–219

    Article  PubMed  CAS  Google Scholar 

  3. Bergsagel D, Finegold M, Butel J et al (1992) DNA sequences similar to those of Simian virus 4o in ependymomas and choroid plexus tumors of childhood. N Engl J Med 326: 988–993

    Article  PubMed  CAS  Google Scholar 

  4. Birch B, Johnson J, Parsa A et al (1996) Frequent type 2 neurofibromatosis gene transcript mutations in sporadic intramedullary spinal cord ependymomas. Neurosurgery 39: 135–140

    Article  PubMed  CAS  Google Scholar 

  5. Carbone M, Rizzo P, Pass H (1997) Simian virus 4o, poliovaccines and human tumors: a review of recent developments. Oncogene 15: 1877–1888

    Article  PubMed  CAS  Google Scholar 

  6. Cervoni L, Celli P, Fortuna A et al (1994) Recurrence of spinal ependymoma. Risk factors and long-term survival. Spine 19: 2838–2841

    Google Scholar 

  7. Ciraldo A, Platt M, Agamanolis D et al (1986) Sacrococcygeal myxopapillary ependymomas and ependymal rests in infants and children. J Pediatr Surg 21:49–52

    Google Scholar 

  8. Constantini S, Allen J, Epstein F (1997) Pediatric and adult primary spinal cord tumors. In: Black P, Loeffler J (eds) Cancer of the nervous system. Blackwell Science, Cambridge, pp 638–639

    Google Scholar 

  9. Coulon R, Till K (1977) Intracranial ependymomas in children. Child’s Brain 3x54–168

    Google Scholar 

  10. Debiec-Rychter M, Hagemeijer A, Sciot R (woo) Cytogenetic analysis in three cerebral subependymomas: further evidence for a hamartomatous nature? Cancer Genet Cytogenet 122: 63–64

    Google Scholar 

  11. Dohrmann G, Farwell J, Flannery J (1976) Ependymomas and ependymoblastomas in children. J Neurosurg 45: 273–281

    Article  PubMed  CAS  Google Scholar 

  12. Duffner P, Horowitz M, Krischer J 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

    Google Scholar 

  13. Furie D, Provenzale J (1995) Supratentorial ependymomas and subependymomas: CT and MR appearance. J Comput Assist Tomogr 19: 518–526

    Google Scholar 

  14. Garrett P, Simpson W (1983) Ependymomas: results of radia- tion treatment. Int J Radiat Oncol Biol Phys 9: 1121–1124

    Article  PubMed  CAS  Google Scholar 

  15. Gerston K, Suprun H, Cohen H et al (1985) Presacral myxopapillary ependymoma presenting as an abdominal mass in a child. J Pediatr Surg 20: 276–278

    Article  PubMed  CAS  Google Scholar 

  16. Geyer R, Zeltzer P, Boyett J et al (1994) Survival of infants with primitive neuroectodermal tumors or malignant ependymomas of the CNS treated with eight drugs in I day: a report from the Childrens Cancer Group. J Clin Oncol 12x607–1615

    Google Scholar 

  17. Gold E, Leviton A, Lopez R et al (1994) The role of family histo- ry in risk of childhood brain tumors. Cancer 73x302–1311

    Google Scholar 

  18. Goldwein J, Glauser T, Packer R et al (1990) Recurrent intracranial ependymomas in children. Cancer 66: 557–563

    Article  PubMed  CAS  Google Scholar 

  19. Goldwein J, Corn B, Finlay J et al (1991) Is craniospinal irradiation required to cure children with malignant (anaplastic) intracranial ependymomas? Cancer 67: 2766–2771

    Article  PubMed  CAS  Google Scholar 

  20. Good C, Wade A, Hayward R et al (2001) Surveillance neuroimaging in childhood intracranial ependymoma: how effective, how often, and for how long? J Neurosurg 94: 27–32

    Article  PubMed  CAS  Google Scholar 

  21. Gornet M, Buckner J, Marks R et al (1999) Chemotherapy for advanced CNS ependymoma. J Neurooncol 45: 61–67

    Google Scholar 

  22. Grill J, Kalifa C, Doz F et al (1996) A high-dose busulfanthiotepa combination followed by autologous bone marrow transplantation in childhood recurrent ependymoma. Pediatr Neurosurg 25: 7–12

    Article  PubMed  CAS  Google Scholar 

  23. Grill J, Renaux V, Bulteau C et al (1999) Long-term intellectual outcome in children with posterior fossa tumors according to radiation doses and volumes. Int J Radiat Oncol Biol Phys 45: 137–145

    Article  PubMed  CAS  Google Scholar 

  24. Grill J, Le Deley M, Gambarelli D et al (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: 1288–1296

    PubMed  CAS  Google Scholar 

  25. Hamilton R, Pollack I (1997) The molecular biology of ependymomas. Brain Pathol 7: 807–822

    Article  PubMed  CAS  Google Scholar 

  26. Hemminki K, Li X, Vaittinen Pet al (woo) Cancers in the first-degree relatives of children with brain tumours. Br J Cancer 83: 407–411

    Google Scholar 

  27. Hirose Y, Aldape K, Bollen A et al (2001) Chromosomal abnormalities subdivide ependymal tumors into clinically relevant groups. Am J Pathol 158: 1137–1143

    Article  PubMed  CAS  Google Scholar 

  28. Hodgson D, Goumnerova L, Loeffler J et al (2001) Radiosurgery in the management of pediatric brain tumors. Int J Radiat Oncol Biol Phys 50: 929–935

    Article  PubMed  CAS  Google Scholar 

  29. Horn B, Heidman R, Geyer R et al (1999) A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors. J Pediatr Hematol Oncol

    Google Scholar 

  30. -211

    Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  32. Hulsebos T, Oskam N, Bijleveld E et al (1999) Evidence for an ependymoma tumour suppressor gene in chromosome region 22pter-22q11.2. Br J Cancer 81: 1150–1154

    Google Scholar 

  33. Ilgren E, Stiller C, Hughes J et al (1984) Ependymomas: a clinical and pathologic study. I. Biologic features. Clin Neuropathol 3: 113–121

    Google Scholar 

  34. Jinnouchi T, Shibata S, Fukushima M et al (1989) Ultrastructure of blood capillary permeability in human brain tumors, part 7. Ependymoma. J Clin Electron Micros 22: 57–63

    Google Scholar 

  35. Kirschstein R, Gerger P (1962) Ependymomas produced after intracerebral inoculation of SV4o into newborn hamsters. Nature 195:299–300

    Google Scholar 

  36. Kleihues P, Cayenne WK (2000) Pathology and genetics of the nervous system. IARC Press, Lyon

    Google Scholar 

  37. Lamszus K, Lachenmayer L, Heinemann U (2001) Molecular genetic alterations on chromosomes n and 22 in ependymomas. Int J Cancer 91: 803–808

    Article  PubMed  CAS  Google Scholar 

  38. Lee M, Rezai A, Freed D et al (1996) Intramedullary spinal cord tumors in neurofibromatosis. Neurosurgery 38: 32–37

    Article  PubMed  CAS  Google Scholar 

  39. Martini F, Iaccheri L, Lazzarini L et al (1996) SV4o early region and large T antigen in human brain tumors, peripheral blood cells and sperm fluids from healthy individuals. Cancer Res 56: 4820–4825

    PubMed  CAS  Google Scholar 

  40. Mason W, Grovas A, Halpern S et al (1998a) Intensive chemotherapy and bone marrow rescue for young children with newly diagnosed malignant brain tumors. J Clin Oncol 16: 210–221

    PubMed  CAS  Google Scholar 

  41. Mason W, Goldman S, Yates A et al (1998b) Survival following intensive chemotherapy with bone marrow reconstitution for children with recurrent intracranial ependymoma. J Neurooncol 37:135–143

    Google Scholar 

  42. Merchant T, Thompson S, Williams T et al (2000) Preliminary results from a phase II trial of conformal radiation therapy for localized pediatric brain tumors. 2000 annual meeting of the American Society of Therapeutic Radiology and Oncology. Boston, MA, 22–26 Oct. Int J Radiol Oncol Biol Phys 48380–181

    Google Scholar 

  43. Mork S, Loken A (1977) Ependymoma: a follow-up study of 101 cases. Cancer 40: 907–915

    Article  PubMed  CAS  Google Scholar 

  44. Nadkarni T, Rekate H (1999) Pediatric intramedullary spinal cord tumors. Child’s Nery Syst 15: 17–28

    Google Scholar 

  45. Nazar G, Hoffman H, Becker L et al (1990) Infratentorial ependymomas in childhood: prognostic factors and treatment. J Neurosurg 72: 408–417

    Article  PubMed  CAS  Google Scholar 

  46. Newton H, Henson J, Walker R (1992) Extraneural metastases in ependymoma. J Neurooncol 14:135–142

    Google Scholar 

  47. Osterdock R, Sanford R, Merchant T (2000) Pediatric ependymoma (40 in 36 months). 2000 AANS/CNS Section on Pediatric Neurological Surgery Annual Meeting. Coronado, California, 6–9 Dec 2000

    Google Scholar 

  48. Perilongo G, Massimino M, Sotti G et al (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

    Google Scholar 

  49. Prayson R, Suh J (1999) Subependymomas: clinicopathologic study of 14 tumors, including comparative MIB-1 immunohistochemical analysis with other ependymal neoplasms. Arch Pathol Lab Med 123: 306–309

    PubMed  CAS  Google Scholar 

  50. Reardon D, Entrekin R, Sublett J et al (1999) Chromosome arm 6q loss is the most common recurrent autosomal alteration detected in primary pediatric ependymoma. Genes Chromosom Cancer 24:230–237

    Google Scholar 

  51. Reyes-Mugica M, Chou PM, Myint MM et al (1994) Ependymomas in children: histologic and DNA-flow cytometric study. Pediatr Pathol 14: 453–466

    Google Scholar 

  52. Ries L, Smith M, Gurney J et al (eds) (1999) Cancer incidence and survival among children and adolescents: United States SEER Program 1975–1995 National Cancer Institute, SEER Program. NIH Publ no 99–4649. Bethesda, MD

    Google Scholar 

  53. Robertson P, Zeltzer P, Boyett J et al (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  PubMed  CAS  Google Scholar 

  54. Ross G, Rubinstein L (1989) Lack of histological correlation of malignant ependymomas with postoperative survival. J Neurosurg 70: 31–36

    Article  PubMed  CAS  Google Scholar 

  55. Rousseau P, Habrand J, Serrazin D et al (1994) Treatment of intracranial ependymomas of children: review of a 15-year experience. Int J Radiat Oncol Biol Phys 28: 381–386

    Article  PubMed  CAS  Google Scholar 

  56. Salazar O, Rubin P, Bassano D et al (1975) Improved survival of patients with intracranial ependymoma by irradiation therapy. Dose escalation and field extension. Cancer 35:1563–1573

    Google Scholar 

  57. Sara A, Bruner J, Mackay B (1994) Ultrastructure of ependymoma. Ultrastruct Pathol 18: 33–42

    Article  PubMed  CAS  Google Scholar 

  58. Scheil S, Bruderlein S, Eicker M et al (2001) Low frequency of chromosomal imbalances in anaplastic ependymomas as detected by comparative genomic hybridization. Brain Pathol 11333–143

    Google Scholar 

  59. Schiffer D, Chio A, Giordana M et al (1991) Histologic prognos- tic factors in ependymoma. Child’s Nery Syst 7: 177–182

    CAS  Google Scholar 

  60. Shaw E, Evans R, Scheithauer B et al (1987) Postoperative radiotherapy of intracranial ependymoma in pediatric and adult patients. Int J Radiat Oncol Biol Phys 13:1457–1462

    Google Scholar 

  61. Slasky B, Bydder G, Niendrof H et al (1987) MR imaging with gadolinium-DTPA in the differentiation of tumor, syrinx and cyst of the spinal cord. J Compt Tomogr 11: 845–850

    Article  CAS  Google Scholar 

  62. Strickler H, Rosenberg P, Devesa S et al (1998) Contamination of poliovirus vaccines with Simian virus 40 (1955–1963) and subsequent cancer rates. JAMA 279: 292–295

    Article  PubMed  CAS  Google Scholar 

  63. Timmermann B, Kortmann R, Kuhl J et al (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: 287–295

    Article  PubMed  CAS  Google Scholar 

  64. Tortori-Donati P, Fondelli M, Cama A et al (1995) Ependymomas of the posterior cranial fossa: CT and MRI findings. Neuroradiology 37: 238–243

    Google Scholar 

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

    Google Scholar 

  66. Wager M, Lapierre F, Blanc J et al (2000) Cauda equina tumors: a French multicenter retrospective review of 231 adult cases and review of the literature. Neurosurg Rev 23: 119–129

    Article  PubMed  CAS  Google Scholar 

  67. Waldron J, Laperriere N, Jaakkimainen L et al (1993) Spinal cord ependymomas: a retrospective analysis of 59 cases. Int J Radiat Oncol Biol Phys 27: 223–229

    Google Scholar 

  68. Walker R, Allen J (1988) Cisplatin in the treatment of recurrent primary brain tumors. J Clin Oncol 6: 62–66

    PubMed  CAS  Google Scholar 

  69. Ward S, Harding B, Wilkins P et al (2001) Gain 1q and loss of 22 are the most common changes detected by comparative genomic hybridisation in pediatric ependymoma. Genes Chromosom Cancer 32: 59–66

    Article  PubMed  CAS  Google Scholar 

  70. White L, Kellie S, Gray E et al (1998) Postoperative chemotherapy in children less than 4 years of age with malignant brain tumors: promising initial response to a VETOPECbased regimen. J Pediatr Hematol Oncol 20x25–130

    Google Scholar 

  71. Whitaker S, Bessel E, Ashley Set al (1991) Postoperative radiotherapy in the management of spinal cord ependymoma. J Neurosurg 74: 720–728

    Article  PubMed  CAS  Google Scholar 

  72. Woesler B, Moskopp D, Kuchelmeister K et al (1998) Intracranial metastasis of a spinal myxopapillary ependymoma: Case report. Neurosurg Rev 21: 62–65

    Google Scholar 

  73. Zhen H, Zhang X, Bu X et al (1999) Expression of the Simian virus 40 large tumor antigen (Tag) and formation of Tag-p53 and Tag-pRb complexes in human brain tumors. Cancer 86: 2124–2132

    Google Scholar 

  74. Zheng P, Pang J, Hui A et al (2000) Comparative genomic hybridization detects losses of chromosomes 22 and 16 as the most common recurrent genetic alterations in primary ependymomas. Cancer Genet Cytogenet 122: 18–15

    Article  PubMed  CAS  Google Scholar 

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Authors
  1. B. N. Horn
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  2. M. Smyth
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Editors and Affiliations

  1. Division of Pediatric Neurosurgery, Departments of Neurological Surgery and Pediatrics, University of California, San Francisco, USA

    Nalin Gupta MD, PhD (Assistant Professor, Chief) (Assistant Professor, Chief)

  2. Division of Neuro-Oncology, Departments of Neurological Surgery and Pediatrics, University of California, San Francisco, USA

    Anuradha Banerjee MD, MPH (Assistant Professor) (Assistant Professor)

  3. Department of Radiation Oncology, University of California, San Francisco, USA

    Daphne Haas-Kogan MD (Associate Professor) (Associate Professor)

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© 2004 Springer-Verlag Berlin Heidelberg

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Horn, B.N., Smyth, M. (2004). Ependymoma. In: Gupta, N., Banerjee, A., Haas-Kogan, D. (eds) Pediatric CNS Tumors. Pediatric Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-09227-9_5

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  • DOI: https://doi.org/10.1007/978-3-662-09227-9_5

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-05554-6

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