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Medulloblastomas

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Primary Central Nervous System Tumors

Part of the book series: Current Clinical Oncology ((CCO))

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Abstract

Medulloblastoma, a highly cellular malignant embryonal neoplasm classified as a primitive neuroectodermal tumor (PNET) [1], is the most common malignant brain tumor in children, accounting for 15–25% of all childhood primary central nervous system (CNS) neoplasms [2]. By definition, medulloblastoma arises in the posterior fossa, usually from the cerebellar vermis in the roof of the fourth ventricle (Fig. 18.1). Medulloblastomas have a marked tendency to spread by seeding along the cerebrospinal fluid (CSF) pathways; evidence of this metastatic process is found in up to 35% of cases at diagnosis

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References

  1. Kleihues P, Burger PC, Scheithauer BW. Histological typing of tumors of the central nervous system. WHO International Histological Classification of Tumors. 2nd ed. New York, Tokyo: Springer; 1993.

    Google Scholar 

  2. Peris-Bonet R, Martinez-Garcia C, Lacour B et al. Childhood central nervous system tumours – incidence and survival in Europe (1978–1997): report from Automated Childhood Cancer Information System Project. Eur J Cancer. 2006;42:2064–80.

    Article  PubMed  Google Scholar 

  3. Parkin DM, Whelan SL, Ferlay J, Teppo L, Thomas DB. Cancer incidence in five continents. Vol. VIII. Lyon: International Agency for Research on Cancer; 2002. IARC Scient. Publ. No. 155.

    Google Scholar 

  4. Verdecchia A, Francisci S, Brenner H et al. Recent cancer survival in Europe: a 2000–02 period analysis of EUROCARE-4 data. Lancet Oncol. 2007;8:784–96.

    Article  PubMed  Google Scholar 

  5. Stewart BW, Kleihus P, editors. World cancer report. Lyon: IARC Press. 2003.

    Google Scholar 

  6. Shore RE, Moseson M, Harley N, Pasternack BS. Tumors and other diseases following childhood X-ray treatment for ringworm of the scalp (Tinea capitis). Health Phys. 2003;85:404–8.

    Article  CAS  PubMed  Google Scholar 

  7. Croul S, Otte J, Khalili K. Brain tumors and polyomaviruses. J Neurovirol. 2003;9:173–82.

    CAS  PubMed  Google Scholar 

  8. Buhren J, Christoph AH, Buslei R et al. Expression of the neurotrophin receptor p75NTR in medulloblastomas is correlated with distinct histological and clinical features: evidence for a medulloblastoma subtype derived from the external granule cell layer. J Neuropathol Exp Neurol. 2000;59:229–40.

    CAS  PubMed  Google Scholar 

  9. Pietsch T, Waha A, Koch A et al. Medulloblastomas of the desmoplastic variant carry mutations of the human homologue of Drosophila patched. Cancer Res. 1997;57:2085–8.

    CAS  PubMed  Google Scholar 

  10. Sarkar C, Pramanik P, Karak AK et al. Are childhood and adult medulloblastomas different? A comparative study of clinicopathological features, proliferation index and apoptotic index. J Neurooncol. 2002;59:49–61.

    Article  PubMed  Google Scholar 

  11. Herms J, Neidt I, Luscher B et al. C-MYC expression in medulloblastoma and its prognostic value. Int J Cancer. 2000;%20 89:395–402.

    Article  CAS  PubMed  Google Scholar 

  12. Grotzer MA, Hogarty MD, Janss AJ et al. MYC messenger RNA expression predicts survival outcome in childhood primitive neuroectodermal tumor/medulloblastoma. Clin Cancer Res. 2001;7:2425–33.

    CAS  PubMed  Google Scholar 

  13. Batra SK, McLendon RE, Koo JS et al. Prognostic implications of chromosome 17p deletions in human medulloblastomas. J Neurooncol. 1995;24:39–45.

    Article  CAS  PubMed  Google Scholar 

  14. Biegel JA, Janss AJ, Raffel C et al. Prognostic significance of chromosome 17p deletions in childhood primitive neuroectodermal tumors (medulloblastomas) of the central nervous system. Clin Cancer Res. 1997;3:473–8.

    CAS  PubMed  Google Scholar 

  15. Cogen PH, McDonald JD. Tumor suppressor genes and medulloblastoma. J Neurooncol. 1996;29:103–12.

    Article  CAS  PubMed  Google Scholar 

  16. Brandes AA, Ermani M, Amista P et al. The treatment of adults with medulloblastoma: a prospective study. Int J Radiat Oncol Biol Phys. 2003;57:755–61.

    Article  PubMed  Google Scholar 

  17. Schofield D, West DC, Anthony DC, Marshal R, Sklar J. Correlation of loss of heterozygosity at chromosome 9q with histological subtype in medulloblastomas. Am J Pathol. 1995;146:472–80.

    CAS  PubMed  Google Scholar 

  18. Rudin CM, Hann CL, Laterra J et al. Treatment of medulloblastoma with hedgehog pathway inhibitor GDC-0449. N Engl J Med. 2009;361:1173–8.

    Article  CAS  PubMed  Google Scholar 

  19. Frost PJ, Laperriere NJ, Wong CS et al. Medulloblastoma in adults. Int J Radiat Oncol Biol Phys. 1995;32:951–7.

    Article  CAS  PubMed  Google Scholar 

  20. Carrie C, Lasset C, Alapetite C et al. Multivariate analysis of prognostic factors in adult patients with medulloblastoma. Retrospective study of 156 patients. Cancer 1994;74:2352–60.

    Article  CAS  PubMed  Google Scholar 

  21. Chan AW, Tarbell NJ, Black PM et al. Adult medulloblastoma: prognostic factors and patterns of relapse. Neurosurgery 2000;47:623–31.

    Article  CAS  PubMed  Google Scholar 

  22. Rochkind S, Blatt I, Sadeh M, Goldhammer Y. Extracranial metastases of medulloblastoma in adults: literature review. J Neurol Neurosurg Psychiatry. 1991;54:80–6.

    Article  CAS  PubMed  Google Scholar 

  23. Ray A, Ho M, Ma J et al. A clinicobiological model predicting survival in medulloblastoma. Clin Cancer Res. 2004;10:7613–20.

    Article  CAS  PubMed  Google Scholar 

  24. Pomeroy SL, Tamayo P, Gaasenbeek M et al. Prediction of central nervous system embryonal tumour outcome based on gene expression. Nature 2002;415:436–42.

    Article  CAS  PubMed  Google Scholar 

  25. Packer RJ, Rood BR, MacDonald TJ. Medulloblastoma: present concepts of stratification into risk groups. Pediatr Neurosurg. 2003;39:60–7.

    Article  PubMed  Google Scholar 

  26. Tabori U, Sung L, Hukin J et al. Distinctive clinical course and pattern of relapse in adolescents with medulloblastoma. Int J Radiat Oncol Biol Phys. 2006;64:402–7.

    Article  PubMed  Google Scholar 

  27. Prados MD, Warnick RE, Wara WM et al. Medulloblastoma in adults. Int J Radiat Oncol Biol Phys. 1995;32:1145–52.

    Article  CAS  PubMed  Google Scholar 

  28. Brandes AA, Franceschi E, Tosoni A, Blatt V, Ermani M. Long-term results of a prospective study on the treatment of medulloblastoma in adults. Cancer 2007;110:2035–41.

    Article  PubMed  Google Scholar 

  29. Padovani L, Sunyach MP, Perol D et al. Common strategy for adult and pediatric medulloblastoma: a multicenter series of 253 adults. Int J Radiat Oncol Biol Phys. 2007;68:433–40.

    Article  PubMed  Google Scholar 

  30. Cohen BH, Packer RJ. Chemotherapy for medulloblastomas and primitive neuroectodermal tumors. J Neurooncol. 1996;29:55–68.

    Article  CAS  PubMed  Google Scholar 

  31. Timmermann B, Kortmann RD, Kuhl J et al. Role of radiotherapy in the treatment of supratentorial primitive neuroectodermal tumors in childhood: results of the prospective German brain tumor trials HIT 88/89 and 91. J Clin Oncol. 2002;20:842–9.

    Article  PubMed  Google Scholar 

  32. Halperin EC, Friedman HS, Schold SC Jr. et al. Surgery, hyperfractionated craniospinal irradiation, and adjuvant chemotherapy in the management of supratentorial embryonal neuroepithelial neoplasms in children. Surg Neurol. 1993;40:278–83.

    Article  CAS  PubMed  Google Scholar 

  33. Kortmann RD, Kuhl J, Timmermann B et al. Postoperative neoadjuvant chemotherapy before radiotherapy as compared to immediate radiotherapy followed by maintenance chemotherapy in the treatment of medulloblastoma in childhood: results of the German prospective randomized trial HIT’91. Int J Radiat Oncol Biol Phys. 2000;46:269–79.

    Article  CAS  PubMed  Google Scholar 

  34. Tomita T. Neurosurgical perspectives in pediatric neurooncology. Childs Nerv Syst. 1998;14:94–6.

    Article  CAS  PubMed  Google Scholar 

  35. Albright AL, Wisoff JH, Zeltzer PM et al. Effects of medulloblastoma resections on outcome in children: a report from the Children’s Cancer Group. Neurosurgery 1996;38:265–71.

    Article  CAS  PubMed  Google Scholar 

  36. Cushing H. Experiences with cerebellar astrocytoma. A critical review. Acta Pathol Microbiol Scand. 1930;7:1–86.

    Article  Google Scholar 

  37. Paterson E, Farr RF. Cerebellar medulloblastoma: treatment by radiation of the whole central nervous system. Acta Radiologica. 1953;39:323–36.

    Article  CAS  PubMed  Google Scholar 

  38. Hubbard JL, Scheithauer BW, Kispert DB et al. Adult cerebellar medulloblastomas: the pathological, radiographic, and clinical disease spectrum. J Neurosurg. 1989;70:536–44.

    Article  CAS  PubMed  Google Scholar 

  39. Ferrante L, Mastronardi L, Celli P et al. Medulloblastoma in adulthood. J Neurosurg Sci. 1991;35:23–30.

    CAS  PubMed  Google Scholar 

  40. Bloom HJ, Bessell EM. Medulloblastoma in adults: a review of 47 patients treated between 1952 and 1981. Int J Radiat Oncol Biol Phys. 1990;18:763–72.

    Article  CAS  PubMed  Google Scholar 

  41. Berry MP, Jenkin RD, Keen CW, Nair BD, Simpson WJ. Radiation treatment for medulloblastoma. A 21-year review. J Neurosurg. 1981;55:43–51.

    Article  CAS  PubMed  Google Scholar 

  42. Kortmann RD, Kuhl J, Timmermann B et al. [Current and future strategies in interdisciplinary treatment of medulloblastomas, supratentorial PNET (primitive neuroectodermal tumors) and intracranial germ cell tumors in childhood]. Strahlenther Onkol. 2001;177:447–61.

    Article  CAS  PubMed  Google Scholar 

  43. Hazuka MB, DeBiose DA, Henderson RH, Kinzie JJ. Survival results in adult patients treated for medulloblastoma. Cancer 1992;69:2143–8.

    Article  CAS  PubMed  Google Scholar 

  44. Abacioglu U, Uzel O, Sengoz M, Turkan S, Ober A. Medulloblastoma in adults: treatment results and prognostic factors. Int J Radiat Oncol Biol Phys. 2002;54:855–60.

    Article  PubMed  Google Scholar 

  45. Thomas PR, Deutsch M, Kepner JL et al. Low-stage medulloblastoma: final analysis of trial comparing standard-dose with reduced-dose neuraxis irradiation. J Clin Oncol. 2000;18:3004–11.

    CAS  PubMed  Google Scholar 

  46. Packer RJ, Goldwein J, Nicholson HS et al. Treatment of children with medulloblastomas with reduced-dose craniospinal radiation therapy and adjuvant chemotherapy: a Children’s Cancer Group Study. J Clin Oncol. 1999;17:2127–36.

    CAS  PubMed  Google Scholar 

  47. Packer RJ, Gajjar A, Vezina G et al. Phase III study of craniospinal radiation therapy followed by adjuvant chemotherapy for newly diagnosed average-risk medulloblastoma. J Clin Oncol. 2006;24:4202–8.

    Article  CAS  PubMed  Google Scholar 

  48. Carrie C, Muracciole X, Gomez F et al. Conformal radiotherapy, reduced boost volume, hyperfractionated radiotherapy, and online quality control in standard-risk medulloblastoma without chemotherapy: results of the French M-SFOP 98 protocol. Int J Radiat Oncol Biol Phys. 2005;63:711–6.

    Article  PubMed  Google Scholar 

  49. Tabori U, Sung L, Hukin J et al. Medulloblastoma in the second decade of life: a specific group with respect to toxicity and management: a Canadian Pediatric Brain Tumor Consortium Study. Cancer 2005;103:1874–80.

    Article  PubMed  Google Scholar 

  50. Greenberg HS, Chamberlain MC, Glantz MJ, Wang S. Adult medulloblastoma: multiagent chemotherapy. Neuro-oncol 2001;3:29–34.

    CAS  PubMed  Google Scholar 

  51. Grabenbauer GG, Beck JD, Erhardt J et al. Postoperative radiotherapy of medulloblastoma. Impact of radiation quality on treatment outcome. Am J Clin Oncol. 1996;19:73–7.

    Article  CAS  PubMed  Google Scholar 

  52. Miralbell R, Bleher A, Huguenin P et al. Pediatric medulloblastoma: radiation treatment technique and patterns of failure. Int J Radiat Oncol Biol Phys. 1997;37:523–9.

    Article  CAS  PubMed  Google Scholar 

  53. Carrie C, Hoffstetter S, Gomez F et al. Impact of targeting deviations on outcome in medulloblastoma: study of the French Society of Pediatric Oncology (SFOP). Int J Radiat Oncol Biol Phys. 1999;45:435–9.

    CAS  PubMed  Google Scholar 

  54. Kortmann RD, Timmermann B, Kuhl J et al. HIT’91 (prospective, co-operative study for the treatment of malignant brain tumors in childhood): accuracy and acute toxicity of the irradiation of the craniospinal axis. Results of the quality assurance program. Strahlenther Onkol. 1999;175:162–9.

    Article  CAS  PubMed  Google Scholar 

  55. Taylor RE, Bailey CC, Robinson K et al. Results of a randomized study of preradiation chemotherapy versus radiotherapy alone for nonmetastatic medulloblastoma: The International Society of Paediatric Oncology/United Kingdom Children’s Cancer Study Group PNET-3 Study. J Clin Oncol. 2003;21:1581–91.

    Article  CAS  PubMed  Google Scholar 

  56. Chang CH, Housepian EM, Herbert C Jr. An operative staging system and a megavoltage radiotherapeutic technic for cerebellar medulloblastomas. Radiology 1969;93:1351–9.

    CAS  PubMed  Google Scholar 

  57. Evans AE, Jenkin RD, Sposto R et al. The treatment of medulloblastoma. Results of a prospective randomized trial of radiation therapy with and without CCNU, vincristine, and prednisone. J Neurosurg. 1990;72:572–82.

    Article  CAS  PubMed  Google Scholar 

  58. Corn BW, Yousem DM, Scott CB et al. White matter changes are correlated significantly with radiation dose. Observations from a randomized dose-escalation trial for malignant glioma (Radiation Therapy Oncology Group 83-02). Cancer 1994;74:2828–35.

    Article  CAS  PubMed  Google Scholar 

  59. Crossen JR, Garwood D, Glatstein E, Neuwelt EA. Neurobehavioral sequelae of cranial irradiation in adults: a review of radiation-induced encephalopathy. J Clin Oncol. 1994;12:627–42.

    CAS  PubMed  Google Scholar 

  60. Kumar AJ, Leeds NE, Fuller GN et al. Malignant gliomas: MR imaging spectrum of radiation therapy- and chemotherapy-induced necrosis of the brain after treatment. Radiology 2000;217:377–84.

    CAS  PubMed  Google Scholar 

  61. Klein M, Heimans JJ, Aaronson NK et al. Effect of radiotherapy and other treatment-related factors on mid-term to long-term cognitive sequelae in low-grade gliomas: a comparative study. Lancet 2002;360:1361–8.

    Article  CAS  PubMed  Google Scholar 

  62. Gregor A, Cull A, Traynor E et al. Neuropsychometric evaluation of long-term survivors of adult brain tumours: relationship with tumour and treatment parameters. Radiother Oncol. 1996;41:55–9.

    CAS  PubMed  Google Scholar 

  63. Brandes AA, Pasetto LM, Lumachi F, Monfardini S. Endocrine dysfunctions in patients treated for brain tumors: incidence and guidelines for management. J Neurooncol. 2000;47:85–92.

    Article  CAS  PubMed  Google Scholar 

  64. MacDonald TJ, Brown KM, LaFleur B et al. Expression profiling of medulloblastoma: PDGFRA and the RAS/MAPK pathway as therapeutic targets for metastatic disease. Nat Genet. 2001;29:143–52.

    Article  CAS  PubMed  Google Scholar 

  65. Packer RJ, Sutton LN, Goldwein JW, Perilongo G, Bunin G, Ryan J, et al. Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. J Neurosurg 1991;74:433–440.

    Article  CAS  PubMed  Google Scholar 

  66. Farwell JR, Flannery JT. Adult occurrence of medulloblastoma. Acta Neurochir (Wien) 1987;86:1–5.

    Article  CAS  Google Scholar 

  67. Peterson K, Walker RW. Medulloblastoma/primitive neuroectodermal tumor in 45 adults. Neurology 1995;45:440–442.

    CAS  PubMed  Google Scholar 

  68. Giordana MT, Cavalla P, Chio A, Marino S, Soffietti R, Vigliani MC, et al. Prognostic factors in adult medulloblastoma. A clinico-pathologic study. Tumori 1995;81:338–346.

    CAS  PubMed  Google Scholar 

  69. Giordana MT, Schiffer P, Lanotte M, Girardi P, Chio A. Epidemiology of adult medulloblastoma. Int J Cancer 1999;80:689–692.

    Article  CAS  PubMed  Google Scholar 

  70. Coulbois S, Civit T, Grignon Y, Taillandier L, Girard F, Marchal C, et al. [Adult medulloblastoma. Review of 22 patients]. Neurochirurgie 2001;47:6–12.

    CAS  PubMed  Google Scholar 

  71. Louis E, Chinot O, Honnorat J, Broet Ph, Delattre JY, Tailandier L. Which optimal treatment of adult medulloblastoma? The ANOCEF group experience: retrospective study of 72 cases. J Neurooncol 2002;4 Suppl 1:258.

    Google Scholar 

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Authors and Affiliations

  1. Department of Medical Oncology, Azienda USL-Bellaria-Maggiore Hospital Bologna, Bologna, Italy

    Alba A. Brandes & Enrico Franceschi MD

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  1. Alba A. Brandes
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  2. Enrico Franceschi MD
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Correspondence to Alba A. Brandes .

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Editors and Affiliations

  1. Dana-Farber Cancer Institute, Harvard Medical School, Binney St. 44, Boston, 02115, Massachusetts, USA

    Andrew D. Norden

  2. Departments of Surgery and Pediatrics, Duke University Medical Center, Durham, 27710, North Carolina, USA

    David A. Reardon

  3. Dana-Farber Cancer Institute, Harvard Medical School, Binney St. 44, Boston, 02115, Massachusetts, USA

    Patrick C. Y. Wen

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Brandes, A.A., Franceschi, E. (2011). Medulloblastomas. In: Norden, A., Reardon, D., Wen, P. (eds) Primary Central Nervous System Tumors. Current Clinical Oncology. Humana Press. https://doi.org/10.1007/978-1-60761-166-0_18

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