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Medulloblastoma

  • Chapter
Oncology of CNS Tumors

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Abstract

After hematological malignancies, central nervous system (CNS) tumors comprise the most common cancers in children and are the most common solid malignancy in this age group. Of these, medulloblastoma is the most common malignant pediatric brain tumor, accounting for up to 25% of all tumors in this group [1]. Although medulloblastoma has been diagnosed in all age groups, including fetuses in utero and in octogenarians, the majority of cases present between 5 and 10 years of age with a peak incidence at 7 years of age [1, 2]. There does appear to be a slight gender predilection, with most case series reporting a 1.8:1 ratio in favor of males [1]. Most cases are sporadic, although several familial tumor syndromes have been associated with medulloblastoma, including Gorlin syndrome, Rubenstein—Taybi syndrome, Li—Fraumeni syndrome, ataxia-telangiectasia, Turcot syndrome, neurofibroma-tosis, and tuberous sclerosis [3]. The term medullo-blastoma dates back to 1925 when it was coined by Bailey and Cushing to describe distinct, midline, highly malignant cerebellar tumors [4]. They had postulated that medulloblastoma tumor cells resembled cells of the developing neural tube. Decades later, their hypothesis is still proving insightful [4, 5]. Today, medulloblastoma is classified by the WHO as a distinct embryonal tumor of the cerebellum [6].

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References

  1. Rutka JT. (1997) Medulloblastoma. Clin Neurosurg 44:571–585

    PubMed  CAS  Google Scholar 

  2. Mitchell D, Rojiani AM, Richards D, Yachnis AT, Powell SZ. (1995) Congenital CNS primitive neuroectodermal tumor: case report and review of the literature. Pediatr Pathol Lab Med 15(6):949–956

    Article  PubMed  CAS  Google Scholar 

  3. Kunscher LF, Lang FF. (2004). Medulloblastoma. In: Winn HR, Youmans JR (eds) Youmans neurological surgery, 5th ed. W.B. Saunders, Philadelphia, PA, pp. 1031–1042

    Google Scholar 

  4. Rutka JT, Hoffman HJ. (1996) Medulloblastoma: a historical perspective and overview. J Neurooncol 29(1):1–7

    Article  PubMed  CAS  Google Scholar 

  5. Wechsler-Reya R, Scott MP. (2001) The developmental biology of brain tumors. Annu Rev Neurosci 24:385–428

    Article  PubMed  CAS  Google Scholar 

  6. Kleihues P, Louis DN, Scheithauer BW, et al (2002) The WHO classification of tumors of the nervous system. J Neuropathol Exp Neurol 61(3):215–225; discussion 26–29

    PubMed  Google Scholar 

  7. Sutton LN, Phillips PC, Molloy PT. (1996) Surgical management of medulloblastoma. J Neurooncol 29(1):9–21

    Article  PubMed  CAS  Google Scholar 

  8. Park TS, Hoffman HJ, Hendrick EB, Humphreys RP, Becker LE. (1983) Medulloblastoma: clinical presentation and management. Experience at the Hospital for Sick Children, Toronto, 1950–1980. J Neurosurg 58(4):543–552

    Article  PubMed  CAS  Google Scholar 

  9. Takei H, Bhattacharjee MB, Rivera A, Dancer Y, Powell SZ. (2007) New immunohistochemical markers in the evaluation of central nervous system tumors: a review of 7 selected adult and pediatric brain tumors. Arch Pathol Lab Med 131(2):234–241

    PubMed  CAS  Google Scholar 

  10. Crawford JR, MacDonald TJ, Packer RJ. (2007) Medulloblastoma in childhood: new biological advances. Lancet Neurol 6(12):1073–1085

    Article  PubMed  CAS  Google Scholar 

  11. Sarkar C, Deb P, Sharma MC. (2005) Recent advances in embryonal tumors of the central nervous system. Childs Nerv Syst 21(4):272–293

    Article  PubMed  Google Scholar 

  12. Giangaspero F, Perilongo G, Fondelli MP, et al (1999) Medulloblastoma with extensive nodularity: a variant with favorable prognosis. J Neurosurg 91(6):971–977

    Article  PubMed  CAS  Google Scholar 

  13. Rossi A, Caracciolo V, Russo G, Reiss K, Giordano A. (2008) Medulloblastoma: from molecular pathology to therapy. Clin Cancer Res 14(4):971–976

    Article  PubMed  CAS  Google Scholar 

  14. Raffel C. (2004) Medulloblastoma: molecular genetics and animal models. Neoplasia 6(4):310–322

    Article  PubMed  Google Scholar 

  15. Packer RJ, Biegel JA, Blaney S, et al (2002) Atypical tera-toid/rhabdoid tumor of the central nervous system: report on workshop. J Pediatr Hematol Oncol 24(5):337–342

    Article  PubMed  Google Scholar 

  16. Reddy AT. (2005) Atypical teratoid/rhabdoid tumors of the central nervous system. J Neurooncol 75(3):309–313

    Article  PubMed  CAS  Google Scholar 

  17. Biegel JA, Zhou JY, Rorke LB, Stenstrom C, Wainwright LM, Fogelgren B. (1999) Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors. Cancer Res 59(1):74–79

    PubMed  CAS  Google Scholar 

  18. Warmuth-Metz M, Bison B, Dannemann-Stern E, Kortmann R, Rutkowski S, Pietsch T. (2008) CT and MR imaging in atypical teratoid/rhabdoid tumors of the central nervous system. Neuroradiology 50(5):447–452

    Article  PubMed  Google Scholar 

  19. Parmar H, Hawkins C, Bouffet E, Rutka J, Shroff M. (2006) Imaging findings in primary intracranial atypical teratoid/ rhabdoid tumors. Pediatr Radiol 36(2):126–132

    Article  PubMed  Google Scholar 

  20. Fogarty MP, Kessler JD, Wechsler-Reya RJ. (2005) Morphing into cancer: the role of developmental signaling pathways in brain tumor formation. J Neurobiol 64(4):458–475

    Article  PubMed  CAS  Google Scholar 

  21. Polkinghorn WR, Tarbell NJ. (2007) Medulloblastoma: tumorigenesis, current clinical paradigm, and efforts to improve risk stratification. Nat Clin Pract Oncol 4(5):295–304

    Article  PubMed  CAS  Google Scholar 

  22. Knoepfler PS, Kenney AM. (2006) Neural precursor cycling at sonic speed. N-Myc pedals, GSK-3 brakes. Cell cycle (Georgetown, Tex) 5(1):47–52

    Article  CAS  Google Scholar 

  23. Sarkar C, Deb P, Sharma MC. (2005) Recent advances in embryonal tumors of the central nervous system. Childs Nerv Syst 21(4):272–293

    Article  PubMed  Google Scholar 

  24. Herms J, Neidt I, Luscher B, et al (2000) C-MYC expression in medulloblastoma and its prognostic value. Int J Cancer 89(5):395–402

    Article  PubMed  CAS  Google Scholar 

  25. Bruggers CS, Tai KF, Murdock T, et al (1998) Expression of the c-Myc protein in childhood medulloblastoma. J Pediatr Hematol Oncol 20(1):18–25

    Article  PubMed  CAS  Google Scholar 

  26. Moriuchi S, Shimizu K, Miyao Y, Hayakawa T. (1996) An immunohistochemical analysis of medulloblastoma and PNET with emphasis on N-myc protein expression. Anticancer Res 16(5A):2687–2692

    PubMed  CAS  Google Scholar 

  27. Eberhart CG, Burger PC. (2003).Anaplasia and grading in medulloblastomas. Brain Pathol (Zurich, Switzerland) 13(3):376–385

    Article  Google Scholar 

  28. Evans DG, Farndon PA, Burnell LD, Gattamaneni HR, Birch JM. (1991) The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma. Br J Cancer 64(5):959–961

    Article  PubMed  CAS  Google Scholar 

  29. Taylor MD, Mainprize TG, Rutka JT. (2000) Molecular insight into medulloblastoma and central nervous system primitive neuroectodermal tumor biology from hereditary syndromes: a review. Neurosurgery 47(4):888–901

    Article  PubMed  CAS  Google Scholar 

  30. Taylor MD, Liu L, Raffel C, et al (2002) Mutations in SUFU predispose to medulloblastoma. Nat Genet 31(3):306–310

    Article  PubMed  CAS  Google Scholar 

  31. Adesina AM, Nalbantoglu J, Cavenee WK. (1994) p53 gene mutation and mdm2 gene amplification are uncommon in medulloblastoma. Cancer Res 54(21):5649–5651

    PubMed  CAS  Google Scholar 

  32. Hamilton SR, Liu B, Parsons RE, et al (1995) The molecular basis of Turcot's syndrome. New Engl J Med 332(13):839–847

    Article  PubMed  CAS  Google Scholar 

  33. Huang H, Mahler-Araujo BM, Sankila A, et al (2000) APC mutations in sporadic medulloblastomas. Am J Pathol 156(2):433–437

    Article  PubMed  CAS  Google Scholar 

  34. Mori T, Nagase H, Horii A, et al (1994) Germ-line and somatic mutations of the APC gene in patients with Turcot syndrome and analysis of APC mutations in brain tumors. Genes Chromosomes Cancer 9(3):168–172

    Article  PubMed  CAS  Google Scholar 

  35. Eberhart CG, Tihan T, Burger PC. (2000) Nuclear localization and mutation of beta-catenin in medulloblastomas. J Neuropathol Exp Neurol 59(4):333–337

    PubMed  CAS  Google Scholar 

  36. Zurawel RH, Chiappa SA, Allen C, Raffel C. (1998) Sporadic medulloblastomas contain oncogenic beta-catenin mutations. Cancer Res 58(5):896–899

    PubMed  CAS  Google Scholar 

  37. Rutka J, Hoffman HJ, Duncan JA. (1996). Astrocytomas of the posterior fossa. In: Cohen A (ed) Surgical disorders of the fourth ventricle. Blackwell Science, Cambridge, MA, pp. 189–208

    Google Scholar 

  38. Bognar L, Borgulya G, Benke P, Madarassy G. (2003) Analysis of CSF shunting procedure requirement in children with posterior fossa tumors. Childs Nerv Syst 19(5–6):332–336

    Article  PubMed  Google Scholar 

  39. Culley DJ, Berger MS, Shaw, D., Geyer, R. (1994) An analysis of factors determining the need for ventriculoperitoneal shunts after posterior fossa tumor surgery in children. Neurosurgery 34(3):402–407; discussion 7–8

    Article  PubMed  CAS  Google Scholar 

  40. Kumar V, Phipps K, Harkness W, Hayward RD. (1996) Ventriculo-peritoneal shunt requirement in children with posterior fossa tumors: an 11-year audit. Br J Neurosurg 10(5):467–470

    Article  PubMed  CAS  Google Scholar 

  41. Sainte-Rose C, Cinalli G, Roux FE, et al (2001) Management of hydrocephalus in pediatric patients with posterior fossa tumors: the role of endoscopic third ventriculostomy. J Neurosurg 95(5):791–797

    Article  PubMed  CAS  Google Scholar 

  42. Papo I, Caruselli G, Luongo A. (1982) External ventricular drainage in the management of posterior fossa tumors in children and adolescents. Neurosurgery 10(1):13–15

    Article  PubMed  CAS  Google Scholar 

  43. Ruggiero C, Cinalli G, Spennato P, et al (2004) Endoscopic third ventriculostomy in the treatment of hydrocephalus in posterior fossa tumors in children. Childs Nerv Syst 20(11–12):828–833

    Article  PubMed  Google Scholar 

  44. Rekate HL, Grubb RL, Aram DM, Hahn JF, Ratcheson RA. (1985).Muteness of cerebellar origin. Arch Neurol 42(7):697–698

    Article  PubMed  CAS  Google Scholar 

  45. Siffert J, Poussaint T Y, Goumnerova LC, et al (2000) Neurological dysfunction associated with postoperative cer-ebellar mutism. J Neurooncol 48(1):75–81

    Article  PubMed  CAS  Google Scholar 

  46. Pollack IF, Polinko P, Albright AL, Towbin R, Fitz C. (1995) Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children: incidence and pathophysiol-ogy. Neurosurgery 37(5):885–893

    Article  PubMed  CAS  Google Scholar 

  47. Doxey D, Bruce D, Sklar F, Swift D, Shapiro K. (1999) Posterior fossa syndrome: identifiable risk factors and irreversible complications. Pediatr Neurosurg 31(3):131–136

    Article  PubMed  CAS  Google Scholar 

  48. Gajjar A, Chintagumpala M, Ashley D, et al (2006) Risk-adapted craniospinal radiotherapy followed by high-dose chemotherapy and stem-cell rescue in children with newly diagnosed medulloblastoma (St Jude Medulloblastoma-96): long-term results from a prospective, multicentre trial. Lancet Oncol 7(10):813–820

    Article  PubMed  Google Scholar 

  49. Salaroli R, Di Tomaso T, Ronchi A, et al (2008) Radiobiologic response of medulloblastoma cell lines: involvement of beta-catenin? J Neurooncol 90(3):243–251

    Article  PubMed  CAS  Google Scholar 

  50. Mulhern RK, Merchant TE, Gajjar A, Reddick WE, Kun LE. (2004) Late neurocognitive sequelae in survivors of brain tumors in childhood. Lancet Oncol 5(7):399–408

    Article  PubMed  Google Scholar 

  51. Fossati P, Ricardi U, Orecchia R. (2009) Pediatric medullo-blastoma: toxicity of current treatment and potential role of protontherapy. Cancer Treat Rev 35(1):79–96

    Article  PubMed  CAS  Google Scholar 

  52. Gottardo NG, Gajjar A. (2006) Current therapy for medullo-blastoma. Curr Treat Options Neurol 8(4):319–334

    Article  PubMed  Google Scholar 

  53. Albright AL, Wisoff JH, Zeltzer PM, Boyett JM, Rorke LB, Stanley P. (1996) Effects of medulloblastoma resections on outcome in children: a report from the Children's Cancer Group. Neurosurgery 38(2):265–271

    Article  PubMed  CAS  Google Scholar 

  54. Sanders RP, Onar A, Boyett JM, et al (2008) M1 Medullo-blastoma: high risk at any age. J Neurooncol 90(3):351–355

    Article  PubMed  Google Scholar 

  55. Mason WP, Grovas A, Halpern S, et al (1998) Intensive chemotherapy and bone marrow rescue for young children with newly diagnosed malignant brain tumors. J Clin Oncol 16(1):210–221

    PubMed  CAS  Google Scholar 

  56. Cohen BH, Packer RJ. (1996) Chemotherapy for medullo-blastomas and primitive neuroectodermal tumors. J Neurooncol 29(1):55–68

    Article  PubMed  CAS  Google Scholar 

  57. Polkinghorn WR, Tarbell NJ. (2007) Medulloblastoma: tumorigenesis, current clinical paradigm, and efforts to improve risk stratification. Nat Clin Pract Oncol 4(5):295–304

    Article  PubMed  CAS  Google Scholar 

  58. Gajjar A, Hernan R, Kocak M, et al (2004) Clinical, histo-pathologic, and molecular markers of prognosis: toward a new disease risk stratification system for medulloblastoma. J Clin Oncol 22(6):984–993

    Article  PubMed  CAS  Google Scholar 

  59. Pomeroy SL, Tamayo P, Gaasenbeek M, et al (2002) Prediction of central nervous system embryonal tumor outcome based on gene expression. Nature 415(6870):436–442

    Article  PubMed  CAS  Google Scholar 

  60. Bhat SR, Goodwin TL, Burwinkle TM, et al (2005) Profile of daily life in children with brain tumors: an assessment of health-related quality of life. J Clin Oncol 23(24):5493–5500

    Article  PubMed  Google Scholar 

  61. Ribi K, Relly C, Landolt MA, Alber FD, Boltshauser E, Grotzer MA. (2005) Outcome of medulloblastoma in children: long-term complications and quality of life. Neuropediatrics 36(6):357–365

    Article  PubMed  CAS  Google Scholar 

  62. Benesch M, Spiegl K, Winter A, et al (2008) A scoring system to quantify late effects in children after treatment for medulloblastoma/ependymoma and its correlation with quality of life and neurocognitive functioning. Childs Nerv Syst 25:779

    Google Scholar 

  63. Romer JT, Kimura H, Magdaleno S, et al (2004) Suppression of the Shh pathway using a small molecule inhibitor eliminates medulloblastoma in Ptc1(+/−)p53(−/−) mice. Cancer Cell 6(3):229–240

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Division of Neurosurgery, Hospital for Sick Children, Toronto, ON, Canada

    Shobhan Vachhrajani & Michael D. Taylor

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  1. Shobhan Vachhrajani
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  2. Michael D. Taylor
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Editors and Affiliations

  1. Universitätsklinikum München Klinikum Großhadern Neurochirurgische Klinik, Marchioninistr. 15, München, 81377, Germany

    Jörg-Christian Tonn

  2. Universitätsklinikum Hamburg Krankenhaus Eppendorf Klinik und Poliklinik für Neurochirurgie, Martinistr. 52, Neurologie, Hamburg, 20246, Germany

    Manfred Westphal

  3. Division of Neurosurgery, The Hospital for Sick Children, 555 University Avenue Suite 1502, Toronto, ON, M5G 1X8, Canada

    James T. Rutka

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Vachhrajani, S., Taylor, M.D. (2010). Medulloblastoma. In: Tonn, JC., Westphal, M., Rutka, J.T. (eds) Oncology of CNS Tumors. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-02874-8_36

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  • DOI: https://doi.org/10.1007/978-3-642-02874-8_36

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