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Abstract

Medulloblastoma generally affects patients in the first two decades of life, accounting for about a fifth of all intracranial neoplasms of childhood. According to the 2007 WHO classification of tumors of the central nervous system, it belongs to primitive embryonal grade IV tumors.

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References

  1. Packer RJ, Cogen P, Vezina G, Rorke LB (1999) Medulloblastoma: clinical and biologic aspects. Neuro Oncol 1(3):232–250

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  2. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114(2):97

    Article  PubMed Central  PubMed  Google Scholar 

  3. Rorke LB (1983) The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors. J Neuropathol Exp Neurol 42(1):1–15

    Article  CAS  PubMed  Google Scholar 

  4. Pomeroy SL, Tamayo P, Gaasenbeek M, Sturla LM, Angelo M, McLaughlin ME, Kim JY, Goumnerova LC, Black PM, Lau C, Allen JC, Zagzag D, Olson JM, Curran T, Wetmore C, Biegel JA, Poggio T, Mukherjee S, Rifkin R, Califano A, Stolovitzky G, Louis DN, Mesirov JP, Lander ES, Golub TR (2002) Prediction of central nervous system embryonal tumour outcome based on gene expression. Nature 415(6870):436–442

    Article  CAS  PubMed  Google Scholar 

  5. Kleihues P, Cavenee W (2000) Tumours of the nervous system. World Health Organization Classification of Tumours. IARC, Lyon

    Google Scholar 

  6. Zeltzer PM, Boyett JM, Finlay JL, Albright AL, Rorke LB, Milstein JM, Allen JC, Stevens KR, Stanley P, Li H, Wisoff JH, Geyer JR, McGuire-Cullen P, Stehbens JA, Shurin SB, Packer RJ (1999) Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: conclusions from the Children?s Cancer Group 921 Randomized Phase III Study. J Clin Oncol 17:832–845

    CAS  PubMed  Google Scholar 

  7. Giangaspero F, Wellek S, Masuoka J, Gessi M, Kleihues P, Ohgaki H (2006) Stratification of medulloblastoma on the basis of histopathological grading. Acta Neuropathol 112(1):5–12

    Article  PubMed  Google Scholar 

  8. Eberhart CG, Kepner JL, Goldthwaite PT, Kun LE, Duffner PK, Friedman HS, Strother DR, Burger PC (2002) Histopathologic grading of medulloblastomas: a Pediatric Oncology Group study. Cancer 94(2):552–560

    Article  PubMed  Google Scholar 

  9. McManamy CS, Lamont JM, Taylor RE, Cole M, Pearson AD, Clifford SC, Ellison DW, United Kingdom Children’s Cancer Study Group (2003) Morphophenotypic variation predicts clinical behavior in childhood nondesmoplastic medulloblastomas. J Neuropathol Exp Neurol 62(6):627–632

    PubMed  Google Scholar 

  10. Urberuaga A, Navajas A, Burgos J, Pijoán JI (2006) A review of clinical and histological features of Spanish paediatric medulloblastomas during the last 21 years. Childs Nerv Syst 22(5):466–474

    Article  PubMed  Google Scholar 

  11. Rutkowski S, Bode U, Deinlein F, Ottensmeier H, Warmuth-Metz M, Soerensen N, Graf N, Emser A, Pietsch T, Wolff JE, Kortmann RD, Kuehl J (2005) Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. N Engl J Med 352(10):978–986

    Article  CAS  PubMed  Google Scholar 

  12. Taylor MD, Northcott PA, Korshunov A, Remke M, Cho YJ, Clifford SC, Eberhart CG, Parsons DW, Rutkowski S, Gajjar A, Ellison DW, Lichter P, Gilbertson RJ, Pomeroy SL, Kool M, Pfister SM (2012) Molecular subgroups of medulloblastoma: the current consensus. Acta Neuropathol 123(4):465–472

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  13. Globus JH, Strauss I (1925) Spongioblastoma multiforme. A primary malignant form of brain neoplasm: its clinical and anatomic features. Arch Neurol Psychiatry 14(2):139–191

    Article  Google Scholar 

  14. Bailey P, Cushing H (1925) Medulloblastoma cerebelli: a common type of midcerebellar glioma of childhood. Arch Neurol Psychiatry 14:192–224

    Article  Google Scholar 

  15. Bailey OT (1985–1986) Genesis of the Percival Bailey-Cushing classification of gliomas. Pediatr Neurosci 12(4–5):261–265

    Google Scholar 

  16. Cushing H (1930) Experiences in cerebellar medulloblastoma. Acta Pathol Microbiol Scand 1:1–86

    Google Scholar 

  17. Sümer-Turanlıgil NC, Cetin EÖ, Uyanıkgil Y (2013) A contemporary review of molecular candidates for the development and treatment of childhood medulloblastoma. Childs Nerv Syst 29(3):381–388

    Article  PubMed  Google Scholar 

  18. Gilbertson RJ, Ellison DW (2008) The origins of medulloblastoma subtypes. Annu Rev Pathol 3:341–365

    Article  CAS  PubMed  Google Scholar 

  19. Bühren J, Christoph AH, Buslei R, Albrecht S, Wiestler OD, Pietsch T (2000) 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 59(3):229–240

    PubMed  Google Scholar 

  20. Pomeroy SL, Sutton ME, Goumnerova LC, Segal RA (1997) Neurotrophins in cerebellar granule cell development and medulloblastoma. J Neurooncol 35(3):347–352

    Article  CAS  PubMed  Google Scholar 

  21. Salsano E, Pollo B, Eoli M, Giordana MT, Finocchiaro G (2004) Expression of MATH1, a marker of cerebellar granule cell progenitors, identifies different medulloblastoma sub-types. Neurosci Lett 370(2–3):180–185

    Article  CAS  PubMed  Google Scholar 

  22. Yokota N, Aruga J, Takai S, Yamada K, Hamazaki M, Iwase T, Sugimura H, Mikoshiba K (1996) Predominant expression of human zic in cerebellar granule cell lineage and medulloblastoma. Cancer Res 56(2):377–383

    CAS  PubMed  Google Scholar 

  23. Choux M, Lena G, Gentet JC, Paz Paredes A (2001) Medulloblastoma. In: MacLone DG (ed) Pediatric neurosurgery: surgery of the developing nervous system. Elsevier Health Sciences, Philadelphia, pp 804–821

    Google Scholar 

  24. Sandhou A, Kendall B (1987) Computed tomography in management of medulloblastomas. Neuroradiology 29:444–452

    Article  Google Scholar 

  25. Kleihues P, Cavenee W (eds) (2000) Pathology and genetics of tumours of the central nervous system. International Agency for Research on Cancer, Lyon, pp 129–137

    Google Scholar 

  26. Choux M, Lena G (1982) Medulloblastoma. Neurochirurgie 28(Suppl):1–229

    PubMed  Google Scholar 

  27. Ellison DW, Clifford SC, Giangaspero F (2006) Medulloblastoma. In: McLendon RE, Rosenblum MK, Bigner DD (eds) Russell and Rubinstein’s pathology of tumors of the nervous system. CRC Press, Boca Raton, FL, USA, pp 247–264

    Google Scholar 

  28. Eberhart CG, Cohen KJ, Tihan T, Goldthwaite PT, Burger PC (2003) Medulloblastoma with systemic metastases: evaluation of tumor histopathology and clinical behaviour in 23 patients. J Pediatr Hematol Oncol 25:198–203

    Article  PubMed  Google Scholar 

  29. Campbell AN, Chan HS, Becker LE, Daneman A, Park TS, Hoffman HJ (1984) Extracranial metastases in childhood primary intracranial tumors. A report of 21 cases and review of the literature. Cancer 53:974–981

    Article  CAS  PubMed  Google Scholar 

  30. Sure U, Bertalanffy H, Isenmann S, Brandner S, Berghorn WJ, Seeger W, Aguzzi A (1995) Secondary manifestation of medulloblastoma: metastases and local recurrences in 66 patients. Acta Neurochir 136:117–126

    Article  CAS  PubMed  Google Scholar 

  31. Kessler LA, Dugan P, Concannon JP (1975) Systemic metastases of medulloblastoma promoted by shunting. Surg Neurol 3:147–152

    CAS  PubMed  Google Scholar 

  32. Kool M, Korshunov A, Remke M, Jones DT, Schlanstein M, Northcott PA, Cho YJ, Koster J, Schouten-van Meeteren A, van Vuurden D, Clifford SC, Pietsch T, von Bueren AO, Rutkowski S, McCabe M, Collins VP, Bäcklund ML, Haberler C, Bourdeaut F, Delattre O, Doz F, Ellison DW, Gilbertson RJ, Pomeroy SL, Taylor MD, Lichter P, Pfister SM (2012) Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, group 3, and group 4 medulloblastomas. Acta Neuropathol 123(4):473–484

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  33. Leonard JR, Cai DX, Rivet DJ, Kaufman BA, Park TS, Levy BK, Perry A (2001) Large cell/anaplastic medulloblastomas and medullomyoblastomas: clinicopathological and genetic features. J Neurosurg 95(1):82–88

    Article  CAS  PubMed  Google Scholar 

  34. Helton KJ, Fouladi M, Boop FA, Perry A, Dalton J, Kun L, Fuller C (2004) Medullomyoblastoma: a radiographic and clinicopathologic analysis of six cases and review of the literature. Cancer 101(6):1445–1454

    Article  PubMed  Google Scholar 

  35. Smith TW, Davidson RI (1984) Medullomyoblastoma. A histologic, immunohistochemical, and ultrastructural study. Cancer 54(2):323–332

    Article  CAS  PubMed  Google Scholar 

  36. Dolman CL (1988) Melanotic medulloblastoma. A case report with immunohistochemical and ultrastructural examination. Acta Neuropathol 76(5):528–531

    Article  CAS  PubMed  Google Scholar 

  37. Jimenez CL, Carpenter BF, Robb IA (1987) Melanotic cerebellar tumor. Ultrastruct Pathol 11(5–6):751–759

    Article  CAS  PubMed  Google Scholar 

  38. Kalimo H, Paljärvi L, Ekfors T, Pelliniemi LJ (1987) Pigmented primitive neuroectodermal tumor with multipotential differentiation in cerebellum (pigmented medullomyoblastoma). A case with light- and electron-microscopic, and immunohistochemical analysis. Pediatr Neurosci 13(4):188–195

    Article  CAS  PubMed  Google Scholar 

  39. Gilbertson RJ, Jaros E, Perry RH, Kelly PJ, Lunec J, Pearson AD (1997) Mitotic percentage index: a new prognostic factor for childhood medulloblastoma. Eur J Cancer 33(4):609–615

    Article  CAS  PubMed  Google Scholar 

  40. Rubinstein LJ, Northfield DW (1964) The medulloblastoma and the so-called “arachnoidal cerebellar sarcoma”. Brain 87:379–412

    Article  CAS  PubMed  Google Scholar 

  41. McManamy CS, Pears J, Weston CL, Hanzely Z, Ironside JW, Taylor RE, Grundy RG, Clifford SC, Ellison DW, Clinical Brain Tumour Group (2007) Nodule formation and desmoplasia in medulloblastomas-defining the nodular/desmoplastic variant and its biological behavior. Brain Pathol 17(2):151–164

    Article  PubMed  Google Scholar 

  42. Pearl GS, Takei Y (1981) Cerebellar “neuroblastoma”: nosology as it relates to medulloblastoma. Cancer 47(4):772–779

    Article  CAS  PubMed  Google Scholar 

  43. Massimino M, Giangaspero F, Garrè ML, Gandola L, Poggi G, Biassoni V, Gatta G, Rutkowski S (2011) Childhood medulloblastoma. Crit Rev Oncol Hematol 79(1):65–83

    Article  PubMed  Google Scholar 

  44. Giangaspero F, Perilongo G, Fondelli MP, Brisigotti M, Carollo C, Burnelli R, Burger PC, Garrè ML (1999) Medulloblastoma with extensive nodularity: a variant with favorable prognosis. J Neurosurg 91(6):971–977

    Article  CAS  PubMed  Google Scholar 

  45. Ellison D (2002) Classifying the medulloblastoma: insights from morphology and molecular genetics. Neuropathol Appl Neurobiol 28(4):257–282

    Article  CAS  PubMed  Google Scholar 

  46. Geyer JR, Schofield D, Berger M, Milstein J (1992) Differentiation of a primitive neuroectodermal tumor into a benign ganglioglioma. J Neurooncol 14(3):237–241

    Article  CAS  PubMed  Google Scholar 

  47. Brown HG, Kepner JL, Perlman EJ, Friedman HS, Strother DR, Duffner PK, Kun LE, Goldthwaite PT, Burger PC (2000) “Large cell/anaplastic” medulloblastomas: a pediatric oncology group study. J Neuropathol Exp Neurol 59(10):857–865

    CAS  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  49. Maraziotis T, Perentes E, Karamitopoulou E, Nakagawa Y, Gessaga EC, Probst A, Frankfurter A (1992) Neuronassociated class III beta-tubulin isotype, retinal S-antigen, synaptophysin, and glial fibrillary acidic protein in human medulloblastomas: a clinicopathological analysis of 36 cases. Acta Neuropathol 84(4):355–363

    Article  CAS  PubMed  Google Scholar 

  50. Katsetos CD, Del Valle L, Legido A, de Chadarévian JP, Perentes E, Mörk SJ (2003) On the neuronal/neuroblastic nature of medulloblastomas: a tribute to Pio del Rio Hortega and Moises Polak. Acta Neuropathol 105(1):1–13

    PubMed  Google Scholar 

  51. Katsetos CD, Herman MM, Frankfurter A, Gass P, Collins VP, Walker CC, Rosemberg S, Barnard RO, Rubinstein LJ (1989) Cerebellar desmoplastic medulloblastomas. A further immunohistochemical characterization of the reticulin-free pale islands. Arch Pathol Lab Med 113(9):1019–1029

    CAS  PubMed  Google Scholar 

  52. Clifford SC, Lusher ME, Lindsey JC, Langdon JA, Gilbertson RJ, Straughton D, Ellison DW (2006) Wnt/Wingless pathway activation and chromosome 6 loss characterize a distinct molecular sub-group of medulloblastomas associated with a favorable prognosis. Cell Cycle 5(22):2666–2670

    Article  CAS  PubMed  Google Scholar 

  53. Ellison DW, Dalton J, Kocak M, Nicholson SL, Fraga C, Neale G, Kenney AM, Brat DJ, Perry A, Yong WH, Taylor RE, Bailey S, Clifford SC, Gilbertson RJ (2011) Medulloblastoma: clinicopathological correlates of SHH, WNT, and non-SHH/WNT molecular subgroups. Acta Neuropathol 121(3):381–396

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  54. Ellison DW, Kocak M, Dalton J, Megahed H, Lusher ME, Ryan SL, Zhao W, Nicholson SL, Taylor RE, Bailey S, Clifford SC (2011) Definition of disease-risk stratification groups in childhood medulloblastoma using combined clinical, pathologic, and molecular variables. J Clin Oncol 29(11):1400–1407

    Article  PubMed Central  PubMed  Google Scholar 

  55. Ellison DW, Onilude OE, Lindsey JC, Lusher ME, Weston CL, Taylor RE, Pearson AD, Clifford SC, United Kingdom Children’s Cancer Study Group Brain Tumour Committee (2005) beta-Catenin status predicts a favorable outcome in childhood medulloblastoma: the United Kingdom Children’s Cancer Study Group Brain Tumour Committee. J Clin Oncol 23(31):7951–7957

    Article  CAS  PubMed  Google Scholar 

  56. Northcott PA, Korshunov A, Witt H, Hielscher T, Eberhart CG, Mack S, Bouffet E, Clifford SC, Hawkins CE, French P, Rutka JT, Pfister S, Taylor MD (2011) Medulloblastoma comprises four distinct molecular variants. J Clin Oncol 29(11):1408–1414

    Article  PubMed  Google Scholar 

  57. Remke M, Hielscher T, Korshunov A, Northcott PA, Bender S, Kool M, Westermann F, Benner A, Cin H, Ryzhova M, Sturm D, Witt H, Haag D, Toedt G, Wittmann A, Schöttler A, von Bueren AO, von Deimling A, Rutkowski S, Scheurlen W, Kulozik AE, Taylor MD, Lichter P, Pfister SM (2011) FSTL5 is a marker of poor prognosis in non-WNT/non- SHH medulloblastoma. J Clin Oncol 29(29):3852–3861

    Article  CAS  PubMed  Google Scholar 

  58. Fernandez-L A, Northcott PA, Dalton J, Fraga C, Ellison D, Angers S, Taylor MD, Kenney AM (2009) YAP1 is amplified and up-regulated in hedgehog-associated medulloblastomas and mediates Sonic hedgehog-driven neural precursor proliferation. Genes Dev 23(23):2729–2741

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  59. Zhao B, Wei X, Li W, Udan RS, Yang Q, Kim J, Xie J, Ikenoue T, Yu J, Li L, Zheng P, Ye K, Chinnaiyan A, Halder G, Lai ZC, Guan KL (2007) Inactivation of YAP oncoprotein by the Hippo pathway is involved in cell contact inhibition and tissue growth control. Genes Dev 21(21):2747–2761

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  60. Al-Halabi H, Nantel A, Klekner A, Guiot MC, Albrecht S, Hauser P, Garami M, Bognar L, Kavan P, Gerges N, Shirinian M, Roberge D, Muanza T, Jabado N (2011) Preponderance of sonic hedgehog pathway activation characterizes adult medulloblastoma. Acta Neuropathol 121(2):229–239

    Article  PubMed  Google Scholar 

  61. Remke M, Hielscher T, Northcott PA, Witt H, Ryzhova M, Wittmann A, Benner A, von Deimling A, Scheurlen W, Perry A, Croul S, Kulozik AE, Lichter P, Taylor MD, Pfister SM, Korshunov A, Thompson MC, Fuller C, Hogg TL, Dalton J, Finkelstein D, Lau CC, Chintagumpala M, Adesina A, Ashley DM, Kellie SJ, Taylor MD, Curran T, Gajjar A, Gilbertson RJ (2006) Genomics identifies medulloblastoma subgroups that are enriched for specific genetic alterations. J Clin Oncol 24(12):1924–1931

    Article  Google Scholar 

  62. Chang CH, Housepian EM, Herbert C Jr (1969) An operative staging system and a megavoltage radiotherapeutic technic for cerebellar medulloblastomas. Radiology 93(6):1351–1359

    Article  CAS  PubMed  Google Scholar 

  63. Halperin EC, Constine LS, Tarbell NJ et al (1999) Tumors of the posterior fossa and the spinal cana. In: Pediatric radiation oncology, 3rd edn. Lippincott-Williams & Wilkins, Philadelphia, pp 80–125

    Google Scholar 

  64. Terterov S, Krieger MD, Bowen I, McComb JG (2010) Evaluation of intracranial cerebrospinal fluid cytology in staging pediatric medulloblastomas, supratentorial primitive neuroectodermal tumors, and ependymomas. J Neurosurg Pediatr 6(2):131–136

    Article  PubMed  Google Scholar 

  65. Wieczorek V, Kluge H, Linke E, Zimmermann K, Kuehn HJ, Witte OW, Isenmann S (2007) Pathological CSF cell findings in primary and metastatic CNS tumors, malignant lymphoma and leukemia. In: Kluge H (ed) Atlas of CSF cytology. Thieme, Stuttgard, Germany, pp 84–87

    Google Scholar 

  66. Gowda KK, Gupta K, Kapoor R, Vasishta RK (2012) Nuclear expression of β-catenin and stem cell markers as potential prognostic indicators in medulloblastoma. Neurol India 60(5):487–494

    Article  PubMed  Google Scholar 

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Correspondence to Giuliana Di Martino M.D. .

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Di Martino, G., Guadagno, E., Del Basso De Caro, M.L. (2015). Medulloblastoma: Pathology. 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_17

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  • DOI: https://doi.org/10.1007/978-3-319-11274-9_17

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