Abstract
The current survival outcome for children with medulloblastoma is a remarkable testimony for the multidisciplinary approach in the management of pediatric brain tumors. Children older than 3 years of age who have a less than 1.5 cm2 residual tumor after resection with no evidence of dissemination (M0) are classified as having standard-risk disease, while those with a larger tumor bed residual or with tumor dissemination are classified as having high-risk disease. Treatment for these patients includes craniospinal irradiation followed by a boost to the posterior fossa or posterior fossa tumor bed as well as cisplatin-based chemotherapy. For children younger than 3 years, surgery followed by chemotherapy is the most common treatment approach with or without primary site radiotherapy (RT). Currently, four molecular subtypes of medulloblastoma with prognostic implications have been identified. Current protocols are examining de-escalation of treatment for some children with Wnt-pathway tumors with more aggressive therapy for Group 3 and 4 subtypes.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Ashley DM et al (2012) Induction chemotherapy and conformal radiation therapy for very young children with nonmetastatic medulloblastoma: Children’s Oncology Group study P9934. J Clin Oncol 30(26):3181–3186
Bailey CC et al (1995) Prospective randomised trial of chemotherapy given before radiotherapy in childhood medulloblastoma. International Society of Paediatric Oncology (SIOP) and the (German) Society of Paediatric Oncology (GPO): SIOP II. Med Pediatr Oncol 25(3):166–178
Belza MG et al (1991) Medulloblastoma: freedom from relapse longer than 8 years—a therapeutic cure? J Neurosurg 75(4):575–582
Bouffet E et al (1992) M4 protocol for cerebellar medulloblastoma: supratentorial radiotherapy may not be avoided. Int J Radiat Oncol Biol Phys 24(1):79–85
Brown AP et al (2013) Proton beam craniospinal irradiation reduces acute toxicity for adults with medulloblastoma. Int J Radiat Oncol Biol Phys 86(2):277–284
Buchsbaum J, Paulino AC (2015) Pediatric brain tumors. In: Riaz N, Lee NY, Lu JJ (eds) Target volume delineation for conformal and intensity-modulated radiation therapy. Springer, Cham, pp 495–514
Camara-Costa H et al (2015) Neuropsychological outcome of children treated for standard risk medulloblastoma in the PNET4 European randomized controlled trial of Hyperfractionated versus standard radiation therapy and maintenance chemotherapy. Int J Radiat Oncol Biol Phys 92(5):978–985
Carrie C et al (1992) Quality control of radiotherapeutic treatment of medulloblastoma in a multicentric study: the contribution of radiotherapy technique to tumour relapse. The French Medulloblastoma Group. Radiother Oncol 24(2):77–81
Carrie C et al (1999) Impact of targeting deviations on outcome in medulloblastoma: study of the French Society of Pediatric Oncology (SFOP). Int J Radiat Oncol Biol Phys 45(2):435–439
Carrie C et al (2009) Online quality control, hyperfractionated radiotherapy alone and reduced boost volume for standard risk medulloblastoma: long-term results of MSFOP 98. J Clin Oncol 27(11):1879–1883
Castro-Vita H et al (1980) Medulloblastomas. Rev Interam Radiol 5(3):77–82
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
Chintagumpala M, Paulino A, Panigrahy A, Hawkins C, Jae A, Parsons DW (2016) Embryonal and pineal region tumors. In: Poplack DG, Pizzo PA (eds) Principles and practice of pediatric oncology. Wolters Kluwer, Philadelphia, pp 671–699
del Charco JO et al (1998) Medulloblastoma: time-dose relationship based on a 30-year review. Int J Radiat Oncol Biol Phys 42(1):147–154
Deschavanne PJ, Fertil B (1996) A review of human cell radiosensitivity in vitro. Int J Radiat Oncol Biol Phys 34(1):251–266
Douglas JG et al (2004) Concurrent chemotherapy and reduced-dose cranial spinal irradiation followed by conformal posterior fossa tumor bed boost for average-risk medulloblastoma: efficacy and patterns of failure. Int J Radiat Oncol Biol Phys 58(4):1161–1164
Doxey D et al (1999) Posterior fossa syndrome: identifiable risk factors and irreversible complications. Pediatr Neurosurg 31(3):131–136
Duffner PK 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(24):1725–1731
Eaton BR et al (2016a) Clinical outcomes among children with standard-risk medulloblastoma treated with proton and photon radiation therapy: a comparison of disease control and overall survival. Int J Radiat Oncol Biol Phys 94(1):133–138
Eaton BR et al (2016b) Endocrine outcomes with proton and photon radiotherapy for standard risk medulloblastoma. Neuro-Oncology 18(6):881–887
Evans AE et al (1990) The treatment of medulloblastoma. Results of a prospective randomized trial of radiation therapy with and without CCNU, vincristine, and prednisone. J Neurosurg 72(4):572–582
Evans G et al (1993) Congenital anomalies and genetic syndromes in 173 cases of medulloblastoma. Med Pediatr Oncol 21(6):433–434
Fouladi M et al (1999) Comparison of CSF cytology and spinal magnetic resonance imaging in the detection of leptomeningeal disease in pediatric medulloblastoma or primitive neuroectodermal tumor. J Clin Oncol 17(10):3234–3237
Gajjar A et al (1999) Comparison of lumbar and shunt cerebrospinal fluid specimens for cytologic detection of leptomeningeal disease in pediatric patients with brain tumors. J Clin Oncol 17(6):1825–1828
Gajjar A 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
Gandola L et al (2009) Hyperfractionated accelerated radiotherapy in the Milan strategy for metastatic medulloblastoma. J Clin Oncol 27(4):566–571
Garton GR et al (1990) Medulloblastoma—prognostic factors and outcome of treatment: review of the Mayo Clinic experience. Mayo Clin Proc 65(8):1077–1086
Gentet JC et al (1995) Preirradiation chemotherapy including “eight drugs in 1 day” regimen and high-dose methotrexate in childhood medulloblastoma: results of the M7 French Cooperative study. J Neurosurg 82(4):608–614
Geyer JR et al (2005) Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from the Children’s Cancer Group. J Clin Oncol 23(30):7621–7631
Gibson P et al (2010) Subtypes of medulloblastoma have distinct developmental origins. Nature 468(7327):1095–1099
Goldwein JW et al (1996) Updated results of a pilot study of low dose craniospinal irradiation plus chemotherapy for children under five with cerebellar primitive neuroectodermal tumors (medulloblastoma). Int J Radiat Oncol Biol Phys 34(4):899–904
Grill J et al (2005) Treatment of medulloblastoma with postoperative chemotherapy alone: an SFOP prospective trial in young children. Lancet Oncol 6(8):573–580
Gupta T et al (2012) Early clinical outcomes demonstrate preserved cognitive function in children with average-risk medulloblastoma when treated with hyperfractionated radiation therapy. Int J Radiat Oncol Biol Phys 83(5):1534–1540
Gurney JG et al (1996) Trends in cancer incidence among children in the U.S. Cancer 78(3):532–541
Harisiadis L, Chang CH (1977) Medulloblastoma in children: a correlation between staging and results of treatment. Int J Radiat Oncol Biol Phys 2(9–10):833–841
Huang E et al (2002) Intensity-modulated radiation therapy for pediatric medulloblastoma: early report on the reduction of ototoxicity. Int J Radiat Oncol Biol Phys 52(3):599–605
Jakacki RI et al (2004) A pilot study of preirradiation chemotherapy and 1800 cGy craniospinal irradiation in young children with medulloblastoma. Int J Radiat Oncol Biol Phys 60(2):531–536
Jenkin RD (1969) Medulloblastoma in childhood: radiation therapy. Can Med Assoc J 100(2):51–53
Jereb B et al (1984) Radiation for medulloblastoma adjusted to prevent recurrence to the cribriform plate region. Cancer 54(3):602–604
Johnston DL et al (2009) Medulloblastoma in children under the age of three years: a retrospective Canadian review. J Neuro-Oncol 94(1):51–56
Johnstone PA et al (2013) Pediatric CSI: are protons the only ethical approach? Int J Radiat Oncol Biol Phys 87(2):228–230
Kennedy C et al (2014) Quality of survival and growth in children and young adults in the PNET4 European controlled trial of hyperfractionated versus conventional radiation therapy for standard-risk medulloblastoma. Int J Radiat Oncol Biol Phys 88(2):292–300
Korah MP et al (2010) Incidence, risks, and sequelae of posterior fossa syndrome in pediatric medulloblastoma. Int J Radiat Oncol Biol Phys 77(1):106–112
Kortmann RD (2014) The chemotherapy before or after radiation therapy does not influence survival of children with high-risk medulloblastomas: results of the multicenter and randomized study of the Pediatric Oncology Group (POG 9031). Strahlenther Onkol 190(1):106–108
Kortmann RD et al (2000) 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 46(2):269–279
Krischer JP et al (1991) Nitrogen mustard, vincristine, procarbazine, and prednisone as adjuvant chemotherapy in the treatment of medulloblastoma. A Pediatric Oncology Group study. J Neurosurg 74(6):905–909
Lannering B et al (2012) Hyperfractionated versus conventional radiotherapy followed by chemotherapy in standard-risk medulloblastoma: results from the randomized multicenter HIT-SIOP PNET 4 trial. J Clin Oncol 30(26):3187–3193
Lin CT, Riva-Cambrin JK (2015) Management of posterior fossa tumors and hydrocephalus in children: a review. Childs Nerv Syst 31(10):1781–1789
Mazloom A, Zangeneh AH, Paulino AC (2010) Prognostic factors after extraneural metastasis of medulloblastoma. Int J Radiat Oncol Biol Phys 78(1):72–78
Merchant TE et al (2008) Multi-institution prospective trial of reduced-dose craniospinal irradiation (23.4 Gy) followed by conformal posterior fossa (36 Gy) and primary site irradiation (55.8 Gy) and dose-intensive chemotherapy for average-risk medulloblastoma. Int J Radiat Oncol Biol Phys 70(3):782–787
Michalski JM et al (2016) Results of COG ACNS0331: A phase III trial of involved-field radiotherapy (IFRT) and low dose craniospinal irradiation (LD-CSI) with chemotherapy in average-risk medulloblastoma: a report from the children’s oncology group. Int J Radiat Oncol Biol Phys 96(5):937–938
Miralbell R et al (2002) Potential reduction of the incidence of radiation-induced second cancers by using proton beams in the treatment of pediatric tumors. Int J Radiat Oncol Biol Phys 54(3):824–829
Moxon-Emre I et al (2014) Impact of craniospinal dose, boost volume, and neurologic complications on intellectual outcome in patients with medulloblastoma. J Clin Oncol 32(17):1760–1768
Northcott PA et al (2011) Medulloblastoma comprises four distinct molecular variants. J Clin Oncol 29(11):1408–1414
Oyharcabal-Bourden V et al (2005) Standard-risk medulloblastoma treated by adjuvant chemotherapy followed by reduced-dose craniospinal radiation therapy: a French Society of Pediatric Oncology Study. J Clin Oncol 23(21):4726–4734
Packer RJ et al (1999) Treatment of children with medulloblastomas with reduced-dose craniospinal radiation therapy and adjuvant chemotherapy: a Children’s Cancer Group study. J Clin Oncol 17(7):2127–2136
Packer RJ et al (2006) Phase III study of craniospinal radiation therapy followed by adjuvant chemotherapy for newly diagnosed average-risk medulloblastoma. J Clin Oncol 24(25):4202–4208
Paraf F, Jothy S, Van Meir EG (1997) Brain tumor-polyposis syndrome: two genetic diseases? J Clin Oncol 15(7):2744–2758
Paterson E, Farr RF (1953) Cerebellar medulloblastoma: treatment by irradiation of the whole central nervous system. Acta Radiol 39(4):323–336
Paulino AC (1997) Radiotherapeutic management of medulloblastoma. Oncology (Williston Park) 11(6):813–823; discussion 823, 827–8, 831.
Paulino AC et al (2003) Protracted radiotherapy treatment duration in medulloblastoma. Am J Clin Oncol 26(1):55–59
Paulino AC et al (2010) Ototoxicity after intensity-modulated radiation therapy and cisplatin-based chemotherapy in children with medulloblastoma. Int J Radiat Oncol Biol Phys 78(5):1445–1450
Paulino AC et al (2011) Local control after craniospinal irradiation, intensity-modulated radiotherapy boost, and chemotherapy in childhood medulloblastoma. Cancer 117(3):635–641
Pollack IF (1997) Posterior fossa syndrome. Int Rev Neurobiol 41:411–432
Ramaswamy V et al (2013) Recurrence patterns across medulloblastoma subgroups: an integrated clinical and molecular analysis. Lancet Oncol 14(12):1200–1207
Ris MD et al (2001) Intellectual outcome after reduced-dose radiation therapy plus adjuvant chemotherapy for medulloblastoma: a Children’s Cancer Group study. J Clin Oncol 19(15):3470–3476
Ris MD et al (2013) Intellectual and academic outcome following two chemotherapy regimens and radiotherapy for average-risk medulloblastoma: COG A9961. Pediatr Blood Cancer 60(8):1350–1357
Rutkowski S et al (2005) Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. N Engl J Med 352(10):978–986
Shaw DW et al (1997) Asymptomatic recurrence detection with surveillance scanning in children with medulloblastoma. J Clin Oncol 15(5):1811–1813
Tait DM et al (1990) Adjuvant chemotherapy for medulloblastoma: the first multi-centre control trial of the International Society of Paediatric Oncology (SIOP I). Eur J Cancer 26(4):464–469
Tarbell NJ et al (1991) The change in patterns of relapse in medulloblastoma. Cancer 68(7):1600–1604
Tarbell NJ et al (2013) High-risk medulloblastoma: a pediatric oncology group randomized trial of chemotherapy before or after radiation therapy (POG 9031). J Clin Oncol 31(23):2936–2941
Taylor RE et al (2003) 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 21(8):1581–1591
Taylor RE et al (2004) Impact of radiotherapy parameters on outcome in the International Society of Paediatric Oncology/United Kingdom Children’s Cancer Study Group PNET-3 study of preradiotherapy chemotherapy for M0-M1 medulloblastoma. Int J Radiat Oncol Biol Phys 58(4):1184–1193
Taylor RE et al (2005) Outcome for patients with metastatic (M2-3) medulloblastoma treated with SIOP/UKCCSG PNET-3 chemotherapy. Eur J Cancer 41(5):727–734
Taylor MD et al (2012) Molecular subgroups of medulloblastoma: the current consensus. Acta Neuropathol 123(4):465–472
Teo WY et al (2013) Implications of tumor location on subtypes of medulloblastoma. Pediatr Blood Cancer 60(9):1408–1410
Torres CF et al (1994) Surveillance scanning of children with medulloblastoma. N Engl J Med 330(13):892–895
Verma J et al (2015) Comparison of supine and prone craniospinal irradiation in children with medulloblastoma. Pract Radiat Oncol 5(2):93–98
von Bueren AO et al (2011) Treatment of young children with localized medulloblastoma by chemotherapy alone: results of the prospective, multicenter trial HIT 2000 confirming the prognostic impact of histology. Neuro-Oncology 13(6):669–679
von Hoff K et al (2009) Long-term outcome and clinical prognostic factors in children with medulloblastoma treated in the prospective randomised multicentre trial HIT’91. Eur J Cancer 45(7):1209–1217
Walter AW et al (1999) Survival and neurodevelopmental outcome of young children with medulloblastoma at St Jude Children’s Research Hospital. J Clin Oncol 17(12):3720–3728
Wolden SL (2013) Protons for craniospinal radiation: are clinical data important? Int J Radiat Oncol Biol Phys 87(2):231–232
Wolden SL et al (2003) Patterns of failure using a conformal radiation therapy tumor bed boost for medulloblastoma. J Clin Oncol 21(16):3079–3083
Zeltzer PM et al (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(3):832–845
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2018 Springer International Publishing AG
About this chapter
Cite this chapter
Paulino, A.C., Carrie, C. (2018). Medulloblastoma. In: Mahajan, A., Paulino, A. (eds) Radiation Oncology for Pediatric CNS Tumors. Springer, Cham. https://doi.org/10.1007/978-3-319-55430-3_6
Download citation
DOI: https://doi.org/10.1007/978-3-319-55430-3_6
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-55428-0
Online ISBN: 978-3-319-55430-3
eBook Packages: MedicineMedicine (R0)