Introduction: Medulloblastoma (MB) is one of the commonest malignant brain tumors in children. Still, its overall incidence in the general population is low. We present a single center experience of cases of medulloblastoma, seen over 25 years from 1990-2015 at the Massachusetts General Hospital.

Methods: Medical records of Massachusetts General Hospital (MGH) in Boston were searched for medulloblastoma occurring between ages of 0-19 and from 1990-2015.

Results: Between 1990 and 2015, 39 cases of pediatric medulloblastoma tumors were evaluated at MGH. 15 patients had received surgery and follow up treatment at MGH. 24 were operated upon at outside hospitals and either the tumors were examined or the patients were followed at MGH post-operatively. Of the 15 patients operated on at MGH, all presented with headache, nausea and vomiting. Most had diplopia and all had obstructive hydrocephalus. Of the 14 patients with operative records available, 11 had gross-total and 3 sub-total resection. Post-operatively, all showed clinical and radiological improvement. Four had posterior fossa syndrome. Patients were followed for a mean period of 7.4 years (range 0.5 to 16.8 years). Nine patients were followed for more than 5 years, and five for more than 10 years. Five patients relapsed, three of whom died. Known overall survival rates at 1, 3, and 5 years were 100%, 92%, and 90% respectively. Progression free survival rates at 1, 3, and 5 years were 86%, 67%, and 60%.

Conclusions: Medulloblastoma is the commonest brain tumor in children but its overall incidence in population is low. Treatment with surgery, RT and chemotherapy is improving outcomes but morbidity and mortality remain high.

Patient Care: Providers will have a more informed perspective when designing treatment plans for and managing pediatric patients with medulloblastoma.

Learning Objectives: By the conclusion of this session, participants should be familiar with presentation, management and prognosis of pediatric medulloblastoma.

References: 1. Ostrom QT, Gittleman H, Fulop J, et al. CBTRUS Statistical Report: Primary Brain and Central Nervous System Tumors Diagnosed in the United States in 2008-2012. Neuro Oncol 2015; 17 Suppl 4:iv1-iv62. 2. Ward E, DeSantis C, Robbins A, et al. Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin 2014;64:83-103 3. Peris-Bonet R, Martínez-García 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. 4. Alston RD, Newton R, Kelsey A, et al. Childhood medulloblastoma in northwest England 1954 to 1997: incidence and survival. Dev Med Child Neuro 2003;45:308-14. 5. Harder T, Plagemann A, Harder A. Birth weight and subsequent risk of childhood primary brain tumors: a meta-analysis. Am J Epidemiol 2008;168:366-73. 6. Fear NT, Roman E, Ansell P, et al. Malignant neoplasms of the brain during childhood: the role of prenatal and neonatal factors (United Kingdom). Cancer Causes Control 2001;12:443-9. 7. Harding NJ, Birch JM, Hepworth SJ, et al. Infectious exposure in the first year of life and risk of central nervous system tumors in children: analysis of day care, social contact, and overcrowding. Cancer Causes Control 2009;20:129-36. 8. Pogoda JM, Preston-Martin S, Howe G. An international case-control study of maternal diet during pregnancy and childhood brain tumor risk: a histology-specific analysis by food group. Ann Epidemiol 2009;19:148-60. 9. Rosso AL, Hovinga ME, Rorke-Adams LB, et al. Children's Oncology Group. A case-control study of childhood brain tumors and fathers' hobbies: a Children's Oncology Group study. Cancer Causes Control 2008;19:1201-7. 10. Cordier S, Lefeuvre B, Filippini G, et al. Parental occupation, occupational exposure to solvents and polycyclic aromatic hydrocarbons and risk of childhood brain tumors (Italy, France, Spain). Cancer Causes Control 1997;8:688-97. Erratum in: Cancer Causes Control 1997;8:934. 11. Giangaspero, F., Eberhart, C.G., Ellison, D.W., et al. WHO Classification of Tumours of the Nervous System. Lyon IARC Press; 2007. 12. Rutkowski S, Bode U, Deinlein F, et al. Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. N Engl J Med 2005;352:978-86. 13. McManamy CS, Pears J, Weston CL, et al. Clinical Brain Tumour Group. Nodule formation and desmoplasia in medulloblastomas-defining the nodular/desmoplastic variant and its biological behavior. Brain Pathol 2007;17:151-64. 14. Massimino M, Biassoni V, Gandola L, et al. Childhood medulloblastoma. Crit Rev Oncol Hematol 2016;105:35-51. 15. Meyers SP, Kemp SS, Tarr RW. MR imaging features of medulloblastomas. AJR Am J Roentgenol 1992;158:859-65. 16. Sutton LN, Phillips PC, Molloy PT. Surgical management of medulloblastoma. J Neurooncol 1996;29:9-21. Review. 17. Robertson PL, Muraszko KM, Holmes EJ, et al. Children's Oncology Group. Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: a prospective study by the Children's Oncology Group. J Neurosurg 2006;105:444-51. 18. Korah MP, Esiashvili N, Mazewski CM, et al. Incidence, risks, and sequelae of posterior fossa syndrome in pediatric medulloblastoma. Int J Radiat Oncol Biol Phys 2010;77:106-12. 19. Merchant TE, Kun LE, Krasin MJ, et al. 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 2008;70:782-7. 20. Miralbell R, Lomax A, Cella L, Schneider U. 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 2002;54:824-9. 21. Carrie C, Muracciole X, Gomez F, et al. French Society of Pediatric Oncology. 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. 22. DeSouza RM, Jones BR, Lowis SP, et al. Pediatric medulloblastoma - update on molecular classification driving targeted therapies. Front Oncol 2014;4:176. 23. Ramaswamy V, Remke M, Bouffet E, et al. Risk stratification of childhood medulloblastoma in the molecular era: the current consensus. Acta Neuropathol 2016;131:821-31. 24. Yeom KW, Mobley BC, Lober RM, et al. Distinctive MRI features of pediatric medulloblastoma subtypes. Am J Roentgenol 2013;200:895–903. 25. Ivanov DP, Coyle B, Walker DA, et al. In vitro models of medulloblastoma: Choosing the right tool for the job. J Biotechnol 2016;236:10-25.