Medulloblastoma is the most common posterior fossa tumor of childhood. Affected patients typically present with rapidly progressive headache, nausea, and vomiting from obstructive hydrocephalus. Medulloblastomas are inherently invasive lesions and show a high frequency of dissemination at diagnosis. Thus, although surgery plays an essential role in reducing the tumor burden and in opening the ventricular system and although it has an impact on prognosis, multi modal adjuvant therapy is essential to treat what is in reality an invasive, potentially multicentric disease process. During the past 25 years, advances in the surgical and adjuvant management have substantially improved the outlook for children with these tumors, making this one of the true “success stories” of pediatric neuro-oncologyi however, there is still substantial room for further progress, particularly in children with extensive postoperative residual disease and in those with dissemination. Ongoing studies are exploring ways of improving further the survival rate in these “high-risk” patients and reducing the treatment-related long-term morbidity in “standard-risk” patients. This chapter reviews recent advances in the molecular biology, diagnosis, and treatment of these tumors.
Keywords
- Pediatric Brain Tumor
- Primitive Neuroectodermal Tumor
- Posterior Fossa Tumor
- Craniospinal Irradiation
- Implantable Drug Delivery System
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References
Pollack IF: Brain tumors in children.N Engl J Med1994, 331:1500–1507.
Roberts RO, Lynch CF, Jones MP,et al.: Medulloblastoma: A population-based study of 532 cases.J Neuropathol Exp Neurol1991,50:134–144.
Maleci A, Cervoni L, Delfini R: Medulloblastoma in children and adults: A comparative study.Acta Neurochir1992, 119:62–67.
Haie-Meder C, Song PY: Medulloblastoma: Differences in adults and children.Int J Radiat Oncol Biol Phys1995,32:1255–1257.
Bunin GR, Kuijten RR, Buckley JD,et al.: Relation between maternal diet and subsequent primitive neuroectodermal brain tumors in young children.N Engl J Med1993, 329:536–541.
Evans DGR, Farndon PA, Burnell LD,et al.: The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma.Br J Cancer1991, 64:959–961.
Johnson RL, Rothman AL, Xie J: Human homolog of patched, a candidate gene for the basal cell nevus synrome.Science1996, 272:1668–1671.
Raffel C, Jenkins RB, Frederick L,et al.: Sporadic medulloblastomas contain PTCH mutations.Cancer Res1997,57:842–845.
Mori T, Nagase H, Hori A,et al.: Germ-line and somatic mutations of the APC gene in patients with Turcot syndrome and analysis of APC mutations in brain tumors.Genes Chromosomes Cancer1994,9:168–172.
Hamilton SR, Lui B, Parsons RE,et al.: The molecular basis of Turcot's syndrome.N Engl J Med1995,332: 839–847.
Yong WH, Raffel C, von Deimling A,et al.: The APC gene in Turcot's syndrome [letter].N Engl J Med1995, 333:524.
Malkin D, Li FP, Strong LC,et al.: Gennline p53 mutations in a familial syndrome of breast cancer, sarcomas, and other neoplasms.Science1990, 250:1222–1228.
Bigner SH, Mark J, Friedman HS,et al.: Structural chromosoinal abnormalities in human medulloblastoma.Cancer Genet Cytogenet1988, 30:91–101.
Biegel JA, Rorke LB, Packer RJ,et al.: Isochromosome 17q in primitive neuroectodermal tumors of the central nervous system.Genes Chromosomes Cancer1989, 1:139–147.
Raffel C, Thomas GA, Tishler DM,et al.: Absence of p53 mutations in childhood central nervous system primitive neuroectodermal tumors.Neurosurgery1993,33:301–306.
Saylors RL, Sidransky D, Friedman HS: Infrequent p53 mutations in medulloblastoma.Cancer Res1991,51:4721–4723.
Cogen PH, McDonald JD: Tumor suppressor genes and medulloblastoma.J Neurooncol1996, 29:103–112.
Griffin CA, Hawkins AL, Packer RJ,et al.: Chromosome abnormalities in pediatric brain tumors.Cancer Res1988,48: 175–180.
Wasson JC, Saylors RL III, Zeltzer P,et al.: Oncogene amplification in pediatric brain tumors.Cancer Res1990,50:2987–2990.
Segal RA, Goumnerova LC, Kwon YK,et al.: Expression of the neurotrophin receptor TrkC is linked to a favorable outcome in medulloblastoma.Proc Natl Acad Sci USA1994,91:12867–12871.
Provias JP, Becker LE: Cellular and molecular pathology of medulloblastoma.J Neurooncol1996; 29: 35–43.
Packer RJ, Sutton LN, Rorke LB,et al.: Prognostic importance of cellular differentiation in medulloblastoma of childhood.J Neurosurg1984, 61:296–301.
GoldbergStern H, Gadoth N, Stern S,et al.: The prognostic significance of glial fibrillary acidic protein staining in medulloblastoma.Cancer1991,68:568–573.
Maraziotis T, Perentes E, Karamitopoulou E,et al. Neuron-associated class III flitubulin isotope, retinal Santigen, synaptbphysin, and glial fibrillary acidic protein in human medulloblastomas: A clinicopathological analysis of 36 cases.Acta Neuropathol (Berl)1992, 84:355–363. 25. Bailey P, Cuship.g H: Medulloblastoma cerebelli, a common type of midcerebellar glioma of childhood. Arch Neurol Psychiat 1925, 14:192–224.
Cushing H: Experiences with cerebellar medulloblas tomas: Critical review.APMIS1930, 1:1–86.
Rorke LB: The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors.J Neuropathol Exp Neurol1983, 42: 1–15.
Rubinstein LJ: Embryonal central neuroepithelial tumors and their differentiating potential: A cytogenetic view of a complex neuiooncgl6gical problem.J Neurosurg1985,62:795–805.
Albright AL, Wisoff JR, Zeltzer P,et al.: Prognostic factors in children with supratentorial (nonpineal) primitive neuroectodermal tumors.Pediatr Neurosurg1995, 22:1–7.
Pigott TJD, Punt JAG, Lowe JS,et al.: The clinical, radiological and histopathological features of cerebral primitive neuroectodermal tumors.Br J Neurosurg1990,4:287–298.
Kayama T, Yoshimoto T, Shimizu H,et al.: Neonatal medulloblastoma.J Neurooncol1993, 15:157–163.
Meyers SP; Kemp SS, Tarr RW: MR imaging features of medulloblastomas.AJR1992, 158:859–865.
Mueller DP, Moore SA, Sato Y,et al.: MRI spectrum of medulloblastoma.Clin Imaging1992, 16:250–255.
Albright AL, Wisoff JH, Zeltzer PM,et al.: Effects of medulloblastoma resections on outcome in children: A report from the Children's Cancer Group.Neurosurgery1996, 38:264–271.
Abraham J, Chandy J: Ventriculo-atrial shunt in the management of posterior fossa tumors. Preliminary report.J Neurosurg1963, 20:252–253.
Grabb PA, Albright AL, Sclabassi RJ,et al: Conkuous intraoperative electromyography of cranial nerves during resection of fourth ventricular tumors in children.J Neurosurg1997, 86:1–4
Tomita T: Medulloblastomas. InNeurological Surgery. Edited by Youmans JR, edn 4. Philadelphia: WB Saunders; 1996:2570–2592.
Park TS; Hoffman HJ, Hendrick EB,et al.: Medulloblastoma: Clinical presentation and management. Experience at the Hospital for Sick Children, Toronto, 1950–1980.J Neurosurg1983, 58:543–552.
Hoffman H, Hendrick E, Humphreys R: Metastasis via ventriculoperitoneal shunt in patients with medulloblastoma. J Neurosurg 1976, 44:562–566.
Berger MS, Baumeister B, Geyer JR,et al.: The risks of metastases from shunting in children with primary central nervous system tumors.J Neurosurg1991, 74:872–877.
Sutton LN, Phillips PC, Molloy PT: Surgical management of medulloblastoma.J Neurooncol1996, 29:9–21.
Cochrane DD, Gustavsson B, Poskitt KP,et al: The surgical and natural morbidity of aggressive resection for posterior fossa tumors in childhood. Pediatr Neurosurg 1994, 20:19–29.
Wisoff JH, Epstein FJ: Pseudobulbar palsy after posterior fossa operation in children.Neurosurgery1984, 15:707–709.
Pollack IF, Polinko P, Albright AL,et al: Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children: Incidence and pathophysiology.Neurosurgery 1995, 37:885–893.
Pollack IF, Polinko P, Albright AL,et al.: Neurobehavioral syndromes following posterior fossa surgery in children. InThe Cerebellum and Cognition. Edited by Schmahmann JD. San Diego: Academic Press; 1997.
Chang CH, Housepian EM, Herbert C Jr: An operative staging system and megavoltage radiotherapeutic technique for cerebellar medulloblastoma.Radiology1969, 93:1351–1359.
Evans AE, Jenkin DT, 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 Neurosurg1990, 72:572–582.
Tait DM, Thornton-Jones H, Bloom HJG,et al.: Adjuvant chemotherapy for medulloblastoma: The first multi-centre control,.trial of the International Society of Paediatric Oncology (SIOP I).Bur J Cancer1990, 26:464–469.
Bourne JP, Geyer R, Berger M,et al: The prognostic significance of postoperative residual contrast enhancement on CT scan in pediatric patients with medulloblastoma.J Neurooncol1992, 14:262–270.
Jenkin D, Goddard K, Armstrong D,et al: Posterior fossa medulloblastoma in childhood: Treatment results and a proposal for a new staging system.Int J Radiat Oncol Biol Phys1990,19:265–274.
Krischer JP, Ragab AR, Kun L,et al.: Nitrogen mustard, vincristine, procarbazine, and prednisone as adjuvant chemotherapy in the treatment of medulloblastoma.J Neurosurg1991,74:905–909.
Patterson R, Farr RF: Cerebellar medulloblastomas: treatment by irradiation of the whole central nervous system. Acta Radiol1953, 39:323–336.
Kun LE, Constine LS: Medulloblastoma: Caution regarding new treatment approaches.Int J Radiat Oncol Biol Phys1991,20:897–899.
Deutsch M, Thomas PRM, Krischer J,et al.: Results of a prospective randomized trial comparing standard dose neuraxis irradiation (3,600 cGy/20) with reduced neuraxis irradiation (2,340/13) in patients with low-stage medulloblastoma: A combined Children's Cancer Group Pediatric Oncology Group study.Pediatr Neurosurg1996, 24:167–177.
Packer RJ, Sutton LN, Goldwein JW,et al.: Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma.J Neurosurg1991, 74:433–440.
Packer RJ, Sutton LN, Elterman R,et al.: Outcome for children with medulloblastoma treated with radiation and cisplatin, CCNU, and vincristine chemotherapy.J Neurosurg1994,81: 690–698.
Halberg FE, Wara WM, Fippin LF,et al.: Low-dose craniospinal radiation therapy for medulloblastoma.Int J Radiat Oncol Biol Phys1991, 20:651–654.
Bailey CC, Gnekow A, Wellek S,et al: Prospective randomized trial of chemotherapy given before radiotherapy in childhood medulloblastoma. International Society of Paediatric OncQlogy (SIOP) and the German Society of Paediatric Oncology(GPO):SIOP II.Med Pediatr Oncol 1995,25:166–178.
Packer RJ, Goldwein J, Nicholson S,et al: Reduced-dose radiotherapy plus chemotherapy for medulloblastoma. Proceedings of the Northern American Society for Neurooncology Meeting 1996[abstract 97]. Sante Fe: The Northern American Society for Neuro-oncology Meeting; 1996.
Pendergrass TW, Milstein JM, Geyer JR,et al.: Eight drugs in one day chemotherapy for brain tumors: Experience in 107 children and rationale for preradiation chemotherapy. J Clin Oncol1987, 5:1221–1231.
Gentet JC, Bouffet E, Doz F,et al.: Preirradiation chemotherapy including" eight drugs in one day" regimen and high-dose methotrexate in childhood medulloblastoma: Results of the M7 French Cooperative Study. J Neurosurg 1995, 82:60–14.
Duffner PK, Horowitz ME, Krischer JP,et al: Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors.N Engl J Med1993,24:1725–1731.
Kovnar EH, Kellie SJ, Horowitz ME,et al: Preirradiation cisplatin and etoposide in the treatment of high-risk medulloblastoma and other malignant embryonal tumors of the central nervous system: A phase II study.J Clin Oncol1990, 8:330–336.
Belza MG, Donaldson SS, Steinberg GK,et al.: Medulloblastoma: Freedom from relapselonger than 8 years: A therapeutic cure?J Neurosurg1991, 75:575–582.
Torres CF, Rebsamen S, Silber JH,et al.: Surveillance scanning of children with medulloblastoma.N Engl J Med 1994, 330:892–895.
Mendel E, Levy ML, Raffel C,et al.: Surveillance imaging in children with primitive neuroectodermal tumors.Neurosurgery 1996,38:692–695.
Friedman HS, Mahaley MS, Schold SC,et al.: Efficacy of vincristine and cyclophosphamide in the therapy of recurrent medulloblastoma.Neurosurgery1986, 18:335–340.
Bertolone SJ, Baum ES, Krivit W,et al.: A phase II study of cisplatin therapy in recurrent childhood brain tumors: A report from the Childrens's Cancer Group.J Neurooncol 1989,7:5–11.
Patrice SJ, Tarbell NJ, Goumnerova LC,et al.: Results of radiosurgery in the management of recUrrent and residual medulloblastoma.Pediatr Neurosurg1995, 22:197–203.
Lundberg JH, Weissman DE, Beatty PA,et al: Treatment of recurrent metastatic medulloblastoma with intensive chemotherapy and allogeneic bone marrow transplantation. I Neuroonco11992, 13:151–155.
Dunkel IJ, Finlay JL: High dose chemotherapy with autologous stem cell rescue for patients with medulloblastoma. J Neurooncol1996, 29:69–74.
Brown WD, Tavare CJ, Sobel EL,et al.: Medulloblastoma and Collins' Law: A critical review of the concept of a period of risk for tumor recurrence and patient survival. Neurosurgery1995,36:691–697.
Lefkowitz IB, Packer RJ, Ryan SG,et al.: Late recurrence of primitive neuroectodermal tumor/medulloblastoma. Cancer 1988, 62:826–830.
Ellenberg L, McComb JG, Siegel S,et al.: Factors affecting intellectual outcome in pediatric brain tumor patients. Neurosurgery1987,21:638–644.
Radcliffe J, Packer RJ, Atkins TE,et al.: Three and fouryear cognitive outcome in children with noncortical brain tumors treated with whole-brain radiotherapy.Ann Neurol1992,32:551–554.
Duffner PK, Cohen ME, Thomas PRM,et al.: The longterm effects of cranial irradiation on the central nervous system.Cancer1985,56:1841–1846.
Johnson DL, McCabe MA, Nicholson HS,et al: Quality of long-term survival in young children with medulloblastoma,J Neurosurg1994, 80: 1004–10 10.
Hirsch JF, Renier D, Czernichow P,et al: Medulloblastoma in childhood: Survival and functional results.Acta Neurochir1979,48:1–15.
Seaver E, Geyer R, Sulzbacher S,et al: Psychosocial adjustment in long-term survivors of childhood medulloblastoma and ependymoma treated with craniospinal irradiation.Pediatr Neurosurg1994, 20:248–253.
Hoppe-Hirsch E, Renier D, Lellouch-Tubiana A,et al.: Medulloblastoma in childhood: Progressive intellectual deterioration.Child's Nero Syst1990, 6:60–65.
Kanev PM, Lefebvre JF, Mauseth RS,et al.: Growth hormone deficiency following radiation therapy of primary brain tumors in children.J Neurosurg1991, 74:743–748.
Duffner PK, Cohen ME, Voorhess ML,et al.: Long-term effects of cranial irradiation on endocrine function in children with brain tumors: A prospective study.Cancer 1985, 56:2189–193.
Livesey EA, Hiridmarsh PC, Brook CGD,et al.: Endocrine disorders following treatment of childhood brain tumours.Br J Cancer1990,61:622–625.
Probert JC, Parker BR, Kaplan HS: Growth retardation in children after megavoltage irradiation of the spine. Cancer1973, 32:634–639.
Clayton PE, Shalet SM, Price DA,et al.: Testicular damage after chemotherapy for childhood brain tumors.J Pediatr 1988, 112:922–926.
Hawkins MM, Draper GJ, Kingston IE: Incidence of second primary tumors among childhood cancer survivors. Br J Cancer1987,56:339–347.
Farwell J, Flannery JT: Second primaries in children with central nervous system tumors.J Neurooncol1984, 2:371–375.
De Vathaire F, Francois P, Hill C,et al: Role of radiotherapy and chemotherapy in the risk of second malignant neoplasms after cancer in childhood.Br J Cancer1989, 59:792–796.
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Follack, I.F. (1998). Current Management of Medulloblastoma. In: Salcman, M. (eds) Current Techniques in Neurosurgery. Springer, New York, NY. https://doi.org/10.1007/978-1-4613-9350-4_19
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