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Medulloblastoma

  • Adekunle M. Adesina
  • J. Hunter
Chapter

Abstract

An invasive, high-grade (WHO grade IV) embryonal tumor defined both by histologic grade and location in the cerebellum. Even though this tumor shares histologic features with other central nervous system (CNS) primitive neuroectodermal tumors, it has been historically regarded as a distinct entity. Recent molecular studies including genomic and gene expression profiling, as well as signaling pathway dysregulation and biologic studies, have now justified its historic clinical definition as a distinct clinicopathologic entity with predominant occurrence in children. Most are sporadic. Less frequent occurrence in the setting of hereditary syndromes including Turcot’s syndrome with germline mutation of the adenomatous polyposis coli (APC) gene and Gorlin’s syndrome, the nevoid basal cell carcinoma syndrome with germ line mutation of the PTCH gene is seen in less than 2% of medulloblastomas.

Keywords

Medulloblastoma Large cell/anaplastic medulloblastoma Nodular/desmoplastic Extensively nodular Medullo-myoblastoma 

Suggested Reading

  1. Bailey P, Cushing H (1925) Medulloblastoma cerebelli: a common type of mid-cerebellar glioma of childhood. Arch Neurol Psychiatry 14:192–224Google Scholar
  2. Giangaspero F, Wellek S, Masuoka J, Gessi M, Kleihues P, Oghaki H (2006) Stratification on the basis of histopathologic grading. Acta Neuropathol 112:5–12PubMedCrossRefGoogle Scholar
  3. Giangaspero F, Perilongo G, Fondelli MP, Brisigotti M, Carollo C, Burnelli R, Burger PC, Garre ML (1999) Medulloblastoma with extensive nodularity: a variant with favorable prognosis. J Neurosurg 91:971–977PubMedCrossRefGoogle Scholar
  4. Giangaspero F, Rigobello L, Badiali M, Loda M, Andreini L, Basso G, Zorzi F, Montaldi A (1992) Large cell medulloblastoma. A distinct variant with highly aggressive behavior. Am J Surg pathol 16:687–693PubMedCrossRefGoogle Scholar
  5. 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 101:1445–1454CrossRefGoogle Scholar
  6. Meyers SP, Kemp SS, Tarr RW (1992) MR imaging features of medulloblastomas. AJR American J Roentgenol 158:859–865Google Scholar
  7. Panigrahy A, Krieger MD, Gonzalez-Gomez I et al (2006) Quantitative short echo time 1H-MR spectroscopy of untreated pediatric brain tumors: preoperative diagnosis and characterization. AJNR Am J Neuroradiol 27:560–572PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Adekunle M. Adesina
    • 1
  • J. Hunter
    • 2
  1. 1.Section of Neuropathology and Molecular Neuropathology Laboratory, Department of PathologyTexas Children’s Hospital, Baylor College of MedicineHoustonUSA
  2. 2.Department of RadiologyTexas Children’s Hospital, Baylor College of MedicineHoustonUSA

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