Nodule formation and desmoplasia in medulloblastomas - Defining the nodular/desmoplastic variant and its biological behavior

Charles S. McManamy, Jane Pears, Claire L. Weston, Zoltan Hanzely, James W. Ironside, Roger E. Taylor, Richard G. Grundy, Steven C. Clifford, David W. Ellison

Research output: Contribution to journalArticle

105 Citations (Scopus)

Abstract

Among the variants of medulloblastoma in the current WHO classification of nervous system tumors, the desmoplastic variant, which has been reported to constitute 5%-25% of pediatric medulloblastomas, is defined by its nodular collections of neurocytic cells bounded by desmoplastic internodular zones. We have studied the frequency, morphological features and biological behavior of medulloblastomas in two contemporaneous SIOP/UKCCSG trial cohorts of children with medulloblastomas, CNS9102 (n = 315) and CNS9204 (n = 35), focusing on tumors with nodular and desmoplastic phenotypes. In children aged 3-16 years (CNS9102), the nodular/desmoplastic medulloblastoma represented 5% of all tumors, while in infants aged <3 years (CNS9204) this variant represented 57% of medulloblastomas. Using iFISH to detect molecular cytogenetic abnormalities in medulloblastomas with a nodular architecture, we demonstrated distinct genetic profiles in desmoplastic and non-desmoplastic (classic and anaplastic) tumors; in particular, abnormalities of chromosome 17 occurred in the latter, but not the former. Significantly different outcomes were demonstrated for classic, nodular/desmoplastic and large cell/anaplastic medulloblastomas in both cohorts. In conclusion, the nodular/desmoplastic medulloblastoma appears to have clinical, genetic and biological characteristics that set it apart from other variants of this tumor.

Original languageEnglish
Pages (from-to)151-164
Number of pages14
JournalBrain Pathology
Volume17
Issue number2
DOIs
Publication statusPublished - Apr 1 2007

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neuroscience(all)
  • Clinical Neurology

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    McManamy, C. S., Pears, J., Weston, C. L., Hanzely, Z., Ironside, J. W., Taylor, R. E., Grundy, R. G., Clifford, S. C., & Ellison, D. W. (2007). Nodule formation and desmoplasia in medulloblastomas - Defining the nodular/desmoplastic variant and its biological behavior. Brain Pathology, 17(2), 151-164. https://doi.org/10.1111/j.1750-3639.2007.00058.x