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

Lookup NU author(s): Charles McManamy, Dr Jane Pears, Professor Steven Clifford, Professor David Ellison

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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. © 2007 The Authors.


Publication metadata

Author(s): McManamy CS, Pears J, Weston C, Hanzely Z, Ironside J, Taylor R, Grundy R, Clifford SC, Ellison DW

Publication type: Article

Publication status: Published

Journal: Brain Pathology

Year: 2007

Volume: 17

Issue: 2

Pages: 151-164

Print publication date: 01/04/2007

ISSN (print): 1015-6305

ISSN (electronic): 1750-3639

URL: http://dx.doi.org/10.1111/j.1750-3639.2007.00058.x

DOI: 10.1111/j.1750-3639.2007.00058.x

PubMed id: 17388946


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