Supra- and infratentorial pediatric ependymomas differ significantly in NeuN, p75 and GFAP expression
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LABORATORY INVESTIGATION
Supra- and infratentorial pediatric ependymomas differ significantly in NeuN, p75 and GFAP expression Christian Hagel • Andra´s Treszl • Julia Fehlert • Jonas Harder • Franziska von Haxthausen • Meike Kern • Andre´ O. von Bueren • Uwe Kordes
Received: 10 August 2012 / Accepted: 22 January 2013 / Published online: 31 January 2013 Ó Springer Science+Business Media New York 2013
Abstract Ependymomas comprise 8 % of all intracranial tumors in children \15 years. Recent studies revealed that some supratentorial ependymomas express neuronal antigens and that high expression of neurofilament protein light polypeptide (NEFL) correlates with better clinical outcome. We retrospectively analyzed an expanded panel of proteins in 6 supratentorial, 15 posterior fossa and 4 spinal pediatric ependymomas by immunohistochemistry. Expression of high and low affinity neurotrophin receptors TrkA (NTRK1) and p75 (NGFR), pan-neuronal markers NeuN (RBFOX3) and synaptophysin, radial glial marker SOX9, adhesion molecules CD56 (NCAM) and CD44, junctional protein connexin 43 (GJA1), glial fibrillary acidic protein (GFAP), epithelial membrane antigen and proliferation associated antigen Ki-67 were evaluated in a semi-quantitative or quantitative (Ki-67 and NeuN-index) fashion. We found p75 and NeuN to be expressed at significantly higher levels in supratentorial versus infratentorial tumors and GFAP to be expressed at significantly higher levels in infratentorial lesions. In conclusion, immunohistochemical expression of p75, NeuN and GFAP differed in ependymomas depending on tumor topography supporting the
C. Hagel (&) J. Fehlert J. Harder F. von Haxthausen M. Kern Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Martinistr. 52, 20246 Hamburg, Germany e-mail: [email protected] A. Treszl Department of Medical Biometry and Epidemiology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany A. O. von Bueren U. Kordes Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
view of divergent cells of origin. However, because of the small sample size the results are of preliminary nature and replication in a larger cohort would be desirable. Keywords Ependymoma Childhood GFAP EMA p75 NeuN Sox9
Introduction In a population-based analysis of children up to 15 years of age ependymomas constitute the third most frequent brain tumor after pilocytic astrocytomas and medulloblastomas, accounting for 8 % of intracranial neoplasias in this age group [1]. They can be categorized into different groups according to topography, age and biological profile:(a) Spinal ependymomas tend to occur in older patients or as a manifestation of neurofibromatosis-2. The tumors are often low grade indolent lesions, including the myxopapillary variant. Commonly they show increased ploidy, gain of HOX family members or loss of NF2 [2–4].(b) Posterior fossa ependymomas occur more often in young children and tend to be high grade lesion. They may d
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