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Neurofibromin specific antibody differentiates malignant peripheral nerve sheath tumors (MPNST) from other spindle cell neoplasms

Malignant peripheral nerve sheath tumors (MPNST) derive from the Schwann cell or perineurial cell lineage and occur either sporadically or in association with the tumor syndrome neurofibromatosis type 1 (NF1). MPNST often pose a diagnostic challenge due to their frequent lack of pathognomonic morpho...

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Main Authors: Reuss, David (Author) , Habel, Antje (Author) , Hagenlocher, Christian (Author) , Mucha, Jana (Author) , Ackermann, Ulrike (Author) , Tessmer, Claudia (Author) , Meyer, Jochen (Author) , Capper, David (Author) , Moldenhauer, Gerhard (Author) , Mautner, Victor (Author) , Frappart, Pierre-Olivier (Author) , Schittenhelm, Jens (Author) , Hartmann, Christian (Author) , Hagel, Christian (Author) , Katenkamp, Kathrin (Author) , Petersen, Iver (Author) , Mechtersheimer, Gunhild (Author) , Deimling, Andreas von (Author)
Format: Article (Journal)
Language:English
Published: 25 January 2014
In: Acta neuropathologica
Year: 2014, Volume: 127, Issue: 4, Pages: 565-572
ISSN:1432-0533
DOI:10.1007/s00401-014-1246-6
Online Access:Resolving-System, lizenzpflichtig, Volltext: https://doi.org/10.1007/s00401-014-1246-6
Verlag, lizenzpflichtig, Volltext: https://link.springer.com/article/10.1007%2Fs00401-014-1246-6
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Author Notes:David E. Reuss, Antje Habel, Christian Hagenlocher, Jana Mucha, Ulrike Ackermann, Claudia Tessmer, Jochen Meyer, David Capper, Gerhard Moldenhauer, Victor Mautner, Pierre-Olivier Frappart, Jens Schittenhelm, Christian Hartmann, Christian Hagel, Kathrin Katenkamp, Iver Petersen, Gunhild Mechtersheimer, Andreas von Deimling
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Summary:Malignant peripheral nerve sheath tumors (MPNST) derive from the Schwann cell or perineurial cell lineage and occur either sporadically or in association with the tumor syndrome neurofibromatosis type 1 (NF1). MPNST often pose a diagnostic challenge due to their frequent lack of pathognomonic morphological or immunohistochemical features. Mutations in the NF1 tumor suppressor gene are found in all NF1-associated and many sporadic MPNST. The presence of NF1 mutation may have the potential to differentiate MPNST from several morphologically similar neoplasms; however, mutation detection is hampered by the size of the gene and the lack of mutational hot spots. Here we describe a newly developed monoclonal antibody binding to the C-terminus of neurofibromin (clone NFC) which was selected for optimal performance in routinely processed formalin-fixed and paraffin-embedded tissue.
Item Description:Gesehen am 19.08.2020
Physical Description:Online Resource
ISSN:1432-0533
DOI:10.1007/s00401-014-1246-6

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