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SarcomaVolume 2008 2008, Article ID 849156, 7 pages

Research Article

INSERM U602, Hôpital Paul Brousse, 12 Avenue P. Vaillant Couturier, 94800 Villejuif, France

INSERM U590, Centre Léon Bérard, 28 rue Laënnec, 69373 Lyon, Cedex 8, France

INSERM UMR-S775, Université Paris Descartes, Centre Universitaire des Saints-Pères, 45 r des Saints-Pères, 75006 Paris, France

Institut Gustave-Roussy, 39 rue Camille Desmoulins, 94805 Villejuif Cedex, France

INSERM U745, Faculté des Sciences Pharmaceutiques et Biologiques, Université René Descartes, 4 Avenue de L'Observatoire, 75270 Paris, Cedex 6, France

Anatomie et Cytologie Pathologiques, Centre Jean Perrin, 39 rue Montalembert, 63000 Clermont-Ferrand, France

Hôpital Ambroise Paré, APHP and Faculté de médecine PIFO, UVSQ - 9, Avenue Charles de Gaulle, 92104 Boulogne, France

Received 22 October 2007; Revised 28 March 2008; Accepted 7 July 2008

Academic Editor: Beatrice Seddon

Copyright © 2008 Séverine Tabone-Eglinger et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Malignant peripheral nerve sheath tumours MPNSTs are highly malignant and resistant. Transformation might implicate up regulation of epidermal growth factor receptor EGFR. Fifty-two MPNST samples were studied for EGFR, Ki-67, p53, and survivin expression by immunohistochemistry and for EGFR amplification by in situ hybridization. Results were correlated with clinical data. EGFR RNA was also quantified by RT-PCR in 20 other MPNSTs and 14 dermal neurofibromas. Half of the patients had a neurofibromatosis type 1 NF1. EGFR expression, detected in 86% of MPNSTs, was more frequent in NF1 specimens and closely associated with high-grade and p53-positive areas. MPNSTs expressed more EGFR transcripts than neurofibromas. No amplification of EGFR locus was observed. NF1 status was the only prognostic factor in multivariate analysis, with median survivals of 18 and 43 months for patients with or without NF1. Finally, EGFR might become a new target for MPNSTs treatment, especially in NF1-associated MPNSTs.

Autor: Séverine Tabone-Eglinger, Radislav Bahleda, Jean-François Côté, Philippe Terrier, Dominique Vidaud, Anne Cayre, Alain Be



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