Tabone-Eglinger Séverine, Bahleda Radislav, Côté Jean-François, Terrier Philippe, Vidaud Dominique, Cayre Anne, Beauchet Alain, Théou-Anton Nathalie, Terrier-Lacombe Marie-José, Lemoine Antoinette, Penault-Llorca Frédérique, Le Cesne Axel, Emile Jean-François
INSERM U602, Hôpital Paul Brousse, 12 Avenue P. Vaillant Couturier, 94800 Villejuif, France.
Sarcoma. 2008;2008:849156. doi: 10.1155/2008/849156.
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.
恶性外周神经鞘瘤（MPNSTs）具有高度恶性且耐药。其转变可能与表皮生长因子受体（EGFR）的上调有关。通过免疫组织化学研究了52例MPNST样本的EGFR、Ki-67、p53和存活素表达，并通过原位杂交检测EGFR扩增。结果与临床数据相关。还通过RT-PCR对另外20例MPNST和14例皮肤神经纤维瘤中的EGFR RNA进行了定量。一半的患者患有1型神经纤维瘤病（NF1）。在86%的MPNST中检测到EGFR表达，在NF1标本中更常见，且与高级别和p53阳性区域密切相关。MPNST表达的EGFR转录本比神经纤维瘤更多。未观察到EGFR基因座的扩增。在多变量分析中，NF1状态是唯一的预后因素，有或无NF1的患者中位生存期分别为18个月和43个月。最后，EGFR可能成为MPNST治疗的新靶点，尤其是在与NF1相关的MPNST中。
