survival in malignant peripheral nerve sheath tumours a comparison between sporadic and neurofibromatosis type 1-associated tumours生存在恶性周边神经鞘肿瘤比较零星,神经纤维瘤1-associated肿瘤类型.pdfVIP

Hindawi Publishing Corporation Sarcoma Volume 2009, Article ID 756395, 5 pages doi:10.1155/2009/756395 Clinical Study Survival in Malignant Peripheral Nerve Sheath Tumours: A Comparison between Sporadic and Neurofibromatosis Type 1-Associated Tumours D. E. Porter,1 V. Prasad,2 L. Foster,1 G. F. Dall,1 R. Birch,3 and R. J. Grimer2 1 Department of Orthopaedic Surgery, University of Edinburgh, Royal Infirmary of Edinburgh, Little France Crescent, Edinburgh EH16 4SU, UK 2 The Royal Orthopaedic Hospital, Bristol Road South, Northfield, Birmingham B31 2AP, UK 3 Peripheral Nerve Injuries Unit, Royal National Orthopaedic Hospital Trust, Brockley Hill Stanmore, Middlesex HA7 4LP, UK Correspondence should be addressed to G. F. Dall, gdall@ Received 2 October 2008; Accepted 5 January 2009 Recommended by Alessandro Gronchi We studied 123 patients with malignant peripheral nerve sheath tumours (MPNSTs) between 1979 and 2002. However, 90 occurred sporadically whereas 33 were associated with neurofibromatosis type 1 (NF1). Survival was calculated using Kaplan-Meier survival curves and we used Cox’s proportional hazards model to identify independent prognostic factors. A 5-year survival for 110 nonmetastatic patients was 54%; (33% NF1 and 63% sporadic P = .015). Tumour stage and site were significant prognostic indicators after univariate analysis. After multivariate analysis, however, only NF1 (P = .007) and tumour volume more than 200 m (P = .015) remained independent predictors of poor outcome. We recommend that NF1 be taken into account during MPNST staging. As the survival rate in the NF group was dependant on tumour volume, routine screening of these patients with FDG PET and/or MRI may be warranted, thereby staging and controlling the