TY - JOUR AU1 - Lev, Dina AB - ORIGINAL ARTICLES Clinical, Pathological, and Molecular Variables Predictive of Malignant Peripheral Nerve Sheath Tumor Outcome Changye Zou, MD,* Kerrington D. Smith, MD,* Jun Liu, MS,† Guy Lahat, MD,* Sarah Myers, BS,‡ Wei-Lien Wang, MD,§ Wei Zhang, PhD,§ Ian E. McCutcheon, MD,¶ John M. Slopis, MD, Alexander J. Lazar, MD, PhD,§ Raphael E. Pollock, MD, PhD,* and Dina Lev, MD† incidence of MPNST in NF1 patients may be as high as 8% to 12%. Objective: Improved staging systems for malignant peripheral nerve sheath MPNSTs behave aggressively, with a high rate of local recurrence tumor (MPNST) prognostication and management are needed. Conse- and a significant propensity to metastasize. Surgical resection represents quently, we sought to identify clinical, pathologic, and molecular predictors the mainstay of therapy. The benefit of radiation and systemic chemo- of outcome in patients with/without neurofibromatosis type 1 (NF-1) asso- therapy, when commonly administered, is undetermined; despite ag- ciated MPNST. gressive combined modality therapy 5 year survival rates range from Methods: MPNST patients treated from 1986 to 2006 (n  140) were 4–6 35% to 50%. identified; 72 had NF-1 syndrome and 68 did not. A comprehensive database Staging systems that can prospectively stratify and accurately was constructed. Paraffin-embedded TI - Clinical, Pathological, and Molecular Variables Predictive of Malignant Peripheral Nerve Sheath Tumor Outcome JF - Annals of Surgery DO - 10.1097/SLA.0b013e3181a77e9a DA - 2009-06-01 UR - https://www.deepdyve.com/lp/wolters-kluwer-health/clinical-pathological-and-molecular-variables-predictive-of-malignant-a35mcIvsCH SP - 1014 EP - 1022 VL - 249 IS - 6 DP - DeepDyve ER -