title: Outcomes of Treatment for Malignant Peripheral Nerve Sheath Tumors: Different Clinical Features Associated with Neurofibromatosis Type 1.
abstract: OBJECTIVE
  Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of sarcoma that occur spontaneously or in association with neurofibromatosis type 1 (NF-1). This study aimed to clinically differentiate these types of MPNSTs.
  
  
  METHODS
  The study reviewed 95 patients diagnosed with and treated for MPNST at Yonsei University Health System, Seoul, Korea over a 27-year period. The clinical characteristics, prognostic factors, and treatment outcomes of sporadic MPNST (sMPNST) and NF-1 associated MPNST (NF-MPNST) cases were compared.
  
  
  RESULTS
  Patients with NF-MPNST had a significantly lower median age (32 years vs. 45 years for sMPNST, p=0.012), significantly larger median tumor size (8.2 cm vs. 5.0 cm for sMPNST, p < 0.001), and significantly larger numbers of imaging studies and surgeries (p=0.004 and p < 0.001, respectively). The 10-year overall survival (OS) rate of the patients with MPNST was 52±6%. Among the patients with localized MPNST, patients with NF-MPNST had a significantly lower 10-year OS rate (45±11% vs. 60±8% for sMPNST, p=0.046). Univariate analysis revealed the resection margin, pathology grade, and metastasis to be significant factors affecting the OS (p=0.001, p=0.020, and p < 0.001, respectively). Multivariate analysis of the patients with localized MPNST identified R2 resection and G1 as significant prognostic factors for OS.
  
  
  CONCLUSIONS
  NF-MPNST has different clinical features from sMPNST and requires more careful management. Further study will be needed to develop specific management plans for NF-MPNST.
authors.affiliation: Division of Pediatric Hemato-oncology, Department of Pediatrics, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea.|Department of Pediatric Hematology and Oncology, Yonsei Cancer Center, Yonsei University Health System, Seoul, Korea.
authors.forename: In Kyung
authors.initials: IK
authors.lastname: Hwang
authors.affiliation: Division of Pediatric Hemato-oncology, Department of Pediatrics, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea.|Department of Pediatric Hematology and Oncology, Yonsei Cancer Center, Yonsei University Health System, Seoul, Korea.
authors.forename: Seung Min
authors.initials: SM
authors.lastname: Hahn
authors.affiliation: Division of Pediatric Hemato-oncology, Department of Pediatrics, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea.|Department of Pediatric Hematology and Oncology, Yonsei Cancer Center, Yonsei University Health System, Seoul, Korea.
authors.forename: Hyo Sun
authors.initials: HS
authors.lastname: Kim
authors.affiliation: Department of Pathology, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Sang Kyum
authors.initials: SK
authors.lastname: Kim
authors.affiliation: Division of Medical Oncology, Department of Internal Medicine, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Hyo Song
authors.initials: HS
authors.lastname: Kim
authors.affiliation: Department of Orthopedic Surgery, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Kyoo-Ho
authors.initials: KH
authors.lastname: Shin
authors.affiliation: Department of Radiation Oncology, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Chang Ok
authors.initials: CO
authors.lastname: Suh
authors.affiliation: Division of Pediatric Hemato-oncology, Department of Pediatrics, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea.|Department of Pediatric Hematology and Oncology, Yonsei Cancer Center, Yonsei University Health System, Seoul, Korea.
authors.forename: Chuhl Joo
authors.initials: CJ
authors.lastname: Lyu
authors.affiliation: Division of Pediatric Hemato-oncology, Department of Pediatrics, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea.|Department of Pediatric Hematology and Oncology, Yonsei Cancer Center, Yonsei University Health System, Seoul, Korea.
authors.forename: Jung Woo
authors.initials: JW
authors.lastname: Han
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002648:Child; D003131:Combined Modality Therapy / Q000009:adverse effects / Q000379:methods; D019468:Disease Management; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D060787:Neoplasm Grading; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D009456:Neurofibromatosis 1 / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy*; D010524:Peripheral Nervous System Neoplasms / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy*; D010818:Practice Patterns, Physicians'; D016016:Proportional Hazards Models; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome; D047368:Tumor Burden; D055815:Young Adult
keywords: Neoplasms; Neurilemmoma; Neurofibromatoses; Sarcoma