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Outcomes of Treatment for Malignant Peripheral Nerve Sheath Tumors: Different Clinical Features Associated with Neurofibromatosis Type 1

 n Kyung Hwang  ;  Seung Min Hahn  ;  Hyo Sun Kim  ;  Sang Kyum Kim  ;  Hyo Song Kim  ;  Kyoo-Ho Shin  ;  Chang Ok Suh  ;  Chuhl Joo Lyu  ;  Jung Woo Han 
 Cancer Research and Treatment, Vol.49(3) : 717-726, 2017 
Journal Title
 Cancer Research and Treatment 
Issue Date
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Biopsy ; Child ; Combined Modality Therapy/adverse effects ; Combined Modality Therapy/methods ; Disease Management ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Grading ; Neoplasm Metastasis ; Neoplasm Staging ; Neurofibromatosis 1/diagnosis ; Neurofibromatosis 1/mortality* ; Neurofibromatosis 1/therapy* ; Peripheral Nervous System Neoplasms/diagnosis ; Peripheral Nervous System ; Neoplasms/mortality* ; Peripheral Nervous System Neoplasms/therapy* ; Practice Patterns, Physicians' ; Proportional Hazards Models ; Risk Factors ; Survival Analysis ; Treatment Outcome ; Tumor Burden ; Young Adult
Neoplasms ; Neurilemmoma ; Neurofibromatoses ; Sarcoma
PURPOSE: 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. MATERIALS AND 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. CONCLUSION: 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.
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1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Radiation Oncology (방사선종양학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Orthopedic Surgery (정형외과학교실) > 1. Journal Papers
Yonsei Authors
김상겸(Kim, Sang Kyum) ORCID logo https://orcid.org/0000-0003-0768-9923
김효선(Kim, Hyo Sun)
김효송(Kim, Hyo Song) ORCID logo https://orcid.org/0000-0002-0625-9828
서창옥(Suh, Chang Ok)
신규호(Shin, Kyoo Ho)
유철주(Lyu, Chuhl Joo) ORCID logo https://orcid.org/0000-0001-7124-7818
한승민(Hahn, Seung Min) ORCID logo https://orcid.org/0000-0001-9832-6380
한정우(Han, Jung Woo) ORCID logo https://orcid.org/0000-0001-8936-1205
황인경(Hwang, In Kyung)
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