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연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2017.7
- 수록면
- 717 - 726 (10page)
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초록· 키워드
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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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참고문헌 (30)
참고문헌 신청
Amirian ES / 2014 / Pediatric and adult malignant peripheral nerve sheath tumors : an analysis of data from the surveillance, epidemiology, and end results program / J Neurooncol 116:609~616
Tucker T / 2005 / Association between benign and malignant peripheral nerve sheath tumors in NF1 / Neurology 65:205~211
Ferner RE / 2002 / International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis / Cancer Res 62:1573~1577
Baehring JM / 2003 / Malignant peripheral nerve sheath tumor : the clinical spectrum and outcome of treatment / Neurology 61:696~698
Carli M / 2005 / Pediatric malignant peripheral nerve sheath tumor : the Italian and German soft tissue sarcoma cooperative group / J Clin Oncol 23:8422~8430
Tucker T / 2005 / Association between benign and malignant peripheral nerve sheath tumors in NF1 / Neurology 65:205~211
Ferner RE / 2002 / International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis / Cancer Res 62:1573~1577
Baehring JM / 2003 / Malignant peripheral nerve sheath tumor : the clinical spectrum and outcome of treatment / Neurology 61:696~698
Carli M / 2005 / Pediatric malignant peripheral nerve sheath tumor : the Italian and German soft tissue sarcoma cooperative group / J Clin Oncol 23:8422~8430
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