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

• Published in: Cancer research and treatment 2017, 49(3), , pp.717-726
• Main Authors: Hwang, In Kyung, Hahn, Seung Min, Kim, Hyo Sun, Kim, Sang Kyum, Kim, Hyo Song, Shin, Kyoo-Ho, Suh, Chang Ok, Lyu, Chuhl Joo, Han, Jung Woo
• Format: Article
• Language: English
• Subjects: 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; Original; 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; 의학일반
• ISSN: 1598-2998; 2005-9256
• DOI: 10.4143/crt.2016.271

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. 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. 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. 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.