Outcomes of Treatment for Malignant Peripheral Nerve Sheath Tumors: Different Clinical Features Associated with Neurofibromatosis Type 1.
- Author:
In Kyung HWANG
1
;
Seung Min HAHN
;
Hyo Sun KIM
;
Sang Kyum KIM
;
Hyo Song KIM
;
Kyoo Ho SHIN
;
Chang Ok SUH
;
Chuhl Joo LYU
;
Jung Woo HAN
Author Information
- Publication Type:Original Article
- Keywords: Sarcoma; Neurilemmoma; Neoplasms; Neurofibromatoses
- MeSH: Humans; Korea; Multivariate Analysis; Neoplasm Metastasis; Neurilemmoma*; Neurofibromatoses*; Neurofibromatosis 1*; Pathology; Sarcoma; Seoul
- From:Cancer Research and Treatment 2017;49(3):717-726
- CountryRepublic of Korea
- Language:English
- Abstract: 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.
