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

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.

Prognosis and Risk Factors for Malignant Peripheral Nerve Sheath Tumor: A Systematic Review and Meta-analysis

“…When performing meta-analysis, we regard negative and positive as R0 and R2, respectively. Six studies (including subgroups) [13,15,22,24,31] MPNST is a signi cantly protective factor for OS (HR 0.70, 95%CI 0.59-0.83, p=0.000).…”

“…Ten studies [13,14,17,22,24,25,[29][30][31]38] evaluated tumor size (the large vs. the small) as a risk factor for OS. Values of I 2 = 44.4% and p= 0.063 were obtained after the HR values of OS were merged, indicating that heterogeneity existed.…”

“…The sensitivity analysis was performed in these groups. The pooled HR of age became statistical signi cance when to exclude one of these studies including Yuan, Z. N. et al [14], Hwang, I. K. et al [31], Fan, Q. et al [16], and LaFemina, J. et al [23]. The pooled HR of tumor site (Trunk vs. Extremity) became no statistical signi cance when to exclude one of these studies including Stucky, C. C. et al [34], Longhi, A. et al [17], and Anghileri, M. et al [18].…”

“…Kolberg, M et al [45] performed a meta-analysis in 2013 and indicated that NF 1 status had no effect on survival. Some studies [7,18,28,31,34] just identi ed a trend and reported the negative results based on large populations. With the improvement of survival for the NF1 patients in past years, they considered that the survival difference was diminishing.…”

Benign-appearing intra-spinal malignant peripheral nerve sheath tumors (mpnsts): treatments and outcomes of 14 consecutive patients

“…The incidence of MPNSTs in the general population is 1:100,000. Up to 50% of these tumors occur in patients with neurofibromatosis Type 1 (NF1)(3%-5%) (11,16), and 10% of them have a previous history of radiation therapy (12,14,15). The most common locations for MPNSTs are the trunk, limbs, head and neck (6).…”