Cancer Res Treat.  2017 Jul;49(3):717-726. 10.4143/crt.2016.271.

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

Affiliations
  • 1Division of Pediatric Hemato-oncology, Department of Pediatrics, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea. jwhan@yuhs.ac
  • 2Department of Pediatric Hematology and Oncology, Yonsei Cancer Center, Yonsei University Health System, Seoul, Korea.
  • 3Department of Pathology, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea.
  • 4Division of Medical Oncology, Department of Internal Medicine, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea.
  • 5Department of Orthopedic Surgery, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea.
  • 6Department of Radiation Oncology, Yonsei University Health System, Yonsei University College of Medicine, Seoul, Korea.

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.

Keyword

Sarcoma; Neurilemmoma; Neoplasms; Neurofibromatoses

MeSH Terms

Humans
Korea
Multivariate Analysis
Neoplasm Metastasis
Neurilemmoma*
Neurofibromatoses*
Neurofibromatosis 1*
Pathology
Sarcoma
Seoul
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