Annals of Surgical Oncology

, Volume 20, Issue 1, pp 66–72

Oncologic Outcomes of Sporadic, Neurofibromatosis-Associated, and Radiation-Induced Malignant Peripheral Nerve Sheath Tumors

  • Jennifer LaFemina
  • Li-Xuan Qin
  • Nicole H. Moraco
  • Cristina R. Antonescu
  • Ryan C. Fields
  • Aimee M. Crago
  • Murray F. Brennan
  • Samuel Singer
Bone and Soft Tissue Sarcomas

DOI: 10.1245/s10434-012-2573-2

Cite this article as:
LaFemina, J., Qin, LX., Moraco, N.H. et al. Ann Surg Oncol (2013) 20: 66. doi:10.1245/s10434-012-2573-2

Abstract

Background

Malignant peripheral nerve sheath tumors (MPNSTs) occur sporadically, after prior radiation therapy (RT), or in association with neurofibromatosis type 1 (NF1). It is controversial whether patients with NF1-associated MPNST have worse outcomes. We investigated the prognostic significance of sporadic, NF1-associated, and RT-induced MPNST.

Methods

Patients with primary high-grade MPNST from 1982 to 2011 were identified from a prospectively maintained database. Patients with sporadic MPNST were included only if the MPNST was not associated with NF1 or a neurofibroma or if it was immunohistochemically S100-positive.

Results

We studied 105 patients; 42 had NF1-associated tumors, 49 sporadic, and 14 RT-induced. Median age at diagnosis was 38 years. Median follow-up for surviving patients was 4 years. Mean tumor diameter was 5.5 cm for RT-induced tumors and 9.7 cm for NF1-associated and sporadic tumors (P = 0.004). In multivariate analysis, factors associated with worse disease-specific survival (DSS) were larger size (HR 1.08; 95 % CI 1.04–1.13; P < 0.001) and positive margin (HR 3.30; 95 % CI 1.74–6.28; P < 0.001). Age, gender, site of disease, and S100 staining were not associated with DSS. The 3-year and median DSS were similar for NF1 and sporadic cases; combined 3-year DSS was 64 % and median DSS was 8.0 years. For RT-induced tumors, 3-year DSS was 49 % and median DSS was 2.4 years. The relationship between RT association and DSS approached statistical significance (HR 2.29; 95 % CI 0.93–5.67; P = 0.072).

Conclusions

Margin status and size remain the most important predictors of DSS in patients with MPNST. NF1-associated and sporadic MPNSTs may be associated with improved DSS compared with RT-induced tumors.

Copyright information

© Society of Surgical Oncology 2012

Authors and Affiliations

  • Jennifer LaFemina
    • 1
  • Li-Xuan Qin
    • 2
  • Nicole H. Moraco
    • 1
  • Cristina R. Antonescu
    • 3
  • Ryan C. Fields
    • 1
  • Aimee M. Crago
    • 1
  • Murray F. Brennan
    • 1
  • Samuel Singer
    • 1
  1. 1.Department of SurgeryMemorial Sloan-Kettering Cancer CenterNew YorkUSA
  2. 2.Department of Epidemiology and BiostatisticsMemorial Sloan-Kettering Cancer CenterNew YorkUSA
  3. 3.Department of PathologyMemorial Sloan-Kettering Cancer CenterNew YorkUSA