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Pediatric and adult malignant peripheral nerve sheath tumors: an analysis of data from the surveillance, epidemiology, and end results program

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Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas that arise predominantly from Schwann cells. Despite the fact that MPNSTs have high local recurrence rates and are generally associated with poor prognosis, little is known about prognostic factors or effective clinical management for this tumor type. The purpose of this study was to describe the distributions of patient and tumor characteristics and to identify predictors of cause-specific survival among MPNST cases reported to SEER between 1973 and 2008. Patient and tumor characteristics were compared between pediatric and adult MPNST cases. Cox regression and tree-based survival analysis were used to examine factors associated with MPNST-related mortality separately among adults and children. A total of 1,315 MPNST cases were isolated from the 1973–2008 SEER dataset. Among pediatric cases, sex, race, and radiation therapy predicted MPNST survival, whereas among adults, tumor site, tumor grade, number of primary tumors, and tumor size were significant predictors. As tumor size at diagnosis/resection may be the only somewhat “modifiable” prognostic factor, future studies should aim to identify biological and social attributes associated with tumor size at diagnosis, separately among individuals with and without NF-1, in order to help identify earlier opportunities for clinical intervention.

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The authors have no conflicts of interest.

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Authors and Affiliations

  1. Department of Pediatrics & Dan L. Duncan Cancer Center, Baylor College of Medicine, One Baylor Plaza, Houston, TX, 77030, USA

    E. Susan Amirian & Michael E. Scheurer

  2. Department of Pathology & Immunology and Neurology, Baylor College of Medicine, Houston, TX, USA

    J. Clay Goodman

  3. Department of Neurosurgery, The Methodist Hospital Research Institute, Houston, TX, USA

    Pamela New

Authors
  1. E. Susan Amirian
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  2. J. Clay Goodman
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  3. Pamela New
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  4. Michael E. Scheurer
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Correspondence to Michael E. Scheurer.

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Amirian, E.S., Goodman, J.C., New, P. et al. Pediatric and adult malignant peripheral nerve sheath tumors: an analysis of data from the surveillance, epidemiology, and end results program. J Neurooncol 116, 609–616 (2014). https://doi.org/10.1007/s11060-013-1345-6

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  • DOI: https://doi.org/10.1007/s11060-013-1345-6

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