Journal of Neuro-Oncology

, Volume 139, Issue 1, pp 69–75 | Cite as

Racial/ethnic disparities and incidence of malignant peripheral nerve sheath tumors: results from the Surveillance, Epidemiology, and End Results Program, 2000–2014

  • Erin C. Peckham-Gregory
  • Roberto E. Montenegro
  • David A. Stevenson
  • David H. Viskochil
  • Michael E. Scheurer
  • Philip J. Lupo
  • Joshua D. Schiffman
Clinical Study



Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors, generally high-grade, and comprise ~ 5–10% of soft tissue sarcomas. Over two-thirds of MPNSTs metastasize, and upwards of 40% clinically recur. Etiologic risk factors for MPNSTs are historically understudied. There is evidence to suggest MPNST incidence differs across racial/ethnic groups in pediatric populations. Therefore, we sought to estimate differences in MPNST incidence by race/ethnicity among all ages in the United States.


Incidence data were obtained from the Surveillance, Epidemiology, and End Results (SEER-18) Program, 2000–2014. Race/ethnicity was categorized as: White; Black; Asian; Other; and Latino/a (“Spanish–Hispanic–Latino”). Latino/a included all races, while all other categories excluded those identified as Latino/a. Age-adjusted incidence rate ratios (IRR) and 95% confidence intervals (CIs) were generated in SEER-STAT (v8.3.4). We estimated incidence rates among all ages, and among those diagnosed < 25 and ≥ 25 years.


MPNST cases were abstracted from SEER-18 (n = 1047). Among all age groups, Blacks experienced an elevated incidence of MPNSTs compared to Whites (IRRBlacks = 1.26, 95% CI 1.04–1.50). Asian and Latinos/as experienced lower incidences compared to Whites (IRRAsians = 0.78, 95% CI 0.61–0.99; IRRLatinos/as = 0.84, 95% CI 0.69–1.02). In subgroup analyses, no statistically significant associations with MPNSTs were identified among cases diagnosed < 25 years of age, whereas the associations observed among all age groups were prominent among those diagnosed ≥ 25 years of age.


Incidence rates of MPNSTs were highest in Blacks compared to Whites and other minority groups. This study suggests specific patterns exist in terms of race/ethnicity and age at diagnosis of MPNSTs.


Malignant peripheral nerve sheath tumors Race/ethnicity Epidemiology SEER 



This research was supported by the National Institutes of Health (R25-CA160078 to E.C.P.-G. and M.E.S.), Alex’s Lemonade Stand Foundation Epidemiology Grant (to P.J.L.), the American Society of Hematology Scholar Award (to E.C.P.-G.), and the Thrasher Research Fund Early Career Award (to E.C.P.-G.). J.D.S. holds an Edward B. Clark, MD, Chair in Pediatric Research and is supported by the Primary Children’s Hospital (PCH) Pediatric Cancer Research Program through the PCH Foundation and the Intermountain Healthcare Foundation.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Erin C. Peckham-Gregory
    • 1
    • 2
  • Roberto E. Montenegro
    • 3
  • David A. Stevenson
    • 4
  • David H. Viskochil
    • 5
  • Michael E. Scheurer
    • 1
    • 2
  • Philip J. Lupo
    • 1
    • 2
  • Joshua D. Schiffman
    • 6
  1. 1.Department of Pediatrics, Section of Hematology-OncologyBaylor College of MedicineHoustonUSA
  2. 2.Texas Children’s Cancer CenterTexas Children’s HospitalHoustonUSA
  3. 3.Department of Psychiatry and Behavioral MedicineThe University of Washington School of MedicineSeattleUSA
  4. 4.Division of Medical GeneticsStanford UniversityStanfordUSA
  5. 5.Division of Medical GeneticsUniversity of Utah School of MedicineSalt Lake CityUSA
  6. 6.Department of Pediatrics and Department of Oncological SciencesHuntsman Cancer Institute, University of Utah School of MedicineSalt Lake CityUSA

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