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

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Abstract

Background

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.

Methods

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.

Results

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.

Conclusions

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.

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Acknowledgements

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.

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Author notes

  1. Michael E. Scheurer, Philip J. Lupo and Joshua D. Schiffman have contributed equally to this work.

Authors and Affiliations

  1. Department of Pediatrics, Section of Hematology-Oncology, Baylor College of Medicine, One Baylor Plaza, MS: BCM305, Houston, TX, 77030, USA

    Erin C. Peckham-Gregory, Michael E. Scheurer & Philip J. Lupo

  2. Texas Children’s Cancer Center, Texas Children’s Hospital, Feigin Center, 1102 Bates St, Houston, TX, 77030, USA

    Erin C. Peckham-Gregory, Michael E. Scheurer & Philip J. Lupo

  3. Department of Psychiatry and Behavioral Medicine, The University of Washington School of Medicine, 4800 Sand Point Way NE, Seattle, WA, 98105, USA

    Roberto E. Montenegro

  4. Division of Medical Genetics, Stanford University, 300 Pasteur Drive, Boswell Building A097, Stanford, CA, 94304, USA

    David A. Stevenson

  5. Division of Medical Genetics, University of Utah School of Medicine, 295 Chipeta Way, Salt Lake City, UT, 84108, USA

    David H. Viskochil

  6. Department of Pediatrics and Department of Oncological Sciences, Huntsman Cancer Institute, University of Utah School of Medicine, 2000 Circle of Hope, Salt Lake City, UT, 84112, USA

    Joshua D. Schiffman

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  1. Erin C. Peckham-Gregory
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Correspondence to Joshua D. Schiffman.

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Peckham-Gregory, E.C., Montenegro, R.E., Stevenson, D.A. et al. Racial/ethnic disparities and incidence of malignant peripheral nerve sheath tumors: results from the Surveillance, Epidemiology, and End Results Program, 2000–2014. J Neurooncol 139, 69–75 (2018). https://doi.org/10.1007/s11060-018-2842-4

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