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Pediatric choroid plexus tumors: epidemiology, treatments, and outcome analysis on 202 children from the SEER database

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Abstract

Choroid plexus papillomas (CPPs) and carcinomas (CPCs) are rare neoplasms that affect mostly children. Due to their rarity, their epidemiology and outcomes are incompletely understood. The National Cancer Institute’s Surveillance, Epidemiology and End Results (SEER) Program is a well-established population-based group of registries that collects and publishes cancer incidence and survival data representing approximately 28 % of the US population. SEER-STAT v8.1.2 was used to identify patients with ICD-O-3 codes for choroid plexus tumors in patients aged 0–19. Demographics, initial treatment, and follow-up data were collected. Statistical methods including Kaplan–Meier curves, log rank tests, and Cox proportional hazards regression were used to estimate associations between independent variables and survival. The SEER registries contained 107 CPPs (2004–2010) and 95 CPCs (1978–2010). Median follow-up was 38 and 40 months, respectively. More than 75 % of CPCs were diagnosed before the age of 5 years, versus 48 % for CPPs. Sixty-five percent of CPCs and 57 % of CPPs occurred in males. In both groups at least 90 % of children underwent surgical resection. Gross total resection (GTR) was achieved in 67.0 % of CPCs and 63.6 % of CPPs. Almost 17 % of CPCs were treated with radiation versus only 0.9 % of CPPs. More than 98 % of patients with CPP were alive at the last follow-up, versus 62 % of CPC patients. For CPC, surgery was significantly associated with increased overall survival, but contrary to previous reports, extent of surgical resection was not associated with survival. Age, sex, race, and radiation treatment also had no effect on survival. This report, using the SEER datasets, corroborates many findings of previous smaller studies on CPTs. CPC occurs in younger children, with a male predominance, and a much worse prognosis than CPP. As such, these tumors have been treated aggressively with high rates of GTR and radiation treatment. Despite these treatments, overall survival for CPC remains poor.

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Acknowledgments

Special thanks go to Judy Gault, PhD., for all her assistance in the final formatting and submission of this manuscript.

Funding

Grant UL1 TR001082 from NCATS/NIH.

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Authors and Affiliations

  1. Pediatric Neurosurgery, Children’s Hospital Colorado, University of Colorado Denver, 13123 E. 16th Ave, Box 330, Aurora, CO, 80045, USA

    Roy W. R. Dudley, Michael H. Handler & Todd C. Hankinson

  2. Children’s Outcomes Research Program, University of Colorado Denver, 13199 E. Montview Blvd, Suite 300, Aurora, CO, 80045, USA

    Michelle R. Torok, Danielle Gallegos & Todd C. Hankinson

  3. Department of Radiation Oncology, University of Colorado Denver, Anschutz Cancer Pavilion, 1665 Aurora Court, Suite 1032, Aurora, CO, 80045, USA

    Arthur K. Liu

Authors
  1. Roy W. R. Dudley
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  2. Michelle R. Torok
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  6. Todd C. Hankinson
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Correspondence to Todd C. Hankinson.

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Dudley, R.W.R., Torok, M.R., Gallegos, D. et al. Pediatric choroid plexus tumors: epidemiology, treatments, and outcome analysis on 202 children from the SEER database. J Neurooncol 121, 201–207 (2015). https://doi.org/10.1007/s11060-014-1628-6

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  • DOI: https://doi.org/10.1007/s11060-014-1628-6

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