Skip to main content

Advertisement

SpringerLink
Log in

Characteristics and overall survival in pediatric versus adult skull base chordoma: a population-based study

  • Original Article
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

Less than 5% of chordomas occur in pediatric patients. While many studies have explored the treatment and outcomes of skull base chordomas, few have focused on the differences between pediatric and adult populations. The aim of this study is to analyze the epidemiological variables and clinical outcomes between pediatric and adult skull base chordomas using a large-sample, population-based cancer database.

Methods

The National Cancer Database was queried between 2004 and 2015 for skull base chordomas. We stratified patients as pediatric (<18 years) and adults (≥18 years). We compared several clinical covariates between the two groups.

Results

Our cohort consisted of 658 patients, 61 pediatric (9.3%), and 597 adults (90.7%). Pediatric patients were more likely to have larger tumor size (41.4 ± 15.7 mm versus 34.1 ± 15.8 mm, p < 0.01) and universally treated at academic facilities. There was no significant difference in overall survival.

Conclusions

Pediatric skull base chordomas are rare tumors that are managed with aggressive surgical resection, followed by radiation. While there may be difference between tumor presentation, outcomes between pediatric and adult patients are similar.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

Data availability

National Cancer Database (NCDB) is public.

References

  1. Sahyouni R, Goshtasbi K, Mahmoodi A, Chen JW (Apr 2018) A historical recount of chordoma. J Neurosurg Spine 28(4):422–428. https://doi.org/10.3171/2017.7.SPINE17668

    Article  PubMed  Google Scholar 

  2. Salisbury JR (1993) The pathology of the human notochord. J Pathol Dec 171(4):253–255. https://doi.org/10.1002/path.1711710404

    Article  CAS  Google Scholar 

  3. Heffelfinger MJDD, MacCarty CS, Beabout JW (1973) Chordomas and cartilaginous tumors at the skull base. Cancer. 32(2):410–420. https://doi.org/10.1002/1097-0142(197308)32:2

    Article  CAS  PubMed  Google Scholar 

  4. McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM (Jan 2001) Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control 12(1):1–11. https://doi.org/10.1023/a:1008947301735

    Article  CAS  PubMed  Google Scholar 

  5. Al-Mefty O, Borba LAB (1997) Skull base chordomas: a management challenge. J Neurosurg 86(2):182–189. https://doi.org/10.3171/jns.1997.86.2.0182

    Article  CAS  PubMed  Google Scholar 

  6. Lanzino G, Dumont AS, Lopes MB, Laws ER, Jr. Skull base chordomas: overview of disease, management options, and outcome. Neurosurg Focus. Mar 15 2001;10(3):E12. doi:https://doi.org/10.3171/foc.2001.10.3.13

  7. Borba LAB, Al-Mefty O, Mrak RE, Suen J (1996) Cranial chordomas in children and adolescents. J Neurosurg 84(4):584–591. https://doi.org/10.3171/jns.1996.84.4.0584

    Article  CAS  PubMed  Google Scholar 

  8. Lau CS, Mahendraraj K, Ward A, Chamberlain RS (2016) Pediatric chordomas: a population-based clinical outcome study involving 86 patients from the Surveillance, Epidemiology, and End Result (SEER) database (1973-2011). Pediatr Neurosurg 51(3):127–136. https://doi.org/10.1159/000442990

    Article  PubMed  Google Scholar 

  9. Hulou MM, Garcia CR, Slone SA, Dugan A, Lei F, Huang B, Pittman T, Villano JL (Sep 2019) Comprehensive review of cranial chordomas using national databases in the USA. Clin Oncol (R Coll Radiol) 31(9):e149–e159. https://doi.org/10.1016/j.clon.2019.06.004

    Article  CAS  Google Scholar 

  10. Habrand JL, Datchary J, Bolle S, Beaudré A, de Marzi L, Beccaria K, Stefan D, Grill J, Dendale R (Jan-Feb 2016) Chordoma in children: case-report and review of literature. Rep Pract Oncol Radiother 21(1):1–7. https://doi.org/10.1016/j.rpor.2015.10.007

    Article  PubMed  Google Scholar 

  11. Ridenour RV 3rd, Ahrens WA, Folpe AL, Miller DV (Jan-Feb 2010) Clinical and histopathologic features of chordomas in children and young adults. Pediatr Dev Pathol 13(1):9–17. https://doi.org/10.2350/09-01-0584.1

    Article  PubMed  Google Scholar 

  12. Sebro R, DeLaney T, Hornicek F et al (2016) Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas. BMC Med Imaging 16(1):53. https://doi.org/10.1186/s12880-016-0149-5

    Article  PubMed  PubMed Central  Google Scholar 

  13. Boffa DJ, Rosen JE, Mallin K et al (2017) Using the National Cancer Database for outcomes research: a review. JAMA Oncol 3(12):1722–1728. https://doi.org/10.1001/jamaoncol.2016.6905

    Article  PubMed  Google Scholar 

  14. Palma DA (2017) National Cancer Data Base: an important research tool, but not population-based. J Clin Oncol 35(5):571. https://doi.org/10.1200/JCO.2016.69.2855

    Article  PubMed  Google Scholar 

  15. Bakker SH, Jacobs WCH, Pondaag W, Gelderblom H, Nout RA, Dijkstra PDS, Peul WC, Vleggeert-Lankamp CLA (Dec 2018) Chordoma: a systematic review of the epidemiology and clinical prognostic factors predicting progression-free and overall survival. Eur Spine J 27(12):3043–3058. https://doi.org/10.1007/s00586-018-5764-0

    Article  CAS  PubMed  Google Scholar 

  16. Hoch BL, Nielsen GP, Liebsch NJ, Rosenberg AE (Jul 2006) Base of skull chordomas in children and adolescents: a clinicopathologic study of 73 cases. Am J Surg Pathol 30(7):811–818. https://doi.org/10.1097/01.pas.0000209828.39477.ab

    Article  PubMed  Google Scholar 

  17. Dasenbrock HH, Liu KX, Devine CA, Chavakula V, Smith TR, Gormley WB, Dunn IF (Dec 2015) Length of hospital stay after craniotomy for tumor: a National Surgical Quality Improvement Program analysis. Neurosurg Focus 39(6):E12. https://doi.org/10.3171/2015.10.FOCUS15386

    Article  PubMed  Google Scholar 

  18. Albright AL, Pollack IF, Adelson PD, Solot JJ (Jul 1999) Outcome data and analysis in pediatric neurosurgery. Neurosurgery. 45(1):101–106. https://doi.org/10.1097/00006123-199907000-00025

    Article  CAS  PubMed  Google Scholar 

  19. Hayhurst C, Williams D, Yousaf J, Richardson D, Pizer B, Mallucci C (May 2013) Skull base surgery for tumors in children: long-term clinical and functional outcome. J Neurosurg Pediatr 11(5):496–503. https://doi.org/10.3171/2013.1.PEDS12120

    Article  PubMed  Google Scholar 

  20. Kuan EC, Badran KW, Yoo F, Bhandarkar ND, Haidar YM, Tjoa T, Armstrong WB, Palmer JN, Adappa ND, Wang MB, John MAS (Jun 2019) Predictors of short-term morbidity and mortality in open anterior skull base surgery. Laryngoscope. 129(6):1407–1412. https://doi.org/10.1002/lary.27494

    Article  PubMed  Google Scholar 

  21. Wang L, Wu Z, Tian K, Wang K, Li D, Ma J, Jia G, Zhang L, Zhang J (2017) Clinical features and surgical outcomes of patients with skull base chordoma: a retrospective analysis of 238 patients. J Neurosurg Dec 127(6):1257–1267. https://doi.org/10.3171/2016.9.JNS16559

    Article  Google Scholar 

  22. Jian BJ, Bloch OG, Yang I, Han SJ, Aranda D, Parsa AT (Aug 2011) A comprehensive analysis of intracranial chordoma and survival: a systematic review. Br J Neurosurg 25(4):446–453. https://doi.org/10.3109/02688697.2010.546896

    Article  PubMed  Google Scholar 

  23. Chambers KJ, Lin DT, Meier J, Remenschneider A, Herr M, Gray ST (May 2014) Incidence and survival patterns of cranial chordoma in the United States. Laryngoscope. 124(5):1097–1102. https://doi.org/10.1002/lary.24420

    Article  PubMed  Google Scholar 

  24. Abiri A, Abiri P, Goshtasbi K, Lehrich BM, Sahyouni R, Hsu FPK, Cadena G, Kuan EC (Jul 2020) Endoscopic anterior skull base reconstruction: a meta-analysis and systematic review of graft type. World Neurosurg 139:460–470. https://doi.org/10.1016/j.wneu.2020.04.089

    Article  PubMed  Google Scholar 

  25. Kamat A, Lee JY, Goldstein GH et al (May 2015) Reconstructive challenges in the extended endoscopic transclival approach. J Laryngol Otol 129(5):468–472. https://doi.org/10.1017/S0022215115000420

    Article  CAS  PubMed  Google Scholar 

  26. Maio S, Temkin N, Ramanathan D, Sekhar LN (2011) Current comprehensive management of cranial base chordomas: 10-year meta-analysis of observational studies. J Neurosurg. Dec 115(6):1094–1105. https://doi.org/10.3171/2011.7.JNS11355

    Article  Google Scholar 

  27. Hall MD, Bradley JA, Rotondo RL et al (2018) Risk of radiation vasculopathy and stroke in pediatric patients treated with proton therapy for brain and skull base tumors. Int J Radiat Oncol Biol Phys 101(4):854–859. https://doi.org/10.1016/j.ijrobp.2018.03.027

    Article  PubMed  Google Scholar 

  28. Kuan EC, Kaufman AC, Lerner D, Kohanski MA, Tong CCL, Tajudeen BA, Parasher AK, Lee JYK, Storm PB, Palmer JN, Adappa ND (Apr 2019) Lack of sphenoid pneumatization does not affect endoscopic endonasal pediatric skull base surgery outcomes. Laryngoscope. 129(4):832–836. https://doi.org/10.1002/lary.27600

    Article  PubMed  Google Scholar 

  29. Stapleton AL, Tyler-Kabara EC, Gardner PA, Snyderman CH, Wang EW (Feb 2017) Risk factors for cerebrospinal fluid leak in pediatric patients undergoing endoscopic endonasal skull base surgery. Int J Pediatr Otorhinolaryngol 93:163–166. https://doi.org/10.1016/j.ijporl.2016.12.019

    Article  PubMed  Google Scholar 

  30. Ghosh A, Hatten K, Learned KO, Rizzi MD, Lee JY, Storm PB, Palmer JN, Adappa ND (Nov 2015) Pediatric nasoseptal flap reconstruction for suprasellar approaches. Laryngoscope. 125(11):2451–2456. https://doi.org/10.1002/lary.25395

    Article  PubMed  Google Scholar 

  31. Quon JL, Kim LH, Hwang PH, Patel ZM, Grant GA, Cheshier SH, Edwards MSB (2019) Transnasal endoscopic approach for pediatric skull base lesions: a case series. J Neurosurg Pediatr 14:1–12. https://doi.org/10.3171/2019.4.PEDS18693

    Article  Google Scholar 

  32. Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ (Feb 2012) Chordoma: current concepts, management, and future directions. Lancet Oncol 13(2):e69–e76. https://doi.org/10.1016/S1470-2045(11)70337-0

    Article  PubMed  Google Scholar 

  33. Stacchiotti S, Casali PG (Aug 2011) Systemic therapy options for unresectable and metastatic chordomas. Curr Oncol Rep 13(4):323–330. https://doi.org/10.1007/s11912-011-0176-x

    Article  PubMed  Google Scholar 

  34. Zenonos GA, Fernandez-Miranda JC, Mukherjee D, Chang YF, Panayidou K, Snyderman CH, Wang EW, Seethala RR, Gardner PA (2018) Prospective validation of a molecular prognostication panel for clival chordoma. J Neurosurg Jun 1:1–10. https://doi.org/10.3171/2018.3.JNS172321

    Article  Google Scholar 

  35. Goshtasbi K, Lehrich BM, Abouzari M, Abiri A, Birkenbeuel J, Lan MY, Wang WH, Cadena G, Hsu FPK, Kuan EC (2020) Endoscopic versus nonendoscopic surgery for resection of pituitary adenomas: a national database study. J Neurosurg Mar 13:1–9. https://doi.org/10.3171/2020.1.JNS193062

    Article  Google Scholar 

Download references

Funding

Research reported in this publication was in part supported by the National Institute of General Medical Sciences of the National Institutes of Health under Award Number T32GM008208 to BML.

Author information

Authors and Affiliations

  1. Department of Neurosurgery, University of California, Irvine, CA, USA

    Jordan C. Xu, Brendan M. Fong, Frank P. K. Hsu & Edward C. Kuan

  2. Medical Scientist Training Program, University of Pittsburgh and Carnegie Mellon University, Pittsburgh, PA, USA

    Brandon M. Lehrich

  3. Department of Otolaryngology-Head and Neck Surgery, University of California, Irvine, Orange, CA, USA

    Tyler M. Yasaka & Edward C. Kuan

Authors
  1. Jordan C. Xu
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Brandon M. Lehrich
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Tyler M. Yasaka
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Brendan M. Fong
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Frank P. K. Hsu
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Edward C. Kuan
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

All authors contributed to the analysis of the results, writing, editing, and final review of the manuscript.

Corresponding author

Correspondence to Edward C. Kuan.

Ethics declarations

Conflict of interest

N/A

Disclaimer

The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.

Code availability

N/A

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

No portion of this paper has been previously presented nor published.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Xu, J.C., Lehrich, B.M., Yasaka, T.M. et al. Characteristics and overall survival in pediatric versus adult skull base chordoma: a population-based study. Childs Nerv Syst 37, 1901–1908 (2021). https://doi.org/10.1007/s00381-021-05046-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-021-05046-6

Keywords

Search

Navigation