Skip to main content

Advertisement

SpringerLink
Log in

Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management

  • Clinical Study
  • Published:
Journal of Neuro-Oncology Aims and scope Submit manuscript

Abstract

Choroid plexus tumors (CPTs) are rare neoplasms of the central nervous system whose optimal management is not well defined. The Surveillance Epidemiology and End Results (SEER) Database from 1978 to 2009 was queried to define population-based outcomes for all patients with CPTs. Patient demographic data, histological classification (choroid plexus papilloma [CPP], atypical CPP [aCPP], and choroid plexus carcinoma [CPC]), extent of surgery, and use of radiation therapy (RT) as part of an initial course of therapy were analyzed for impact on overall survival (OS) and cause-specific survival (CSS). Chemotherapy data were not available within the SEER database. A total of 349 patients with CPTs were identified (120 CPCs, 26 aCPPs, and 203 CPPs). Patients with CPC presented at a younger age (median 3, mean 14.8 years) relative to CPP (median 25, mean 28.4 years; p < 0.0001). Histology was a significant predictor of OS, with 5-year OS rates of 90, 77, and 58 % for CPP, aCPP, and CPC, respectively. Older age and male sex were prognostic for worse OS and CSS for CPP. Only extent of surgery had a significant impact on survival for CPC. The use of adjuvant RT in patients with CPC undergoing surgery was not associated with a significantly improved OS (p = 0.17). For patients undergoing GTR without RT as part of an initial course of therapy, estimated 5- and 10-year OS were 70 % (±7 %) and 67 % (±8 %), respectively. Prospective data are required to define the optimal combination of surgery with adjuvant therapies, including chemotherapy.

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

References

  1. Bettegowda C, Adogwa O, Mehta V, Chaichana KL, Weingart J, Carson BS, Jallo GI, Ahn ES (2012) Treatment of choroid plexus tumors: a 20-year single institutional experience. J Neurosurg Pediatr 10:398–405

    Article  PubMed  Google Scholar 

  2. Asai A, Hoffman HJ, Hendrick EB, Humphreys RP, Becker LE (1989) Primary intracranial neoplasms in the first year of life. Child’s Nerv Syst 5:230–233

    Article  CAS  Google Scholar 

  3. Jooma R, Hayward RD, Grant DN (1984) Intracranial neoplasms during the first year of life: analysis of one hundred consecutive cases. Neurosurgery 14:31–41

    Article  CAS  PubMed  Google Scholar 

  4. Lafay-Cousin L, Keene D, Carret A-S et al (2011) Choroid plexus tumors in children less than 36 months: the Canadian Pediatric Brain Tumor Consortium (CPBTC) experience. Child’s Nerv Syst 27:259–264

    Article  Google Scholar 

  5. Emerich DF, Skinner SJM, Borlongan CV, Vasconcellos AV, Thanos CG (2005) The choroid plexus in the rise, fall and repair of the brain. BioEssays 27:262–274

    Article  CAS  PubMed  Google Scholar 

  6. Wolburg H, Paulus W (2010) Choroid plexus: biology and pathology. Acta Neuropathol 119:75–88

    Article  PubMed  Google Scholar 

  7. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114:97–109

    Article  PubMed  PubMed Central  Google Scholar 

  8. Lozier AP, Arbaje YM, Scheithauer BW (2009) Supratentorial, extraventricular choroid plexus carcinoma in an adult: case report. Neurosurgery 65:E816–E817

    Article  PubMed  Google Scholar 

  9. Wolff JEA, Sajedi M, Brant R, Coppes MJ, Egeler RM (2002) Choroid plexus tumours. Br J Cancer 87:1086–1091

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. McCall T, Binning M, Blumenthal DT, Jensen RL (2006) Variations of disseminated choroid plexus papilloma: 2 case reports and a review of the literature. Surg Neurol 66:62–67 (discussion 67–8)

    Article  PubMed  Google Scholar 

  11. Wrede B, Hasselblatt M, Peters O et al (2009) Atypical choroid plexus papilloma: clinical experience in the CPT-SIOP-2000 study. J Neurooncol 95:383–392

    Article  CAS  PubMed  Google Scholar 

  12. Wrede B, Liu P, Wolff JEA (2007) Chemotherapy improves the survival of patients with choroid plexus carcinoma: a meta-analysis of individual cases with choroid plexus tumors. J Neurooncol 85:345–351

    Article  PubMed  Google Scholar 

  13. Lafay-Cousin L, Mabbott DJ, Halliday W, Taylor MD, Tabori U, Kamaly-Asl ID, Kulkarni AV, Bartels U, Greenberg M, Bouffet E (2010) Use of ifosfamide, carboplatin, and etoposide chemotherapy in choroid plexus carcinoma. J Neurosurg Pediatr 5:615–621

    Article  PubMed  Google Scholar 

  14. McEvoy AW, Harding BN, Phipps KP, Ellison DW, Elsmore AJ, Thompson D, Harkness W, Hayward RD (2000) Management of choroid plexus tumours in children: 20 years experience at a single neurosurgical centre. Pediatr Neurosurg 32:192–199

    Article  CAS  PubMed  Google Scholar 

  15. Chow E, Reardon DA, Shah AB, Jenkins JJ, Langston J, Heideman RL, Sanford RA, Kun LE, Merchant TE (1999) Pediatric choroid plexus neoplasms. Int J Radiat Oncol Biol Phys 44:249–254

    Article  CAS  PubMed  Google Scholar 

  16. Pencalet P, Sainte-Rose C, Lellouch-Tubiana A et al (1998) Papillomas and carcinomas of the choroid plexus in children. J Neurosurg 88:521–528

    Article  CAS  PubMed  Google Scholar 

  17. Duffner PK, Kun LE, Burger PC, Horowitz ME, Cohen ME, Sanford RA, Krischer JP, Mulhern RK, James HE, Rekate HL (1995) Postoperative chemotherapy and delayed radiation in infants and very young children with choroid plexus carcinomas. The Pediatric Oncology Group. Pediatr Neurosurg 22:189–196

    Article  CAS  PubMed  Google Scholar 

  18. Berger C, Thiesse P, Lellouch-Tubiana A, Kalifa C, Pierre-Kahn A, Bouffet E (1998) Choroid plexus carcinomas in childhood: clinical features and prognostic factors. Neurosurgery 42:470–475

    Article  CAS  PubMed  Google Scholar 

  19. Packer RJ, Perilongo G, Johnson D, Sutton LN, Vezina G, Zimmerman RA, Ryan J, Reaman G, Schut L (1992) Choroid plexus carcinoma of childhood. Cancer 69:580–585

    Article  CAS  PubMed  Google Scholar 

  20. ClinicalTrials.gov (2013) Treatment of tumors of the choroid plexus epithelium. http://clinicaltrials.gov/ct2/show/study/NCT00500890. Accessed 19 Apr 2013

  21. Wolff JE, Sajedi M, Coppes MJ, Anderson RA, Egeler RM (1999) Radiation therapy and survival in choroid plexus carcinoma. Lancet 353:2126

    Article  CAS  PubMed  Google Scholar 

  22. Fitzpatrick LK, Aronson LJ, Cohen KJ (2002) Is there a requirement for adjuvant therapy for choroid plexus carcinoma that has been completely resected? J Neurooncol 57:123–126

    Article  PubMed  Google Scholar 

  23. Tabori U, Shlien A, Baskin B et al (2010) TP53 alterations determine clinical subgroups and survival of patients with choroid plexus tumors. J Clin Oncol 28:1995–2001

    Article  CAS  PubMed  Google Scholar 

  24. Surveillance, Epidemiology and ERP (2013) Overview of the SEER Program. http://seer.cancer.gov/about/overview.html. Accessed 23 Oct 2013

  25. Surveillance, Epidemiology and ERP (1984) Code Manual: the SEER Program. Bethesda, MD

  26. Surveillance, Epidemiology and ERP (1988) The SEER Program Code Manual. Bethesda, MD

  27. Krishnan S, Brown PD, Scheithauer BW, Ebersold MJ, Hammack JE, Buckner JC (2004) Choroid plexus papillomas: a single institutional experience. J Neurooncol 68:49–55

    Article  PubMed  Google Scholar 

  28. Park HS, Gross CP, Makarov DV, Yu JB (2012) Immortal time bias: a frequently unrecognized threat to validity in the evaluation of postoperative radiotherapy. Int J Radiat Oncol Biol Phys 83:1365–1373

    Article  PubMed  Google Scholar 

  29. Mosleh O, Tabori U, Bartels U, Huang A, Schechter T, Bouffet E (2013) Successful treatment of a recurrent choroid plexus carcinoma with surgery followed by high-dose chemotherapy and stem cell rescue. Pediatr Hematol Oncol 30:386–391

    Article  CAS  PubMed  Google Scholar 

  30. Mazloom A, Wolff JE, Paulino AC (2010) The impact of radiotherapy fields in the treatment of patients with choroid plexus carcinoma. Int J Radiat Oncol Biol Phys 78:79–84

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

This work was performed at the University of Wisconsin, Madison, WI.

Conflict of interest

None.

Author information

Authors and Affiliations

  1. Department of Radiation Oncology, Mountain States Tumor Institute, 725 Pole Line Rd. W., Twin Falls, ID, 83301, USA

    Donald M. Cannon

  2. Department of Radiation Oncology, University of Maryland School of Medicine, 22 S. Greene Street, Baltimore, MD, 21201, USA

    Pranshu Mohindra

  3. Cadence Health Central DuPage Hospital Cancer Center, 4405 Weaver Parkway, Warrenville, IL, 60555, USA

    Vinai Gondi

  4. Cadence Cancer Center at Delnor Hospital, 304 N Randall Road, Geneva, IL, 60134, USA

    Tim J. Kruser

  5. Mercy Regional Cancer Center, 1000 Mineral Point Ave, Janesville, WI, 53548, USA

    Kevin R. Kozak

Authors
  1. Donald M. Cannon
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Pranshu Mohindra
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Vinai Gondi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Tim J. Kruser
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Kevin R. Kozak
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Donald M. Cannon.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Cannon, D.M., Mohindra, P., Gondi, V. et al. Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management. J Neurooncol 121, 151–157 (2015). https://doi.org/10.1007/s11060-014-1616-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11060-014-1616-x

Keywords

Search

Navigation