Journal of Neuro-Oncology

, Volume 135, Issue 1, pp 151–160 | Cite as

A French retrospective study on clinical outcome in 102 choroid plexus tumors in children

  • A. Siegfried
  • S. Morin
  • C. Munzer
  • M. B. Delisle
  • M. Gambart
  • S. Puget
  • C. A. Maurage
  • C. Miquel
  • C. Dufour
  • P. Leblond
  • N. André
  • D. Figarella Branger
  • J. Kanold
  • J.-L. Kemeny
  • C. Icher
  • A. Vital
  • E. Uro Coste
  • A. I. Bertozzi
Clinical Study


The aim of this study was to review and describe therapeutic approaches in children with choroid plexus tumor (CPT) based on a nationwide series. The World Health Organization classification subdivides these rare tumors into three histological subtypes corresponding to three grades of malignancy: low grade (grade I) choroid plexus papilloma (CPP), intermediate grade (grade II) atypical choroid plexus papilloma (aCPP) and high grade (grade III) choroid plexus carcinoma (CPC). This retrospective study included 102 French children younger than 18 years, treated from 2000 to 2012: 54 CPP, 26 aCPP and 22 CPC. The 5 year overall survival was 100% in CPP, 96.2% in aCPP and 64.7% in CPC. In patients with localized disease, complete surgical resection was achieved in 48/52 CPP, 20/26 aCPP and 7/14 CPC. In this group, patients with complete surgical resection had better event free survival than patients with partial resection (88.9 vs. 41.6%). 28 patients (1 CPP, 6 aCPP and 22 CPC) had adjuvant chemotherapy. 2 aCPP and 9 CPC had radiotherapy. We underlined the need for a central histological review to accurately analyze clinical data; we reported a much higher overall survival for CPC than in most previous CPT series probably including atypical teratoid rhabdoid tumors. In our series, the 5 years overall survival in CPC (64.7%) was higher than event free survival (25.2%) and could be interpreted as a clue for the efficiency of adjuvant/salvage therapy even if the heterogeneity of applied treatments in this retrospective series does not allow for meaningful statistical comparisons.


Choroid plexus tumor Choroid plexus papilloma Atypical choroid plexus papilloma Choroid plexus carcinoma Atypical teratoid rhabdoid tumor Chemotherapy 



This study was supported by the association <<111 des Arts>>. We thank SFCE participating centers and the pediatric oncologists, pathologists and clinical research assistants for their contribution to this study. We thank Anita from Oscar’s Angels association, for her help in this study. We thank the Groupe d’Etude de Neuropathologie Oncologique Pédiatrique (GENOP) for their histopathological review.

Compliance with ethical standards

Conflict of interest

All authors declare that they have no conflicts of interest.

Supplementary material

11060_2017_2561_MOESM1_ESM.docx (155 kb)
Supplementary material 1 (DOCX 155 KB)


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Copyright information

© Springer Science+Business Media, LLC 2017

Authors and Affiliations

  • A. Siegfried
    • 1
  • S. Morin
    • 2
  • C. Munzer
    • 2
  • M. B. Delisle
    • 3
  • M. Gambart
    • 2
  • S. Puget
    • 4
  • C. A. Maurage
    • 5
  • C. Miquel
    • 6
  • C. Dufour
    • 7
  • P. Leblond
    • 8
  • N. André
    • 9
  • D. Figarella Branger
    • 10
  • J. Kanold
    • 11
  • J.-L. Kemeny
    • 12
  • C. Icher
    • 13
  • A. Vital
    • 14
  • E. Uro Coste
    • 3
  • A. I. Bertozzi
    • 2
  1. 1.Department of PathologyIUC-TToulouseFrance
  2. 2.Department of Hematology–OncologyChildren University HospitalToulouseFrance
  3. 3.Department of PathologyRangueil University HospitalToulouseFrance
  4. 4.Department of NeurosurgeryNecker HospitalParisFrance
  5. 5.Department of PathologyUniversity HospitalLilleFrance
  6. 6.Department of NeuropathologySainte-Anne Hospital, Descartes Paris V UniversityParisFrance
  7. 7.Department of OncologyInstitut Gustave RoussyVillejuifFrance
  8. 8.Department of OncologyOscar LambretLilleFrance
  9. 9.Department of Pediatric Hematology–OncologyChildren University HospitalMarseilleFrance
  10. 10.Department of PathologyUniversity HospitalMarseilleFrance
  11. 11.Department of Hematology–OncologyChildren University HospitalClermont FerrandFrance
  12. 12.Department of PathologyUniversity HospitalClermont FerrandFrance
  13. 13.Department of Hematology–OncologyChildren University HospitalBordeauxFrance
  14. 14.Department of PathologyUniversity HospitalBordeauxFrance

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