Journal of Neuro-Oncology

, Volume 95, Issue 3, pp 383–392 | Cite as

Atypical choroid plexus papilloma: clinical experience in the CPT-SIOP-2000 study

  • Brigitte Wrede
  • Martin Hasselblatt
  • Ove Peters
  • Peter F. Thall
  • Tezer Kutluk
  • Albert Moghrabi
  • Anita Mahajan
  • Stefan Rutkowski
  • Blanca Diez
  • Xuemei Wang
  • Torsten Pietsch
  • Rolf-Dieter Kortmann
  • Werner Paulus
  • Astrid Jeibmann
  • Johannes E. A. Wolff
Clinical Study - Patient Study
First Online:
Received:
Accepted:

Abstract

Atypical choroid plexus papilloma (APP) represents a novel intermediate-grade subtype of choroid plexus tumor (CPT), the clinical outcome of which has not been described yet. We present the first analysis of a group of APP patients enrolled in the ongoing CPT-SIOP-2000 study of CPTs. A worldwide registration and a randomized trial for those patients who require chemotherapy started in 2000. For APP, maximal surgical resection was recommended. After surgery, patients who had undergone complete resection were observed, whereas patients with incompletely resected or metastasized APP were treated with six chemotherapy courses (etoposide and vincristine, combined with either carboplatin or cyclophosphamide). Risk-adapted radiotherapy was given only to patients older than 3 years of age. Of the 106 patients with a centrally confirmed CPT histology, 30 had APP, 42 CPP and 34 CPC. APP patients were significantly younger (median = 0.7 years) than patients with CPP or CPC (both medians = 2.3 years). Complete resection was achieved in 68 (64%) patients (79% in CPP, 63% in APP, and 47% in CPC). Metastases were present at diagnosis in 17% of APP patients, 5% of CPP patients, and 21% of CPC patients. All nine APP patients who received postoperative chemotherapy showed an early response after two cycles: two had complete remission, four had partial response, and three had stable disease. In the observation group of 15 patients, one event was seen, and all patients were alive. In the treatment group, one patient with a metastasized tumor and incompletely resected APP died. While APP was defined histologically, median percentages of both the Ki-67/MIB-1 proliferation marker and the p53 tumor suppressor protein increased across the three histological subtypes (from CPP to APP and then CPC), suggesting that the subtypes comprise an ordinal categorization of increasingly severe CPT tumors. This ordering was reiterated by clinical outcome in the 92 patients treated per the study protocol, with 5-year EFS rates of 92% in 39 CPP patients, 83% in 24 APP patients, and 28% in 29 CPC patients. A similar ordering was seen when all 106 patients were evaluated for EFS. APP responded favorably to chemotherapy. The intermediate position of APP between CPP and CPC was supported by the clinical data.

Keywords

Choroid plexus tumors Atypical choroid plexus papilloma Survival Chemotherapy 
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Notes

Acknowledgements

The CPT-SIOP-2000 study was designed in 1999, and far more people had significantly more academic impact during the past decade than suggested by the list of authors. We thank David Ellison (Pathology, Memphis, TN, USA) for excellent neuropathology work and helpful scientific discussion. We thank Richard Sposto (Children’s Hospital of Los Angeles, USA) for statistical support and enthusiasm to get the study. Among the numerous clinical participants, we wish to thank R. Corbett, M. Nathrath, S. Van Gool, L. Leskova, G. Ebetsberger, J. Sterba, N. Jorch, D. Ashley, B. Erdlenbruch, C. Kramm, J. Finlay, M. Warmuth Metz, R. Kebudi, T. Hassall, M. Dittrich, N. Konoplya, M. v. d. Hagen, I. Slavc, U. Kontny, M. Albert, R. Reddingius, D. C. Kiss, A. Navajos, M. L. Garre, and many others. The CPT-SIOP-2000 study is supported by the Deutsche Kinderkrebsstiftung, also supporting B. W. M. H. and W. P. are supported by Deutsche Krebshilfe (Grant 108263).

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Copyright information

© Springer Science+Business Media, LLC. 2009

Authors and Affiliations

  • Brigitte Wrede
    • 1
  • Martin Hasselblatt
    • 2
  • Ove Peters
    • 1
  • Peter F. Thall
    • 3
  • Tezer Kutluk
    • 4
  • Albert Moghrabi
    • 5
  • Anita Mahajan
    • 6
  • Stefan Rutkowski
    • 7
  • Blanca Diez
    • 8
  • Xuemei Wang
    • 3
  • Torsten Pietsch
    • 9
  • Rolf-Dieter Kortmann
    • 10
  • Werner Paulus
    • 2
  • Astrid Jeibmann
    • 2
  • Johannes E. A. Wolff
    • 1
    • 3
    • 11
  1. 1.Department of Pediatric OncologySt. Hedwig Children’s Hospital, University of RegensburgRegensburgGermany
  2. 2.Institute of NeuropathologyUniversity Hospital MünsterMünsterGermany
  3. 3.Department of BiostatisticsThe University of Texas M. D. Anderson Cancer CenterHoustonUSA
  4. 4.Institute of OncologyHacettepe UniversityAnkaraTurkey
  5. 5.Department of PediatricsSainte-Justine HospitalMontrealCanada
  6. 6.Department of Radiation OncologyThe University of Texas M. D. Anderson Cancer CenterHoustonUSA
  7. 7.Department of Pediatric Hematology and OncologyUniversity of Hamburg-EppendorfHamburgGermany
  8. 8.Neuro Oncology ProgramInstitute of Neurological Research, FLENIBuenos AiresArgentina
  9. 9.Institute for NeuropathologyUniversity of BonnBonnGermany
  10. 10.Department of RadiotherapyUniversity of LeipzigLeipzigGermany
  11. 11.Department of Pediatrics, Unit 87The University of Texas M. D. Anderson Cancer CenterHoustonUSA

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