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Adults with CNS primitive neuroectodermal tumors/pineoblastomas: results of multimodal treatment according to the pediatric HIT 2000 protocol

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Abstract

Central nervous system primitive neuroectodermal tumors (CNS-PNET) and pineoblastomas (PBL) are rare in adulthood. Knowledge on clinical outcome and the efficacy and toxicities of chemotherapy in addition to radiotherapy is limited. Patients older than 21 years at diagnosis were followed in the observational arm of the prospective pediatric multicenter trial HIT 2000. After surgery, craniospinal irradiation and maintenance or sandwich chemotherapy were recommended. Radiotherapy was normo- (35.2 Gy; tumor region, 55.0 Gy; metastasis, 49.6 Gy) or hyperfractionated (40.0 Gy; tumor bed, 68.0 Gy; metastasis, 50–60 Gy). Maintenance chemotherapy consisted of eight courses (vincristine, lomustine, cisplatin). Sandwich chemotherapy included two cycles of postoperative chemotherapy followed by radiotherapy, and four courses of maintenance chemotherapy. Seventeen patients (CNS-PNET, n = 7; PBL, n = 10), median age 30 years, were included. Eight patients had a postoperative residual tumor and four patients metastatic disease. The median follow-up of ten surviving patients was 41 months. The estimated rates for 3-year progression-free survival (PFS) and overall survival were 68 ± 12 and 66 ± 13 %, respectively. PBL compared to CNS-PNET tended towards a better PFS, although the difference was not clear (p = 0.101). Both chemotherapeutic (maintenance, n = 6; sandwich, n = 8) protocols did not differ in their PFS and were feasible with acceptable toxicities. Intensified regimens of combined chemo- and radiotherapy are generally feasible in adults with CNS-PNET/PBL. The impact of intensified chemotherapy on survival should be further assessed.

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Acknowledgments

We thank the participating centers for their valuable cooperation. Special thanks to Wiebke Treulieb and Christine Lindow (HIT data center) for their excellent data management, and to Katharina Petrasch for CSF reference assessments. The HIT 2000 trial office and the reference institutions are supported by the German Children’s Cancer Foundation (Deutsche Kinderkrebsstiftung).

Conflict of interest

None declared.

Author information

Authors and Affiliations

  1. Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Germany

    Carsten Friedrich, Katja von Hoff, André O. von Bueren & Stefan Rutkowski

  2. Department of Radiation Oncology, University of Leipzig, Leipzig, Germany

    Klaus Müller & Rolf D. Kortmann

  3. Institute of Biostatistics and Clinical Research, University of Muenster, Muenster, Germany

    Robert Kwiecien

  4. Department of Neuropathology, University of Bonn, Bonn, Germany

    Torsten Pietsch

  5. Department of Neuroradiology, University of Wuerzburg, Wuerzburg, Germany

    Monika Warmuth-Metz

  6. Department of Oncology, University Children’s Hospital, Zurich, Switzerland

    Nicolas U. Gerber

  7. Department of Neurology and Wilhelm Sander-NeuroOncology Unit, University Hospital Regensburg, Regensburg, Germany

    Peter Hau

  8. Department of Pediatrics, University of Wuerzburg, Wuerzburg, Germany

    Joachim Kuehl

  9. Department of Pediatrics and Adolescent Medicine, Division of Pediatric Hematology and Oncology, University Medical Center Goettingen, Goettingen, Germany

    André O. von Bueren

Authors
  1. Carsten Friedrich
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  2. Klaus Müller
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  3. Katja von Hoff
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  4. Robert Kwiecien
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  7. Nicolas U. Gerber
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  8. Peter Hau
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  10. Rolf D. Kortmann
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  11. André O. von Bueren
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  12. Stefan Rutkowski
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Corresponding author

Correspondence to Stefan Rutkowski.

Additional information

André O.von Bueren and Stefan Rukowski are contributed equally.

Joachim Kuehl—Deceased.

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Friedrich, C., Müller, K., von Hoff, K. et al. Adults with CNS primitive neuroectodermal tumors/pineoblastomas: results of multimodal treatment according to the pediatric HIT 2000 protocol. J Neurooncol 116, 567–575 (2014). https://doi.org/10.1007/s11060-013-1327-8

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  • DOI: https://doi.org/10.1007/s11060-013-1327-8

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