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Primary intracranial soft tissue sarcoma in children and adolescents: a cooperative analysis of the European CWS and HIT study groups

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Abstract

Purely intracranial soft tissue sarcomas (ISTS) are very rare among children. A retrospective database analysis of the Cooperative Weichteilsarkom Studiengruppe (CWS) and brain tumor (HIT) registries was conducted to describe treatment and long-term outcome of children and adolescents with ISTS. Nineteen patients from Germany, Austria and Switzerland were reported between 1988 and 2009. Median age at diagnosis was 9.7 years (range, 0.5–17.8). Central pathological review was performed in 17 patients. Eleven patients underwent a total and five a subtotal tumor resection. A biopsy was done in one patient. In two patients no data concerning extent of initial resection was available. Radiotherapy was performed in 15 patients (first-line, n = 11; following progression, n = 4). All but one patient received chemotherapy (first-line, n = 7, following progression, n = 5; first-line and following progression, n = 6). With a median follow-up of 5.8 years (range, 0.6–19.8) ten patients were alive in either first or second complete remission. Seven patients died due to relapse or progression and two were alive with progressive disease. Estimated progression-free and overall survival at 5 years were 47 % (±12 %) and 74 % (±10 %), respectively. About 50 % of patients with ISTS remain relapse-free after 5 years. Multimodality treatment including complete tumor resection and radio-/chemotherapy is required to achieve sustained tumor control in patients with ISTS. Early initiation of postoperative non-surgical treatment seems to be important to prevent recurrence. Due to the intracranial localization local therapy should follow the recommendations used in brain tumors rather than in soft tissue sarcomas, whereas chemotherapy should be guided by histological subtype.

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Acknowledgments

The authors thank Wibke Treulieb and Christine Lindow (HIT study centre Hamburg, Germany), Gabriele Pammer (HIT study centre Graz, Austria), Erika Hallmen, Iris Veit-Friedrich and Simone Feuchtgruber (CWS study centre Stuttgart, Germany) for excellent data management, Claudia Bremensdorfer for administrative support and Franz Quehenberger (Medical University of Graz) for statistical support. The HIT and CWS studies and the Reference Centre for Neuropathology at the University of Bonn are supported by Grants from the Deutsche Kinderkrebsstiftung (German Childhood Cancer Foundation). The Austrian HIT study centre is supported by the Steirische Kinderkrebshilfe.

Conflict of interest

All authors declare no conflict of interest.

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Authors and Affiliations

  1. Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Auenbruggerplatz 38, 8036, Graz, Austria

    Martin Benesch

  2. Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

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

  3. Pediatrics 5 (Oncology), Olgahospital, Klinikum Stuttgart, Stuttgart, Germany

    Tobias Dantonello & Ewa Koscielniak

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

    Torsten Pietsch

  5. Kiel Pediatric Tumor Registry, Department of Pediatric Pathology, University of Schleswig–Holstein, Kiel, Germany

    Ivo Leuschner

  6. Department of General Pediatrics, University of Schleswig–Holstein, Kiel, Germany

    Alexander Claviez

  7. Department of Pediatrics, University Hospital Leipzig, Leipzig, Germany

    Uta Bierbach

  8. Department of Pediatrics I, Medical University of Innsbruck, Innsbruck, Austria

    Gabriele Kropshofer

  9. Department of Pediatric Neurology and Muscle Disorders, University Hospital Freiburg, Freiburg, Germany

    Rudolf Korinthenberg

  10. Department of Pediatric Hematology and Oncology, Saarland University, Homburg/Saar, Germany

    Norbert Graf

  11. University Children’s Hospital Dresden, Dresden, Germany

    Meinolf Suttorp

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

    Rolf Dieter Kortmann

  13. Pediatric Hematology-Oncology Unit, University Hospital CHUV, Lausanne, Switzerland

    Nicolas von der Weid

  14. German Childhood Cancer Registry, Mainz, Germany

    Peter Kaatsch

  15. Division of Pediatric Hematology and Oncology, Department of Pediatrics, J. W. Goethe University Children’s Hospital of Frankfurt, Frankfurt, Germany

    Thomas Klingebiel

Authors
  1. Martin Benesch
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  2. André O. von Bueren
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  3. Tobias Dantonello
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  4. Katja von Hoff
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  5. Torsten Pietsch
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  7. Alexander Claviez
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  8. Uta Bierbach
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  9. Gabriele Kropshofer
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  10. Rudolf Korinthenberg
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  11. Norbert Graf
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  13. Rolf Dieter Kortmann
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  14. Carsten Friedrich
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  15. Nicolas von der Weid
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  16. Peter Kaatsch
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  17. Thomas Klingebiel
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  18. Ewa Koscielniak
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  19. Stefan Rutkowski
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Correspondence to Martin Benesch.

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Benesch, M., von Bueren, A.O., Dantonello, T. et al. Primary intracranial soft tissue sarcoma in children and adolescents: a cooperative analysis of the European CWS and HIT study groups. J Neurooncol 111, 337–345 (2013). https://doi.org/10.1007/s11060-012-1020-3

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  • DOI: https://doi.org/10.1007/s11060-012-1020-3

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