Journal of Neuro-Oncology

, Volume 111, Issue 3, pp 337–345

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

  • Martin Benesch
  • André O. von Bueren
  • Tobias Dantonello
  • Katja von Hoff
  • Torsten Pietsch
  • Ivo Leuschner
  • Alexander Claviez
  • Uta Bierbach
  • Gabriele Kropshofer
  • Rudolf Korinthenberg
  • Norbert Graf
  • Meinolf Suttorp
  • Rolf Dieter Kortmann
  • Carsten Friedrich
  • Nicolas von der Weid
  • Peter Kaatsch
  • Thomas Klingebiel
  • Ewa Koscielniak
  • Stefan Rutkowski
Clinical Study

DOI: 10.1007/s11060-012-1020-3

Cite this article as:
Benesch, M., von Bueren, A.O., Dantonello, T. et al. J Neurooncol (2013) 111: 337. doi:10.1007/s11060-012-1020-3

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.

Keywords

Soft tissue sarcomasChildrenAdolescentsIntracranialChemotherapyRadiotherapy

Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • Martin Benesch
    • 1
  • André O. von Bueren
    • 2
  • Tobias Dantonello
    • 3
  • Katja von Hoff
    • 2
  • Torsten Pietsch
    • 4
  • Ivo Leuschner
    • 5
  • Alexander Claviez
    • 6
  • Uta Bierbach
    • 7
  • Gabriele Kropshofer
    • 8
  • Rudolf Korinthenberg
    • 9
  • Norbert Graf
    • 10
  • Meinolf Suttorp
    • 11
  • Rolf Dieter Kortmann
    • 12
  • Carsten Friedrich
    • 2
  • Nicolas von der Weid
    • 13
  • Peter Kaatsch
    • 14
  • Thomas Klingebiel
    • 15
  • Ewa Koscielniak
    • 3
  • Stefan Rutkowski
    • 2
  1. 1.Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent MedicineMedical University of GrazGrazAustria
  2. 2.Department of Pediatric Hematology and OncologyUniversity Medical Center Hamburg-EppendorfHamburgGermany
  3. 3.Pediatrics 5 (Oncology)Olgahospital, Klinikum StuttgartStuttgartGermany
  4. 4.Institute of NeuropathologyUniversity of BonnBonnGermany
  5. 5.Kiel Pediatric Tumor Registry, Department of Pediatric PathologyUniversity of Schleswig–HolsteinKielGermany
  6. 6.Department of General PediatricsUniversity of Schleswig–HolsteinKielGermany
  7. 7.Department of PediatricsUniversity Hospital LeipzigLeipzigGermany
  8. 8.Department of Pediatrics IMedical University of InnsbruckInnsbruckAustria
  9. 9.Department of Pediatric Neurology and Muscle DisordersUniversity Hospital FreiburgFreiburgGermany
  10. 10.Department of Pediatric Hematology and OncologySaarland UniversityHomburg/SaarGermany
  11. 11.University Children’s Hospital DresdenDresdenGermany
  12. 12.Department of Radiation OncologyUniversity of LeipzigLeipzigGermany
  13. 13.Pediatric Hematology-Oncology UnitUniversity Hospital CHUVLausanneSwitzerland
  14. 14.German Childhood Cancer RegistryMainzGermany
  15. 15.Division of Pediatric Hematology and Oncology, Department of PediatricsJ. W. Goethe University Children’s Hospital of FrankfurtFrankfurtGermany