gms | German Medical Science

60th Annual Meeting of the German Society of Neurosurgery (DGNC)
Joint Meeting with the Benelux countries and Bulgaria

German Society of Neurosurgery (DGNC)

24 - 27 May 2009, Münster

The therapeutic challenge of rhabdoid tumors of the CNS (AT/RT) in childhood

Meeting Abstract

  • M. Frühwald - Klinik und Poliklinik für Kinder und Jugendmedizin, Pädiatrische Hämatologie und Onkologie, Universitätsklinikum Münster
  • G. Fleischhack - Abt. Pädiatrische Hämatologie/Onkologie, Universitätskinderklinik Bonn
  • S. Rutkowski - Universitätskinderklinik Würzburg
  • R. Schneppenheim - Universitätskinderklinik Hamburg
  • R. Siebert - Institut für Humangenetik, Campus Kiel, Universitätsklinikum Schleswig-Holstein
  • N. Graf - Universitätskinderklinik Homburg/Saar

Deutsche Gesellschaft für Neurochirurgie. 60. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit den Benelux-Ländern und Bulgarien. Münster, 24.-27.05.2009. Düsseldorf: German Medical Science GMS Publishing House; 2009. DocMO.08-04

doi: 10.3205/09dgnc047, urn:nbn:de:0183-09dgnc0475

Published: May 20, 2009

© 2009 Frühwald et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.



Objective: Rhabdoid tumors mainly affect infants and young children and may be found throughout the body. Within the CNS the term AT/RT, atypical teratoid rhabdoid tumor has been applied. Newer registry data suggest that in children below 6 months of age rhabdoid tumors may be the most common intracranial malignancies. Prognostic factors are metastatic spread at presentation, extent of neurosurgical resection and response to chemotherapy approaches.

Methods: In 2006 the German GPOH granted our group the task to develop clinical management strategies for affected patients. We have created a structured protocol for the registration and evaluation of children affected by rhabdoid tumors of any anatomical location. A consensus therapeutic approach was generated, which is based on an intensive induction chemotherapy regimen, early radiotherapy and if possible complete neurosurgical resection.

Results: Since 2006 we have registered the clinical, genetic and treatment data of 31 patients with AT/RT. Only a minority of these patients received a complete radical resection. 8 patients had a biopsy only. All patients received chemotherapy regimens containing anthracyclines and most received radiotherapy. Whenever tumor material was available genetic testing for the gene SMARCB1 was performed. Follow-up is short, but demonstrates that if no complete response can be obtained by multimodality treatment outcome is rather dismal.

Conclusions: We present first data from our German registry and present the development in Europe. We are near completion of a European registry for rhabdoid tumors (EURHAB). This will incorporate registration of all available patient data and the development of a phase I / II strategy to improve outcome.