Article
The therapeutic challenge of rhabdoid tumors of the CNS (AT/RT) in childhood
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Published: | May 20, 2009 |
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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.