Improved 6-year overall survival in AT/RT: results of the registry study Rhabdoid 2007
Atypical teratoid rhabdoid tumors (AT/RT) are characterized by mutations and subsequent inactivation of SMARCB1 (INI1, hSNF5), a predilection for very young children and an unfavorable outcome. The European Registry for rhabdoid tumors (EU-RHAB) was established to generate a common European database...
Gespeichert in:
| Hauptverfasser: | , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
26 May 2016
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| In: |
Cancer medicine
Year: 2016, Jahrgang: 5, Heft: 8, Pages: 1765-1775 |
| ISSN: | 2045-7634 |
| DOI: | 10.1002/cam4.741 |
| Online-Zugang: | Verlag, Volltext: https://doi.org/10.1002/cam4.741 Verlag: https://onlinelibrary.wiley.com/doi/abs/10.1002/cam4.741 
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| Verfasserangaben: | Kerstin Bartelheim, Karolina Nemes, Angela Seeringer, Kornelius Kerl, Jochen Buechner, Joachim Boos, Norbert Graf, Matthias Dürken, Joachim Gerss, Martin Hasselblatt, Rolf-Dieter Kortmann, Irene Teichert von Luettichau, Inga Nagel, Randi Nygaard, Florian Oyen, Eduardo Quiroga, Paul-Gerhardt Schlegel, Irene Schmid, Reinhard Schneppenheim, Reiner Siebert, Palma Solano‐Paez, Beate Timmermann, Monika Warmuth‐Metz and Michael Christoph Frühwald |
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| 520 | |a Atypical teratoid rhabdoid tumors (AT/RT) are characterized by mutations and subsequent inactivation of SMARCB1 (INI1, hSNF5), a predilection for very young children and an unfavorable outcome. The European Registry for rhabdoid tumors (EU-RHAB) was established to generate a common European database and to establish a standardized treatment regimen as the basis for phase I/II trials. Thus, genetic analyses, neuropathologic and radiologic diagnoses, and a consensus treatment regimen were prospectively evaluated. From 2005 to 2009, 31 patients with AT/RT from four countries were recruited into the registry study Rhabdoid 2007 and treated with systemic and intraventricular chemotherapy. Eight patients received high-dose chemotherapy, 23 radiotherapy, and 17 maintenance therapy. Reference evaluations were performed in 64% (genetic analyses, FISH, MLPA, sequencing) up to 97% (neuropathology, INI1 stain). Germ-line mutations (GLM) were detected in 6/21 patients. Prolonged overall survival was associated with age above 3 years, radiotherapy and achievement of a complete remission. 6-year overall and event-free survival rates were 46% (±0.10) and 45% (±0.09), respectively. Serious adverse events and one treatment-related death due to insufficiency of a ventriculo peritoneal shunt (VP-shunt) and consecutive herniation were noted. Acquisition of standardized data including reference diagnosis and a standard treatment schedule improved data quality along with a survival benefit. Treatment was feasible with significant but manageable toxicity. Although our analysis is biased due to heterogeneous adherence to therapy, EU-RHAB provides the best available basis for phase I/II clinical trials. | ||
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