dc.contributor.author | Bartelheim, Kerstin | |
dc.contributor.author | Nemes, Karolina | |
dc.contributor.author | Seeringer, Angela | |
dc.contributor.author | Kerl, Kornelius | |
dc.contributor.author | Buechner, Jochen | |
dc.contributor.author | Boos, Joachim | |
dc.contributor.author | Graf, Norbert | |
dc.contributor.author | Durken, Matthias | |
dc.contributor.author | Gerss, Joachim | |
dc.contributor.author | Hasselblatt, Martin | |
dc.contributor.author | Kortmann, Rolf-Dieter | |
dc.contributor.author | Teichert von Luettichau, Irene | |
dc.contributor.author | Nagel, Inga | |
dc.contributor.author | Nygaard, Randi | |
dc.contributor.author | Oyen, Florian | |
dc.contributor.author | Quiroga, Eduardo | |
dc.contributor.author | Schlegel, Paul-Gerhardt | |
dc.contributor.author | Schmid, Irene | |
dc.contributor.author | Schneppenheim, Reinhard | |
dc.contributor.author | Siebert, Reiner | |
dc.contributor.author | Solano-Paez, Palma | |
dc.contributor.author | Timmermann, Beate | |
dc.contributor.author | Warmuth-Metz, Monika | |
dc.contributor.author | Fruhwald, Michael Christoph | |
dc.date.accessioned | 2022-11-18T13:52:01Z | |
dc.date.available | 2022-11-18T13:52:01Z | |
dc.date.created | 2017-01-21T22:41:31Z | |
dc.date.issued | 2016 | |
dc.identifier.citation | Cancer Medicine. 2016, 5 (8), 1765-1775. | en_US |
dc.identifier.issn | 2045-7634 | |
dc.identifier.uri | https://hdl.handle.net/11250/3032915 | |
dc.description.abstract | A typical 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 eventfree survival rates were 46% (±0.10) and 45% (±0.09), respectively. Serious adverse events and one treatmentrelated 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. | en_US |
dc.language.iso | eng | en_US |
dc.publisher | John Wiley & Sons Ltd. | en_US |
dc.rights | Navngivelse 4.0 Internasjonal | * |
dc.rights.uri | http://creativecommons.org/licenses/by/4.0/deed.no | * |
dc.title | Improved 6-year overall survival in AT/RT – results of the registry study Rhabdoid 2007 | en_US |
dc.type | Peer reviewed | en_US |
dc.type | Journal article | en_US |
dc.description.version | publishedVersion | en_US |
dc.source.pagenumber | 1765-1775 | en_US |
dc.source.volume | 5 | en_US |
dc.source.journal | Cancer Medicine | en_US |
dc.source.issue | 8 | en_US |
dc.identifier.doi | 10.1002/cam4.741 | |
dc.identifier.cristin | 1434778 | |
cristin.ispublished | true | |
cristin.fulltext | original | |
cristin.qualitycode | 1 | |