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Titel: Clinical and genetic risk factors define two risk groups of extracranial malignant rhabdoid tumours (eMRT/RTK)
VerfasserIn: Nemes, Karolina
Bens, Susanne
Kachanov, Denis
Teleshova, Margarita
Hauser, Peter
Simon, Thorsten
Tippelt, Stephan
Woessmann, Wilhelm
Beck, Olaf
Flotho, Christian
Grigull, Lorenz
Driever, Pablo H.
Schlegel, Paul-Gerhardt
Khurana, Claudia
Hering, Kathrin
Kolb, Reinhard
Leipold, Alfred
Abbink, Floor
Gil-Da-Costa, Maria J.
Benesch, Martin
Kerl, Kornelius
Lowis, Stephen
Marques, Carmen H.
Graf, Norbert
Nysom, Karsten
Vokuhl, Christian
Melchior, Patrick
Kröncke, Thomas
Schneppenheim, Reinhard
Kordes, Uwe
Gerss, Joachim
Siebert, Reiner
Furtwängler, Rhoikos
Frühwald, Michael C.
Sprache: Englisch
Titel: European Journal of Cancer
Bandnummer: 142 (2021)
Seiten: 112-122
Verlag/Plattform: Elsevier
Erscheinungsjahr: 2020
DDC-Sachgruppe: 610 Medizin, Gesundheit
Dokumenttyp: Journalartikel / Zeitschriftenartikel
Abstract: Introduction Extracranial rhabdoid tumours are rare, highly aggressive malignancies primarily affecting young children. The EU-RHAB registry was initiated in 2009 to prospectively collect data of rhabdoid tumour patients treated according to the EU-RHAB therapeutic framework. Methods We evaluated 100 patients recruited within EU-RHAB (2009–2018). Tumours and matching blood samples were examined for SMARCB1 mutations by sequencing and cytogenetics. Results A total of 70 patients presented with extracranial, extrarenal tumours (eMRT) and 30 with renal rhabdoid tumours (RTK). Nine patients demonstrated synchronous tumours. Distant metastases at diagnosis (M+) were present in 35% (35/100), localised disease (M0) with (LN+) and without (LN−) loco-regional lymph node involvement in 65% (65/100). SMARCB1 germline mutations (GLM) were detected in 21% (17/81 evaluable) of patients. The 5-year overall survival (OS) and event-free survival (EFS) rates were 45.8 ± 5.4% and 35.2 ± 5.1%, respectively. On univariate analyses, age at diagnosis (≥12 months), M0-stage, absence of synchronous tumours, absence of a GLM, gross total resection (GTR), radiotherapy and achieving a CR were significantly associated with favourable outcomes. In an adjusted multivariate model presence of a GLM, M+ and lack of a GTR were the strongest significant negative predictors of outcome. Conclusions We suggest to stratify patients with localised disease (M0), GTR+ and without proof of a GLM (5-year OS 72.2 ± 9.9%) as ‘standard risk’. Patients presenting with one of the features M+ and/or GTR− and/or GLM+ belong to a high risk group (5-year, OS 32.5 ± 6.2%). These patients need novel therapeutic strategies such as combinations of targeted agents with conventional chemotherapy or novel experimental approaches ideally within international phase I/II trials.
DOI der Erstveröffentlichung: 10.1016/j.ejca.2020.10.004
URL der Erstveröffentlichung: https://doi.org/10.1016/j.ejca.2020.10.004
Link zu diesem Datensatz: urn:nbn:de:bsz:291--ds-406041
hdl:20.500.11880/36478
http://dx.doi.org/10.22028/D291-40604
ISSN: 0959-8049
Datum des Eintrags: 26-Sep-2023
Fakultät: M - Medizinische Fakultät
Fachrichtung: M - Pädiatrie
M - Radiologie
Professur: M - Prof. Dr. Norbert Graf
M - Keiner Professur zugeordnet
Sammlung:SciDok - Der Wissenschaftsserver der Universität des Saarlandes

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