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000294035 1001_ $$00000-0001-7488-6885$$aFincke, Victoria E$$b0
000294035 245__ $$aClinical and Molecular Risk Factors in Extracranial Malignant Rhabdoid Tumors: Toward an Integrated Model of High-Risk Tumors.
000294035 260__ $$aPhiladelphia, Pa. [u.a.]$$bAACR$$c2024
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000294035 520__ $$aExtracranial malignant rhabdoid tumors (eMRT) are a challenging entity. Despite the use of multimodal treatment approaches, therapy failure occurs in 55% to 67% of these. Molecular markers for identification of patients at increased risk for relapse or refractory (R/R) disease are not available. Clinical characteristics may only insufficiently predict the individual course of disease.Using the EU-RHAB database, we analyzed a cohort of 121 patients with eMRT clinically. For 81 patients, molecular and clinical data were available, which were further complemented with publicly available DNA molecular data from 92 eMRTs. We aimed to delineate molecular risk factors by dissecting the DNA methylome of these tumors. Moreover, we establish clinical characteristics and treatment details of R/R disease in a subcohort of 80 patients.Using consensus hierarchical clustering, we identified three distinct subgroups, one of which (eMRT standard risk) was associated with significantly improved survival, irrespective of germline status and/or localization. At the transcriptome level, this subgroup was characterized by an overexpression of genes involved in muscle development. A relevant proportion of patients developed distant relapses or progressions; the median time to the event was 4 months, underlining the need for early identification and risk stratification of R/R disease. The overall survival was significantly decreased in patients with progressive disease when compared with relapse cases, and reaching complete remission during salvage therapy provided a survival benefit.Our analysis of eMRT in this comprehensive cohort provides novel insights into the patterns of relapse and integrates molecular and clinical risk factors to guide clinical decision-making.
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000294035 650_7 $$2NLM Chemicals$$aBiomarkers, Tumor
000294035 650_2 $$2MeSH$$aHumans
000294035 650_2 $$2MeSH$$aRhabdoid Tumor: genetics
000294035 650_2 $$2MeSH$$aRhabdoid Tumor: pathology
000294035 650_2 $$2MeSH$$aRhabdoid Tumor: therapy
000294035 650_2 $$2MeSH$$aMale
000294035 650_2 $$2MeSH$$aFemale
000294035 650_2 $$2MeSH$$aRisk Factors
000294035 650_2 $$2MeSH$$aChild, Preschool
000294035 650_2 $$2MeSH$$aBiomarkers, Tumor: genetics
000294035 650_2 $$2MeSH$$aInfant
000294035 650_2 $$2MeSH$$aChild
000294035 650_2 $$2MeSH$$aPrognosis
000294035 650_2 $$2MeSH$$aDNA Methylation
000294035 650_2 $$2MeSH$$aNeoplasm Recurrence, Local: genetics
000294035 650_2 $$2MeSH$$aNeoplasm Recurrence, Local: pathology
000294035 650_2 $$2MeSH$$aAdolescent
000294035 650_2 $$2MeSH$$aTranscriptome
000294035 650_2 $$2MeSH$$aGene Expression Profiling
000294035 7001_ $$00009-0000-1501-3064$$aSteinbügl, Mona$$b1
000294035 7001_ $$00009-0005-1911-9487$$aChun, Hye-Jung E$$b2
000294035 7001_ $$00000-0003-0270-5875$$aNemes, Karolina$$b3
000294035 7001_ $$00000-0001-9009-3383$$aMucha, Marlena$$b4
000294035 7001_ $$00009-0009-6414-8550$$aLoßner, Maurice$$b5
000294035 7001_ $$00009-0000-0223-9654$$aDorn, Felix$$b6
000294035 7001_ $$00000-0002-2211-6720$$aGastberger, Katharina$$b7
000294035 7001_ $$00009-0003-2308-477X$$aBühner, Sebastian$$b8
000294035 7001_ $$0P:(DE-He78)45440b44791309bd4b7dbb4f73333f9b$$aSill, Martin$$b9
000294035 7001_ $$00000-0003-4889-1036$$aKröncke, Thomas$$b10
000294035 7001_ $$00000-0001-7433-3703$$aSiebert, Reiner$$b11
000294035 7001_ $$00000-0003-2305-8857$$aMelchior, Patrick$$b12
000294035 7001_ $$00000-0002-1967-8343$$aFurtwängler, Rhoikos$$b13
000294035 7001_ $$aSchlesner, Matthias$$b14
000294035 7001_ $$00009-0003-6596-1419$$aVokuhl, Christian$$b15
000294035 7001_ $$00000-0002-6989-8002$$aRöcken, Christoph$$b16
000294035 7001_ $$0P:(DE-He78)3fdc3623477264cb5d0e14f256dbfbb8$$aJohann, Pascal$$b17$$eLast author
000294035 7001_ $$00000-0002-8237-1854$$aFrühwald, Michael C$$b18
000294035 773__ $$0PERI:(DE-600)2036787-9$$a10.1158/1078-0432.CCR-23-3489$$gVol. 30, no. 20, p. 4667 - 4680$$n20$$p4667 - 4680$$tClinical cancer research$$v30$$x1078-0432$$y2024
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