000147254 001__ 147254
000147254 005__ 20240229123022.0
000147254 0247_ $$2doi$$a10.1002/pbc.28022
000147254 0247_ $$2pmid$$apmid:31571386
000147254 0247_ $$2ISSN$$a0098-1532
000147254 0247_ $$2ISSN$$a1096-911X
000147254 0247_ $$2ISSN$$a1545-5009
000147254 0247_ $$2ISSN$$a1545-5017
000147254 0247_ $$2altmetric$$aaltmetric:67547017
000147254 037__ $$aDKFZ-2019-02377
000147254 041__ $$aeng
000147254 082__ $$a610
000147254 1001_ $$00000-0001-7827-4130$$aBenesch, Martin$$b0
000147254 245__ $$aSpinal cord atypical teratoid/rhabdoid tumors in children: Clinical, genetic, and outcome characteristics in a representative European cohort.
000147254 260__ $$aNew York, NY$$bWiley$$c2020
000147254 3367_ $$2DRIVER$$aarticle
000147254 3367_ $$2DataCite$$aOutput Types/Journal article
000147254 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1580111218_3899
000147254 3367_ $$2BibTeX$$aARTICLE
000147254 3367_ $$2ORCID$$aJOURNAL_ARTICLE
000147254 3367_ $$00$$2EndNote$$aJournal Article
000147254 500__ $$a2020 Jan;67(1):e28022
000147254 520__ $$aCase reports have portrayed spinal cord atypical teratoid/rhabdoid tumor (spATRT) as an aggressive form of ATRT. We conducted a retrospective European survey to collect data on clinical characteristics, molecular biology, treatment, and outcome of children with intramedullary spATRT.Scrutinizing a French national series and the European Rhabdoid Registry database, we identified 13 patients (median age 32 months; metastatic disease at diagnosis, n = 6). Systemic postoperative chemotherapy was administered to all patients; three received intrathecal therapy and six were irradiated (craniospinal, n = 3; local, n = 3).Median observation time was 8 (range, 1-93) months. Progression-free and overall survival rates at 1 and (2 years) were 35.2% ± 13.9% (26.4% ± 12.9%) and 38.5% ± 13.5% (23.1% ± 11.7%). Four patients (ATRT-SHH, n = 2; ATRT-MYC, n = 1; DNA methylation subgroup not available, n = 1) achieved complete remission (CR); two of them are alive in CR 69 and 72 months from diagnosis. One patient relapsed after CR and is alive with progressive disease (PD) and one died of the disease. Three patients (ATRT-MYC, n = 2; subgroup not available, n = 1) died after 7 to 22 months due to PD after having achieved a partial remission (n = 1) or stabilization (n = 2). Five patients (ATRT-MYC, n = 2; subgroup not available, n = 3) developed early PD and died. One patient (ATRT-MYC) died of intracerebral hemorrhage prior to response evaluation.Long-term survival is achievable in selected patients with spATRT using aggressive multimodality treatment. Larger case series and detailed molecular analyses are needed to understand differences between spATRT and their inracranial counterparts and the group of extradural malignant rhabdoid tumors.
000147254 536__ $$0G:(DE-HGF)POF3-312$$a312 - Functional and structural genomics (POF3-312)$$cPOF3-312$$fPOF III$$x0
000147254 588__ $$aDataset connected to CrossRef, PubMed,
000147254 7001_ $$00000-0003-0270-5875$$aNemes, Karolina$$b1
000147254 7001_ $$aNeumayer, Petra$$b2
000147254 7001_ $$aHasselblatt, Martin$$b3
000147254 7001_ $$aTimmermann, Beate$$b4
000147254 7001_ $$aBison, Brigitte$$b5
000147254 7001_ $$aEbetsberger-Dachs, Georg$$b6
000147254 7001_ $$aBourdeaut, Franck$$b7
000147254 7001_ $$00000-0001-5993-8077$$aDufour, Christelle$$b8
000147254 7001_ $$aBiassoni, Veronica$$b9
000147254 7001_ $$aMorales La Madrid, Andrés$$b10
000147254 7001_ $$aEntz-Werle, Natacha$$b11
000147254 7001_ $$aLaithier, Véronique$$b12
000147254 7001_ $$aQuehenberger, Franz$$b13
000147254 7001_ $$aWeis, Serge$$b14
000147254 7001_ $$aSumerauer, David$$b15
000147254 7001_ $$aSiebert, Reiner$$b16
000147254 7001_ $$aBens, Susanne$$b17
000147254 7001_ $$aSchneppenheim, Reinhard$$b18
000147254 7001_ $$0P:(DE-He78)4c28e2aade5f44d8eca9dd8e97638ec8$$aKool, Marcel$$b19$$udkfz
000147254 7001_ $$00000-0002-2339-4300$$aModena, Piergiorgio$$b20
000147254 7001_ $$aFouyssac, Fanny$$b21
000147254 7001_ $$00000-0002-8237-1854$$aC Frühwald, Michael$$b22
000147254 773__ $$0PERI:(DE-600)2130978-4$$a10.1002/pbc.28022$$n1$$pe28022$$tPediatric blood & cancer$$v67$$x1545-5017$$y2020
000147254 909CO $$ooai:inrepo02.dkfz.de:147254$$pVDB
000147254 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)4c28e2aade5f44d8eca9dd8e97638ec8$$aDeutsches Krebsforschungszentrum$$b19$$kDKFZ
000147254 9131_ $$0G:(DE-HGF)POF3-312$$1G:(DE-HGF)POF3-310$$2G:(DE-HGF)POF3-300$$3G:(DE-HGF)POF3$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lKrebsforschung$$vFunctional and structural genomics$$x0
000147254 9141_ $$y2020
000147254 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS
000147254 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline
000147254 915__ $$0StatID:(DE-HGF)0310$$2StatID$$aDBCoverage$$bNCBI Molecular Biology Database
000147254 915__ $$0StatID:(DE-HGF)0100$$2StatID$$aJCR$$bPEDIATR BLOOD CANCER : 2017
000147254 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bClarivate Analytics Master Journal List
000147254 915__ $$0StatID:(DE-HGF)0110$$2StatID$$aWoS$$bScience Citation Index
000147254 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection
000147254 915__ $$0StatID:(DE-HGF)0111$$2StatID$$aWoS$$bScience Citation Index Expanded
000147254 915__ $$0StatID:(DE-HGF)1110$$2StatID$$aDBCoverage$$bCurrent Contents - Clinical Medicine
000147254 915__ $$0StatID:(DE-HGF)1050$$2StatID$$aDBCoverage$$bBIOSIS Previews
000147254 915__ $$0StatID:(DE-HGF)9900$$2StatID$$aIF < 5
000147254 9201_ $$0I:(DE-He78)B062-20160331$$kB062$$lPädiatrische Neuroonkologie$$x0
000147254 9201_ $$0I:(DE-He78)HD01-20160331$$kHD01$$lDKTK HD zentral$$x1
000147254 980__ $$ajournal
000147254 980__ $$aVDB
000147254 980__ $$aI:(DE-He78)B062-20160331
000147254 980__ $$aI:(DE-He78)HD01-20160331
000147254 980__ $$aUNRESTRICTED