000275451 001__ 275451 000275451 005__ 20240229154941.0 000275451 0247_ $$2doi$$a10.1002/pbc.30363 000275451 0247_ $$2pmid$$apmid:37066598 000275451 0247_ $$2ISSN$$a1545-5009 000275451 0247_ $$2ISSN$$a1545-5017 000275451 0247_ $$2altmetric$$aaltmetric:145941980 000275451 037__ $$aDKFZ-2023-00771 000275451 041__ $$aEnglish 000275451 082__ $$a610 000275451 1001_ $$00000-0003-3472-9679$$aHeinz, Amadeus T$$b0 000275451 245__ $$aSecond-line treatment of pediatric patients with relapsed rhabdomyosarcoma adapted to initial risk stratification: Data of the European Soft Tissue Sarcoma Registry (SoTiSaR). 000275451 260__ $$aNew York, NY$$bWiley$$c2023 000275451 3367_ $$2DRIVER$$aarticle 000275451 3367_ $$2DataCite$$aOutput Types/Journal article 000275451 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1685020331_26037 000275451 3367_ $$2BibTeX$$aARTICLE 000275451 3367_ $$2ORCID$$aJOURNAL_ARTICLE 000275451 3367_ $$00$$2EndNote$$aJournal Article 000275451 500__ $$a2023 Jul;70(7):e30363 000275451 520__ $$aOutcome of relapsed disease of localized rhabdomyosarcoma remains poor. An individual treatment approach considering the initial systemic treatment and risk group was included in the Cooperative Weichteilsarkom Studiengruppe (CWS) Guidance.Second-line chemotherapy (sCHT) ACCTTIVE based on anthracyclines (adriamycin, carboplatin, cyclophosphamide, topotecan, vincristine, etoposide) was recommended for patients with initial low- (LR), standard- (SR), and high-risk (HR) group after initial treatment without anthracyclines. TECC (topotecan, etoposide, carboplatin, cyclophosphamide) was recommended after initial anthracycline-based regimen in the very high-risk (VHR) group. Data of patients with relapse (n = 68) registered in the European Soft Tissue Sarcoma Registry SoTiSaR (2009-2018) were retrospectively analyzed.Patients of initial LR (n = 2), SR (n = 16), HR (n = 41), and VHR (n = 9) group relapsed. sCHT consisted of ACCTTIVE (n = 36), TECC (n = 12), or other (n = 15). Resection was performed in 40/68 (59%) patients and/or radiotherapy in 47/68 (69%). Initial risk stratification, pattern/time to relapse, and achievement of second complete remission were significant prognostic factors. Microscopically incomplete resection with additional radiotherapy was not inferior to microscopically complete resection (p = .17). The 5-year event-free survival (EFS) and overall survival (OS) were 26% (±12%) and 31% (±14%). The 5-year OS of patients with relapse of SR, HR, and VHR groups was 80% (±21%), 20% (±16%), and 13% (±23%, p = .008), respectively.Adapted systemic treatment of relapsed disease considering the initial risk group and initial treatment is reasonable. New treatment options are needed for patients of initial HR and VHR groups. 000275451 536__ $$0G:(DE-HGF)POF4-312$$a312 - Funktionelle und strukturelle Genomforschung (POF4-312)$$cPOF4-312$$fPOF IV$$x0 000275451 588__ $$aDataset connected to CrossRef, PubMed, , Journals: inrepo02.dkfz.de 000275451 650_7 $$2Other$$aSoft Tissue Sarcoma Registry (SoTiSaR) 000275451 650_7 $$2Other$$alocalized disease 000275451 650_7 $$2Other$$arelapsed disease 000275451 650_7 $$2Other$$arhabdomyosarcoma 000275451 650_7 $$2Other$$asecond-line chemotherapy 000275451 7001_ $$00000-0002-4229-8058$$aEbinger, Martin$$b1 000275451 7001_ $$aSchönstein, Anton$$b2 000275451 7001_ $$aFuchs, Jörg$$b3 000275451 7001_ $$0P:(DE-HGF)0$$aTimmermann, Beate$$b4 000275451 7001_ $$aSeitz, Guido$$b5 000275451 7001_ $$00000-0002-4138-4536$$aVokuhl, Christian$$b6 000275451 7001_ $$aMünter, Marc W$$b7 000275451 7001_ $$0P:(DE-He78)a7c1bbac024fa232d9c6b78443328d9d$$aPajtler, Kristian$$b8$$udkfz 000275451 7001_ $$00000-0001-7281-8811$$aStegmaier, Sabine$$b9 000275451 7001_ $$avon Kalle, Thekla$$b10 000275451 7001_ $$aKratz, Christian P$$b11 000275451 7001_ $$00000-0003-4022-4917$$aRößler, Jochen$$b12 000275451 7001_ $$aLjungman, Gustaf$$b13 000275451 7001_ $$aKlingebiel, Thomas$$b14 000275451 7001_ $$00000-0002-1519-7569$$aKoscielniak, Ewa$$b15 000275451 7001_ $$00000-0001-9551-2399$$aSparber-Sauer, Monika$$b16 000275451 7001_ $$aStudiengruppe, Cooperative Weichteilsarkom$$b17$$eCollaboration Author 000275451 773__ $$0PERI:(DE-600)2130978-4$$a10.1002/pbc.30363$$gp. e30363$$n7$$pe30363$$tPediatric blood & cancer$$v70$$x1545-5009$$y2023 000275451 909CO $$ooai:inrepo02.dkfz.de:275451$$pVDB 000275451 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-HGF)0$$aDeutsches Krebsforschungszentrum$$b4$$kDKFZ 000275451 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)a7c1bbac024fa232d9c6b78443328d9d$$aDeutsches Krebsforschungszentrum$$b8$$kDKFZ 000275451 9131_ $$0G:(DE-HGF)POF4-312$$1G:(DE-HGF)POF4-310$$2G:(DE-HGF)POF4-300$$3G:(DE-HGF)POF4$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lKrebsforschung$$vFunktionelle und strukturelle Genomforschung$$x0 000275451 9141_ $$y2023 000275451 915__ $$0StatID:(DE-HGF)3001$$2StatID$$aDEAL Wiley$$d2023-03-30$$wger 000275451 915__ $$0StatID:(DE-HGF)1190$$2StatID$$aDBCoverage$$bBiological Abstracts$$d2023-03-30 000275451 915__ $$0StatID:(DE-HGF)0113$$2StatID$$aWoS$$bScience Citation Index Expanded$$d2023-03-30 000275451 915__ $$0StatID:(DE-HGF)0160$$2StatID$$aDBCoverage$$bEssential Science Indicators$$d2023-03-30 000275451 915__ $$0StatID:(DE-HGF)0100$$2StatID$$aJCR$$bPEDIATR BLOOD CANCER : 2022$$d2023-10-25 000275451 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS$$d2023-10-25 000275451 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline$$d2023-10-25 000275451 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bClarivate Analytics Master Journal List$$d2023-10-25 000275451 915__ $$0StatID:(DE-HGF)1050$$2StatID$$aDBCoverage$$bBIOSIS Previews$$d2023-10-25 000275451 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection$$d2023-10-25 000275451 915__ $$0StatID:(DE-HGF)1110$$2StatID$$aDBCoverage$$bCurrent Contents - Clinical Medicine$$d2023-10-25 000275451 915__ $$0StatID:(DE-HGF)9900$$2StatID$$aIF < 5$$d2023-10-25 000275451 9201_ $$0I:(DE-He78)ED01-20160331$$kED01$$lDKTK ED ES zentral$$x0 000275451 9201_ $$0I:(DE-He78)HD01-20160331$$kHD01$$lDKTK HD zentral$$x1 000275451 9201_ $$0I:(DE-He78)B062-20160331$$kB062$$lB062 Pädiatrische Neuroonkologie$$x2 000275451 980__ $$ajournal 000275451 980__ $$aVDB 000275451 980__ $$aI:(DE-He78)ED01-20160331 000275451 980__ $$aI:(DE-He78)HD01-20160331 000275451 980__ $$aI:(DE-He78)B062-20160331 000275451 980__ $$aUNRESTRICTED