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024 | 7 | _ | |a 1545-5017 |2 ISSN |
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037 | _ | _ | |a DKFZ-2023-00771 |
041 | _ | _ | |a English |
082 | _ | _ | |a 610 |
100 | 1 | _ | |a Heinz, Amadeus T |0 0000-0003-3472-9679 |b 0 |
245 | _ | _ | |a Second-line treatment of pediatric patients with relapsed rhabdomyosarcoma adapted to initial risk stratification: Data of the European Soft Tissue Sarcoma Registry (SoTiSaR). |
260 | _ | _ | |a New York, NY |c 2023 |b Wiley |
336 | 7 | _ | |a article |2 DRIVER |
336 | 7 | _ | |a Output Types/Journal article |2 DataCite |
336 | 7 | _ | |a Journal Article |b journal |m journal |0 PUB:(DE-HGF)16 |s 1685020331_26037 |2 PUB:(DE-HGF) |
336 | 7 | _ | |a ARTICLE |2 BibTeX |
336 | 7 | _ | |a JOURNAL_ARTICLE |2 ORCID |
336 | 7 | _ | |a Journal Article |0 0 |2 EndNote |
500 | _ | _ | |a 2023 Jul;70(7):e30363 |
520 | _ | _ | |a Outcome 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. |
536 | _ | _ | |a 312 - Funktionelle und strukturelle Genomforschung (POF4-312) |0 G:(DE-HGF)POF4-312 |c POF4-312 |f POF IV |x 0 |
588 | _ | _ | |a Dataset connected to CrossRef, PubMed, , Journals: inrepo02.dkfz.de |
650 | _ | 7 | |a Soft Tissue Sarcoma Registry (SoTiSaR) |2 Other |
650 | _ | 7 | |a localized disease |2 Other |
650 | _ | 7 | |a relapsed disease |2 Other |
650 | _ | 7 | |a rhabdomyosarcoma |2 Other |
650 | _ | 7 | |a second-line chemotherapy |2 Other |
700 | 1 | _ | |a Ebinger, Martin |0 0000-0002-4229-8058 |b 1 |
700 | 1 | _ | |a Schönstein, Anton |b 2 |
700 | 1 | _ | |a Fuchs, Jörg |b 3 |
700 | 1 | _ | |a Timmermann, Beate |0 P:(DE-HGF)0 |b 4 |
700 | 1 | _ | |a Seitz, Guido |b 5 |
700 | 1 | _ | |a Vokuhl, Christian |0 0000-0002-4138-4536 |b 6 |
700 | 1 | _ | |a Münter, Marc W |b 7 |
700 | 1 | _ | |a Pajtler, Kristian |0 P:(DE-He78)a7c1bbac024fa232d9c6b78443328d9d |b 8 |u dkfz |
700 | 1 | _ | |a Stegmaier, Sabine |0 0000-0001-7281-8811 |b 9 |
700 | 1 | _ | |a von Kalle, Thekla |b 10 |
700 | 1 | _ | |a Kratz, Christian P |b 11 |
700 | 1 | _ | |a Rößler, Jochen |0 0000-0003-4022-4917 |b 12 |
700 | 1 | _ | |a Ljungman, Gustaf |b 13 |
700 | 1 | _ | |a Klingebiel, Thomas |b 14 |
700 | 1 | _ | |a Koscielniak, Ewa |0 0000-0002-1519-7569 |b 15 |
700 | 1 | _ | |a Sparber-Sauer, Monika |0 0000-0001-9551-2399 |b 16 |
700 | 1 | _ | |a Studiengruppe, Cooperative Weichteilsarkom |b 17 |e Collaboration Author |
773 | _ | _ | |a 10.1002/pbc.30363 |g p. e30363 |0 PERI:(DE-600)2130978-4 |n 7 |p e30363 |t Pediatric blood & cancer |v 70 |y 2023 |x 1545-5009 |
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