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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$$c2023
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000275451 500__ $$a2023 Jul;70(7):e30363
000275451 520__ $$aOutcome of relapsed disease of localized rhabdomyosarcoma remains poor. An individual treatment approach considering the initial systemic treatment and risk group was included in the Cooperative Weichteilsarkom Studiengruppe (CWS) Guidance.Second-line chemotherapy (sCHT) ACCTTIVE based on anthracyclines (adriamycin, carboplatin, cyclophosphamide, topotecan, vincristine, etoposide) was recommended for patients with initial low- (LR), standard- (SR), and high-risk (HR) group after initial treatment without anthracyclines. TECC (topotecan, etoposide, carboplatin, cyclophosphamide) was recommended after initial anthracycline-based regimen in the very high-risk (VHR) group. Data of patients with relapse (n = 68) registered in the European Soft Tissue Sarcoma Registry SoTiSaR (2009-2018) were retrospectively analyzed.Patients of initial LR (n = 2), SR (n = 16), HR (n = 41), and VHR (n = 9) group relapsed. sCHT consisted of ACCTTIVE (n = 36), TECC (n = 12), or other (n = 15). Resection was performed in 40/68 (59%) patients and/or radiotherapy in 47/68 (69%). Initial risk stratification, pattern/time to relapse, and achievement of second complete remission were significant prognostic factors. Microscopically incomplete resection with additional radiotherapy was not inferior to microscopically complete resection (p = .17). The 5-year event-free survival (EFS) and overall survival (OS) were 26% (±12%) and 31% (±14%). The 5-year OS of patients with relapse of SR, HR, and VHR groups was 80% (±21%), 20% (±16%), and 13% (±23%, p = .008), respectively.Adapted systemic treatment of relapsed disease considering the initial risk group and initial treatment is reasonable. New treatment options are needed for patients of initial HR and VHR groups.
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000275451 650_7 $$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
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