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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
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336 7 _ |a article
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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.
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650 _ 7 |a Soft Tissue Sarcoma Registry (SoTiSaR)
|2 Other
650 _ 7 |a localized disease
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650 _ 7 |a relapsed disease
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650 _ 7 |a rhabdomyosarcoma
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650 _ 7 |a second-line chemotherapy
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700 1 _ |a Ebinger, Martin
|0 0000-0002-4229-8058
|b 1
700 1 _ |a Schönstein, Anton
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700 1 _ |a Fuchs, Jörg
|b 3
700 1 _ |a Timmermann, Beate
|0 P:(DE-HGF)0
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700 1 _ |a Seitz, Guido
|b 5
700 1 _ |a Vokuhl, Christian
|0 0000-0002-4138-4536
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700 1 _ |a Münter, Marc W
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700 1 _ |a Pajtler, Kristian
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700 1 _ |a Stegmaier, Sabine
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700 1 _ |a von Kalle, Thekla
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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
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700 1 _ |a Studiengruppe, Cooperative Weichteilsarkom
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773 _ _ |a 10.1002/pbc.30363
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Marc 21