Journal Article

DKFZ-2023-00771

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Second-line treatment of pediatric patients with relapsed rhabdomyosarcoma adapted to initial risk stratification: Data of the European Soft Tissue Sarcoma Registry (SoTiSaR).

Heinz, A. T. ; Ebinger, M. ; Schönstein, A. ; Fuchs, J. ; Timmermann, B.DKFZ* ; Seitz, G. ; Vokuhl, C. ; Münter, M. W. ; Pajtler, K.DKFZ* ; Stegmaier, S. ; von Kalle, T. ; Kratz, C. P. ; Rößler, J. ; Ljungman, G. ; Klingebiel, T. ; Koscielniak, E. ; Sparber-Sauer, M. ; Studiengruppe, C. W. (Collaboration Author)

2023
Wiley New York, NY

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Please use a persistent id in citations: doi:10.1002/pbc.30363

Abstract: 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.

Keyword(s): Soft Tissue Sarcoma Registry (SoTiSaR) ; localized disease ; relapsed disease ; rhabdomyosarcoma ; second-line chemotherapy

Note: 2023 Jul;70(7):e30363

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Contributing Institute(s):

1. DKTK ED ES zentral (ED01)
2. DKTK HD zentral (HD01)
3. B062 Pädiatrische Neuroonkologie (B062)

Research Program(s):

1. 312 - Funktionelle und strukturelle Genomforschung (POF4-312) (POF4-312)

Appears in the scientific report 2023

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Database coverage:
; BIOSIS Previews ; Biological Abstracts ; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; DEAL Wiley ; Essential Science Indicators ; IF < 5 ; JCR ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection

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