Second-line treatment of pediatric patients with relapsed rhabdomyosarcoma adapted to initial risk stratification: Data of the European Soft Tissue Sarcoma Registry (SoTiSaR).

Heinz, Amadeus T; Ebinger, Martin; Schönstein, Anton; Fuchs, Jörg; Timmermann, Beate; Seitz, Guido; Vokuhl, Christian; Münter, Marc W; Pajtler, Kristian W; Stegmaier, Sabine; von Kalle, Thekla; Kratz, Christian P; Rößler, Jochen; Ljungman, Gustaf; Klingebiel, Thomas; Koscielniak, Ewa; Sparber-Sauer, Monika (2023). Second-line treatment of pediatric patients with relapsed rhabdomyosarcoma adapted to initial risk stratification: Data of the European Soft Tissue Sarcoma Registry (SoTiSaR). Pediatric blood & cancer, 70(7), e30363. Wiley 10.1002/pbc.30363

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BACKGROUND

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.

METHODS

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.

RESULTS

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.

CONCLUSION

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.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine
04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine > Paediatric Haematology/Oncology

UniBE Contributor:

Rössler, Jochen Karl

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1545-5017

Publisher:

Wiley

Language:

English

Submitter:

Pubmed Import

Date Deposited:

18 Apr 2023 10:03

Last Modified:

24 May 2023 00:15

Publisher DOI:

10.1002/pbc.30363

PubMed ID:

37066598

Uncontrolled Keywords:

Soft Tissue Sarcoma Registry (SoTiSaR) localized disease relapsed disease rhabdomyosarcoma second-line chemotherapy

BORIS DOI:

10.48350/181807

URI:

https://boris.unibe.ch/id/eprint/181807

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