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Second-line treatment of pediatric patients with relapsed rhabdomyosarcoma adapted to initial risk stratification: Data of the European Soft Tissue Sarcoma Registry (SoTiSaR)
Authors:Amadeus T. Heinz  Martin Ebinger  Anton Schönstein  Jörg Fuchs  Beate Timmermann  Guido Seitz  Christian Vokuhl  Marc W. Münter  Kristian W. Pajtler  Sabine Stegmaier  Thekla von Kalle  Christian P. Kratz  Jochen Rößler  Gustaf Ljungman  Thomas Klingebiel  Ewa Koscielniak  Monika Sparber-Sauer  the Cooperative Weichteilsarkom Studiengruppe (CWS)
Affiliation:1. Department of Pediatric Hematology and Oncology, University Children´s Hospital Tuebingen, Tuebingen, Germany;2. Network Aging Research, Heidelberg University, Heidelberg, Germany;3. Department of Pediatric Surgery and Urology, University Children's Hospital Tuebingen, Tuebingen, Germany;4. Department of Particle Therapy, West German Proton Therapy Centre Essen (WPE), University Medical Center Essen, West German Cancer Center (WTZ), German Cancer Consortium (DKTK), Essen, Germany;5. Department of Pediatric Surgery, University Hospital Giessen-Marburg, Marburg, Germany;6. Section of Pediatric Pathology, University Hospital Bonn, Bonn, Germany;7. Department of Radiation Oncology, Stuttgart Cancer Center, Klinikum der Landeshauptstadt Stuttgart, Stuttgart, Germany;8. Hopp Children's Cancer Center Heidelberg (KiTZ), Heidelberg University, Heidelberg, Germany

Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK), Heidelberg University, Heidelberg, Germany

Department of Pediatric Oncology, Hematology, and Immunology, Heidelberg University Hospital, Heidelberg, Germany;9. Stuttgart Cancer Center, Zentrum für Kinder-, Jugend- und Frauenmedizin (Olgahospital), Pädiatrie 5 (Pädiatrische Onkologie, Hämatologie, Immunologie), Klinikum der Landeshauptstadt Stuttgart, Stuttgart, Germany;10. Stuttgart Cancer Center, Zentrum für Kinder-, Jugend- und Frauenmedizin (Olgahospital), Department of Radiology, Klinikum der Landeshauptstadt Stuttgart, Stuttgart, Germany;11. Pediatric Hematology and Oncology, Hannover Medical School, Hannover, Germany;12. Division of Pediatric Hematology and Oncology, Department of Pediatrics, University Hospital Bern (Inselspital), Bern, Switzerland;13. Department of Women's and Children's Health, Children's University Hospital, University of Uppsala, Uppsala, Sweden;14. Department of Children and Adolescents, University Hospital of Frankfurt, Frankfurt, Germany
Abstract:

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