Metastatic Rhabdomyosarcoma: Results of the European Paediatric Soft Tissue Sarcoma Study Group MTS 2008 Study and Pooled Analysis With the Concurrent BERNIE Study
| Autor: | Reineke A. Schoot, Julia C. Chisholm, Michela Casanova, Veronique Minard-Colin, Birgit Geoerger, Alison L. Cameron, Beatrice Coppadoro, Ilaria Zanetti, Daniel Orbach, Anna Kelsey, Timothy Rogers, Cecile Guizani, Markus Elze, Myriam Ben-Arush, Kieran McHugh, Rick R. van Rijn, Sima Ferman, Soledad Gallego, Andrea Ferrari, Meriel Jenney, Gianni Bisogno, Johannes H.M. Merks |
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| Přispěvatelé: | Institut Català de la Salut, [Schoot RA] Princess Máxima Centre for Paediatric Oncology, Utrecht, the Netherlands. [Chisholm JC] Children and Young Peoples Unit, Royal Marsden Hospital and Institute of Cancer Research, Sutton, Surrey, United Kingdom. [Casanova M] Paediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. [Minard-Colin V] Gustave-Roussy Cancer Campus, Department of Paediatric and Adolescent Oncology, Université Paris-Saclay, Villejuif, France. [Geoerger B] Gustave-Roussy Cancer Campus, Department of Paediatric and Adolescent Oncology, Université Paris-Saclay, Villejuif, France. Gustave-Roussy Cancer Campus, INSERM U1015, Université Paris Saclay, Villejuif, France. [Cameron AL] Bristol Haematology and Oncology Centre, University Hospitals Bristol. [Gallego S] Servei d’Oncologia i Hematologia Pediàtriques, Vall d’Hebron Hospital Universitari, Barcelona, Spain, Vall d'Hebron Barcelona Hospital Campus, Radiology and Nuclear Medicine, Other Research |
| Jazyk: | angličtina |
| Rok vydání: | 2022 |
| Předmět: |
Cancer Research
Otros calificadores::Otros calificadores::/farmacoterapia [Otros calificadores] Other subheadings::Other subheadings::/drug therapy [Other subheadings] neoplasias::neoplasias por tipo histológico::neoplasias de tejido conjuntivo y de tejidos blandos::neoplasias de tejido muscular::miosarcoma::rabdomiosarcoma [ENFERMEDADES] Otros calificadores::Otros calificadores::/efectos adversos [Otros calificadores] Disease-Free Survival Tumors de parts toves - Tractament Quimioteràpia combinada Neoplasms Therapeutics::Therapeutics::Drug Therapy::Antineoplastic Protocols::Therapeutics::Drug Therapy::Antineoplastic Combined Chemotherapy Protocols [ANALYTICAL DIAGNOSTIC AND THERAPEUTIC TECHNIQUES AND EQUIPMENT] Antineoplastic Combined Chemotherapy Protocols Rhabdomyosarcoma Other subheadings::Other subheadings::/adverse effects [Other subheadings] Humans Sarcoma - Tractament Ifosfamide Child Neoplasms::Neoplasms by Histologic Type::Neoplasms Connective and Soft Tissue::Neoplasms Muscle Tissue::Myosarcoma::Rhabdomyosarcoma [DISEASES] Cyclophosphamide terapéutica::terapéutica::farmacoterapia::protocolos antineoplásicos::terapéutica::farmacoterapia::protocolos de quimioterapia antineoplásica combinada [TÉCNICAS Y EQUIPOS ANALÍTICOS DIAGNÓSTICOS Y TERAPÉUTICOS] Sarcoma Second Primary Oncology Vincristine Dactinomycin Doxorubicin Neoplasms Second Primary |
| Zdroj: | Scientia Journal of clinical oncology, 19(32):JCO.21.02981, 3730-3740. American Society of Clinical Oncology |
| ISSN: | 0732-183X |
| Popis: | PURPOSE Outcome for patients with metastatic rhabdomyosarcoma (RMS) is poor. This study presents the results of the MTS 2008 study with a pooled analysis including patients from the concurrent BERNIE study. PATIENTS AND METHODS In MTS 2008, patients with metastatic RMS received four cycles of ifosfamide, vincristine, and actinomycin D (IVA) plus doxorubicin, five cycles of IVA, and 12 cycles of maintenance chemotherapy (low-dose cyclophosphamide and vinorelbine). The BERNIE study randomly assigned patients to the addition or not of bevacizumab to the same chemotherapy. Local therapy (surgery/radiotherapy) was given to the primary tumor and all metastatic sites when feasible. RESULTS MTS 2008 included 270 patients (median age, 9.6 years; range, 0.07-20.8 years). With a median follow-up of 50.3 months, 3-year event-free survival (EFS) and overall survival (OS) were 34.9% (95% CI, 29.1 to 40.8) and 47.9% (95% CI, 41.6 to 53.9), respectively. In pooled analyses on 372 patients with a median follow-up of 55.2 months, 3-year EFS and OS were 35.5% (95% CI, 30.4 to 40.6) and 49.3% (95% CI, 43.9 to 54.5), respectively. Patients with ≤ 2 Oberlin risk factors (ORFs) had better outcome than those with ≥ 3 ORFs: 3-year EFS was 46.1% versus 12.5% ( P < .0001) and 3-year OS 60.0% versus 26.0% ( P < .0001). Induction chemotherapy and maintenance appeared tolerable; however, about two third of patients needed dose adjustments during maintenance. CONCLUSION Outcome remains poor for patients with metastatic RMS and multiple ORFs. Because of the design of the studies, it was not possible to determine whether the intensive induction regimen and/or the addition of maintenance treatment resulted in apparent improvement of outcome compared with historical cohorts. Further studies, with novel treatment approaches are urgently needed, to improve outcome for the group of patients with adverse prognostic factors. |
| Databáze: | OpenAIRE |
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