Maintenance Chemotherapy for Patients with Rhabdomyosarcoma

Gianni Bisogno; Veronique Minard-Colin; Meriel Jenney; Andrea Ferrari; Julia Chisholm; Daniela Di Carlo; Lisa Lyngsie Hjalgrim; Daniel Orbach; Johannes Hendrikus Maria Merks; Michela Casanova

doi:10.3390/cancers15154012

Maintenance Chemotherapy for Patients with Rhabdomyosarcoma

Cancers (Basel). 2023 Aug 7;15(15):4012. doi: 10.3390/cancers15154012.

Authors

Affiliations

¹ Department of Women's and Children's Health, University of Padua, 35128 Padua, Italy.
² Pediatric Hematology Oncology Division, University Hospital of Padua, 35128 Padua, Italy.
³ Department of Pediatric and Adolescent Oncology, Institut Gustave-Roussy, Université Paris-Saclay, 94800 Villejuif, France.
⁴ Department of Paediatric Oncology, Children's Hospital for Wales, Heath Park, Cardiff CF14 4XW, UK.
⁵ Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, 20133 Milan, Italy.
⁶ Children and Young People's Unit, Royal Marsden Hospital and Institute of Cancer Research, Sutton SM2 5PT, UK.
⁷ Department of Paediatric and Adolescent Medicine, University Hospital Copenhagen, 2100 Copenhagen, Denmark.
⁸ SIREDO Oncology Centre (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Institut Curie, Paris Sciences et Lettres L University, 75005 Paris, France.
⁹ Princess Máxima Centre for Pediatric Oncology, 3584 CS Utrecht, The Netherlands.
¹⁰ Division of Imaging and Oncology, University Medical Center Utrecht, 3584 CX Utrecht, The Netherlands.

Abstract

Maintenance chemotherapy (MC) defines the administration of prolonged relatively low-intensity chemotherapy with the aim of "maintaining" tumor complete remission. This paper aims to report an update of the RMS2005 trial, which demonstrated better survival for patients with high-risk localized rhabdomyosarcoma (RMS) when MC with vinorelbine and low-dose cyclophosphamide was added to standard chemotherapy, and to discuss the published experience on MC in RMS. In the RMS2005 study, the outcome for patients receiving MC vs. those who stopped the treatment remains superior, with a 5-year disease-free survival of 78.1% vs. 70.1% (p = 0.056) and overall survival of 85.0% vs. 72.4% (p = 0.008), respectively. We found seven papers describing MC in RMS, but only one randomized trial that did not demonstrate any advantage when MC with eight courses of trofosfamide/idarubicine alternating with trofosfamide/etoposide has been employed in high-risk RMS. The use of MC showed better results in comparison to high-dose chemotherapy in non-randomized studies, including metastatic patients, and demonstrated feasibility and tolerability in relapsed RMS. Many aspects of MC in RMS need to be investigated, including the best drug combination and the optimal duration. The ongoing EpSSG trial will try to answer some of these questions.

Keywords: low-dose chemotherapy; maintenance chemotherapy; metronomic chemotherapy; rhabdomyosarcoma.

Publication types

Review

Grants and funding

J.C. is supported by The Giant Pledge through the Royal Marsden Cancer Charity. This work represents independent research supported by the National Institute for Health Research (NIHR) Biomedical Research Centre at The Royal Marsden NHS Foundation Trust and the Institute of Cancer Research, London. The views expressed are those of the authors and not necessarily those of the NIHR or the Department of Health and Social Care. Fondazione Città della Speranza, Italy, has funded the trial management (recipient: G.B.), and Alice’s Arc, a children’s cancer charity focusing on rhabdomyosarcoma, United Kingdom (alicesarc.org), has funded data management and statistical processing for current RMS2005 study analyses.