Cancer Management and Research (Oct 2023)

Treatment at Relapse for Synovial Sarcoma of Children, Adolescents and Young Adults: From the State of Art to Future Clinical Perspectives

  • Ferrari A,
  • Berlanga P,
  • Gatz SA,
  • Schoot RA,
  • van Noesel MM,
  • Hovsepyan S,
  • Chiaravalli S,
  • Bergamaschi L,
  • Minard-Colin V,
  • Corradini N,
  • Alaggio R,
  • Gasparini P,
  • Brennan B,
  • Casanova M,
  • Pasquali S,
  • Orbach D

Journal volume & issue
Vol. Volume 15
pp. 1183 – 1196

Abstract

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Andrea Ferrari,1 Pablo Berlanga,2 Susanne Andrea Gatz,3 Reineke A Schoot,4 Max M van Noesel,4,5 Shushan Hovsepyan,6 Stefano Chiaravalli,1 Luca Bergamaschi,1 Veronique Minard-Colin,2 Nadege Corradini,7 Rita Alaggio,8 Patrizia Gasparini,9 Bernadette Brennan,10 Michela Casanova,1 Sandro Pasquali,11,12 Daniel Orbach13 1Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy; 2Department of Pediatric and Adolescent Oncology, Gustave Roussy Cancer Campus, Université Paris-Saclay, Villejuif, France; 3Cancer Research UK Clinical Trials Unit, Institute of Cancer and Genomic Sciences, University of Birmingham, Birmingham, UK; 4Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands; 5Division Imaging & Cancer, University Medical Center Utrecht, Utrecht, the Netherlands; 6Pediatric Cancer and Blood Disorders Center of Armenia, Yerevan, Armenia; 7Department of Pediatric Hematology and Oncology-IHOPe, Léon Bérard Center, Lyon, France; 8Pathology Department, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy; 9Tumor Genomics Unit, Department of Research, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy; 10Pediatric Oncology, Royal Manchester Children’s Hospital, Manchester, UK; 11Molecular Pharmacology Unit, Department of Applied Research and Technological Development, Molecular Pharmacology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy; 12Sarcoma Service, Department of Surgery, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy; 13SIREDO Oncology Center(Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Institut Curie, PSL University, Paris, FranceCorrespondence: Andrea Ferrari, Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via G. Venezian, Milano, MI, 1 20133, Italy, Tel +39 02 23902588, Fax +39 02 23902648, Email [email protected]: While the overall prognosis is generally quite satisfactory in children, adolescents and young adults with localised synovial sarcoma at first diagnosis, the outcome remains poor for patients after relapse. Conversely to the front-line standardised treatment options, patients with relapse generally have an individualised approach and to date, there is still a lack of consensus regarding standard treatment approaches. Studies on relapsed synovial sarcoma were able to identify some prognostic variables that influence post-relapse survival, in order to plan risk-adapted salvage protocols. Treatment proposals must consider previous first-line treatments, potential toxicities, and the possibility of achieving an adequate local treatment by new surgery and/or re-irradiation. Effective second-line drug therapies are urgently needed. Notably, experimental treatments such as adoptive engineered TCR-T cell immunotherapy seem promising in adults and are currently under validation also in paediatric patients.Keywords: synovial sarcoma, relapse, second line therapy, surgery, new agents, TCR-T cell therapy, children

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