Cancer Management and Research (Sep 2022)

Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcomas: Standard of Care and Treatment Recommendations from the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG)

  • Ferrari A,
  • Brennan B,
  • Casanova M,
  • Corradini N,
  • Berlanga P,
  • Schoot RA,
  • Ramirez-Villar GL,
  • Safwat A,
  • Guillen Burrieza G,
  • Dall'Igna P,
  • Alaggio R,
  • Lyngsie Hjalgrim L,
  • Gatz SA,
  • Orbach D,
  • van Noesel MM

Journal volume & issue
Vol. Volume 14
pp. 2885 – 2902

Abstract

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Andrea Ferrari,1 Bernadette Brennan,2 Michela Casanova,1 Nadege Corradini,3 Pablo Berlanga,4 Reineke A Schoot,5 Gema L Ramirez-Villar,6 Akmal Safwat,7 Gabriela Guillen Burrieza,8 Patrizia Dall’Igna,9 Rita Alaggio,10 Lisa Lyngsie Hjalgrim,11 Susanne Andrea Gatz,12 Daniel Orbach,13 Max M van Noesel5 1Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy; 2Pediatric Oncology, Royal Manchester Children’s Hospital, Manchester, UK; 3Department of Pediatric Oncology, Institut d’Hematologie et d’Oncologie Pédiatrique/Centre, Léon Bérard, Lyon, France; 4Department of Pediatric and Adolescent Oncology, Gustave-Roussy, Cancer Campus, Université Paris-Saclay, Villejuif, France; 5Princess Máxima Center for Pediatric Oncology, Utrecht, Netherlands; 6Pediatric Oncology Unit, Hospital Universitario Virgen del Rocío, Seville, Spain; 7Oncology Department and Danish Center for Particle Therapy, Aarhus University Hospital, Aarhus, Denmark; 8Surgical Oncology and Neonatal Surgery, Pediatric Surgery Department, Hospital Infantil Universitari Vall d’Hebron, Barcelona, Spain; 9Department of Emergencies and Organ Transplantation, Pediatric Surgery, University of Bari, Bari, Italy; 10Pathology Department, Ospedale Pediatrico Bambino Gesù IRCCS, Rome, Italy; 11Department of Pediatrics and Adolescent Medicine, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark; 12Cancer Research UK Clinical Trials Unit, Institute of Cancer and Genomic Sciences, University of Birmingham, Birmingham, UK; 13SIREDO Oncology Center, Institut Curie, PSL University, Paris, FranceCorrespondence: Andrea Ferrari, Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Via G. Venezian, 1, Milan, MI, 20133, Italy, Tel +39 02 23902588, Fax +39 02 23902648, Email [email protected]: This paper describes the standard of care for patients with non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) and the therapeutic recommendations developed by the European paediatric Soft tissue sarcoma Study Group (EpSSG). NRSTS form a very mixed group of mesenchymal extraskeletal malignancies. Their rarity, heterogeneity, and aggressiveness make the management of children and adolescents with these tumors complex and challenging. The overall cure rate for patients with NRSTS is around 70%, but survival depends on several prognostic variables, such as histotype and tumor grade, extent of disease and stage, tumor size, and tumor site. While surgery remains the mainstay of treatment for most of these tumors, a multimodal therapeutic approach including radiotherapy and chemotherapy is required in many cases. The EpSSG NRSTS 2005 study was the first prospective protocol tailored specifically to NRSTS. Together with the ARST0332 study developed by the North-American Soft Tissue Sarcoma Committee of the Children’s Oncology Group (COG), the EpSSG NRSTS 2005 study currently represents the benchmark for these tumors, establishing risk-adapted standards of care. The EpSSG has developed common treatment recommendations for the large group of adult-type NRSTS (including synovial sarcoma), and specific treatment recommendations for other particular adult-type histologies (ie, alveolar soft-part sarcoma, clear cell sarcoma and dermatofibrosarcoma protuberans); other highly malignant tumors with a biology and clinical behavior differing from those of adult-type NRSTS (ie, rhabdoid tumors and desmoplastic small round cell tumor); and soft tissue tumors of intermediate malignancy (ie desmoid-type fibromatosis, inflammatory myofibroblastic tumors, and infantile fibrosarcoma). New effective drugs are needed for patients whose NRSTS carries the worst prognosis, ie, those with unresectable tumors, metastases at diagnosis, or relapsing disease. Progress in this area relies on our ability to develop international integrated prospective collaborations, both within existing pediatric oncology networks and, importantly, between the communities of specialists treating pediatric and adult sarcoma.Keywords: non-rhabdomyosarcoma soft tissue sarcomas, NRSTS, EpSSG, treatment, recommendations, pediatric

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