Bernadette Brennan1, Ilaria Zanetti2, Gian Luca De Salvo2, Daniel Orbach3, Soledad Gallego4, Nadine Francotte5, Stefan Schifflers5, Max Van Noesel6, Anna Kelsey7, Michela Casanova8, Gian Paolo Dagrada9, Paola Collini9, Angelica Zin10, Luisa Santoro11, Andrea Ferrari8. 1. Paediatric Oncology, Royal Manchester Children's Hospital, Manchester, UK. 2. Clinical Trials and Biostatistics Unit, IRCCS Istituto Oncologico Veneto, Padua, Italy. 3. SIREDO oncology centre, Institut Curie, Paris, France. 4. Department of Paediatric Hematology/Oncology of Hospital Vall d'Hebron, Barcelona, Spain. 5. Department of Pediatrics, CHC-Clinique Esperance, Montegnée, Belgium. 6. Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands. 7. Department of Diagnostic Paediatric Histopathology, Royal Manchester Children's Hospital, Manchester, UK. 8. Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy. 9. Histopathology and Pediatric Pathology Unit, Department of Diagnostic Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. 10. Fondazione Istituto di Ricerca Pediatrica Città della Speranza, Padua, Italy. 11. Istituto di Anatomia Patologica, Universitá di Padova, Padua, Italy.
Abstract
BACKGROUND: As dermatofibrosarcoma protuberans (DFSP) are rare with no prospective series within paediatric sarcoma trials, the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) examined the clinical data and outcomes of DFSP enrolled in a multinational study of non-rhabdomyosarcoma soft tissue sarcomas (NRSTS). PATIENTS AND METHODS: Forty-six patients with confirmed DFSP were enrolled into the EpSSG NRSTS 2005 study. All had surgical resection and none had any further therapy at diagnosis. RESULTS: The median age at diagnosis was 6.9 years (range 0.4-17.5). All patients had localised disease, and the majority had small <5 cm tumours (93%), and 76% had Intergroup Rhabdomyosarcoma Study (IRS) I tumours. All patients had up front surgery, 32 requiring two operations. There were 11 patients with IRS II tumours, of which only two went on to have a local recurrence. After a median follow up of 49.0 months (range 4.2-130.9), all patients were alive at the time of this report, with 5-year event-free survival of 92.6% (CI 78.8-97.6) with a 100% overall survival. CONCLUSION: This report demonstrates the ability to run prospective paediatric studies in NRSTS in multiple European countries, with reasonable numbers of DFSP patients, with few events and no deaths, and hence excellent outcomes.
BACKGROUND: As dermatofibrosarcoma protuberans (DFSP) are rare with no prospective series within paediatric sarcoma trials, the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) examined the clinical data and outcomes of DFSP enrolled in a multinational study of non-rhabdomyosarcoma soft tissue sarcomas (NRSTS). PATIENTS AND METHODS: Forty-six patients with confirmed DFSP were enrolled into the EpSSG NRSTS 2005 study. All had surgical resection and none had any further therapy at diagnosis. RESULTS: The median age at diagnosis was 6.9 years (range 0.4-17.5). All patients had localised disease, and the majority had small <5 cm tumours (93%), and 76% had Intergroup Rhabdomyosarcoma Study (IRS) I tumours. All patients had up front surgery, 32 requiring two operations. There were 11 patients with IRS II tumours, of which only two went on to have a local recurrence. After a median follow up of 49.0 months (range 4.2-130.9), all patients were alive at the time of this report, with 5-year event-free survival of 92.6% (CI 78.8-97.6) with a 100% overall survival. CONCLUSION: This report demonstrates the ability to run prospective paediatric studies in NRSTS in multiple European countries, with reasonable numbers of DFSPpatients, with few events and no deaths, and hence excellent outcomes.
Authors: Andrea Ferrari; Bernadette Brennan; Michela Casanova; Nadege Corradini; Pablo Berlanga; Reineke A Schoot; Gema L Ramirez-Villar; Akmal Safwat; Gabriela Guillen Burrieza; Patrizia Dall'Igna; Rita Alaggio; Lisa Lyngsie Hjalgrim; Susanne Andrea Gatz; Daniel Orbach; Max M van Noesel Journal: Cancer Manag Res Date: 2022-09-23 Impact factor: 3.602