Dermatofibrosarcoma protuberans in children and adolescents: The European Paediatric Soft Tissue Sarcoma Study Group prospective trial (EpSSG NRSTS 2005)

Bernadette Brennan, Ilaria Zanetti, Gian Luca De Salvo, Daniel Orbach, Soledad Gallego, Nadine Francotte, Stefan Schifflers, Max Van Noesel, Anna Kelsey, Michela Casanova, Gian Paolo Dagrada, Paola Collini, Angelica Zin, Luisa Santoro, Andrea Ferrari

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Article numbere28351
Pages (from-to)1-5
Number of pages5
JournalPediatric Blood and Cancer
Volume67
Issue number10
DOIs
Publication statusPublished - Oct 1 2020

Keywords

  • adolescents
  • dermatofibrosarcoma protuberans
  • paediatric

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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