20 July 2020

We study the RARE Paediatric cancers: check about the DERMATOFIBROSARCOMA PROTUBERANS!

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

Which is our new published paper?
Dermatofibrosarcoma protuberans (DFSP) are rare soft tissue tumours which often present as a soft tissue lump under the skin. Therefore the EpSSG examined the clinical data and outcomes of DFSP enrolled in our multinational study of non-rhabdomyosarcoma soft tissue sarcomas (NRSTS). 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. The average age of the children was nearly 7 years. All children had no spread beyond the site of the primary lump, and the majority had small < 5 cm tumours. All patients had surgery at diagnosis, 32 requiring 2 operations. After a median follow-up of 4 years, all patients were alive and in 2 who had relapsed they were treated with further surgery.
This report demonstrates the ability to run prospective paediatric studies in DFSP in multiple European countries, with reasonable numbers of DFSP patients, with few events and no deaths, and hence excellent outcomes.
Author: Authors: Bernadette Brennan, Anna Kelsey, Ilaria Zanetti, Andrea Ferrari and EpSSG colleagues