Epithelioid hemangioendothelioma in children: The European Pediatric Soft Tissue Sarcoma Study Group experience

Daniel Orbach, Max M Van Noesel, Bernadette Brennan, Nadège Corradini, Rita Alaggio, Myriam Ben Arush, Reineke A Schoot, Pablo Berlanga, Ilaria Zanetti, Lisa Lyngsie Hjalgrim, Federica Di Corti, Gema Ramirez, Michela Casanova, Andrea Ferrari

Abstract

The European pediatric Soft tissue sarcoma Study Group analyzed all children with epithelioid hemangioendothelioma prospectively registered in the NRSTS-05 (EUDRACT 2005-001139-31) and in MTS-2008 (NCT00379457) studies: 10 patients with localized and one with metastatic disease. Median age was 14.3 years (range, 9.0-18.8). Local therapy was initial primary surgery in seven cases, and five patients received systemic therapy. No patients received radiotherapy. After a median follow-up of 50 months (range, 6-176) for living patients, nine patients remain alive off therapy and two died. Five-year progression free and overall survivals are, respectively, 77.1% (95% confidence interval [CI]: 34.5-93.9) and 74.1% (95% CI: 28.1-93.0).

OriginalsprogEngelsk
Artikelnummere29882
TidsskriftPediatric Blood & Cancer
Vol/bind69
Udgave nummer10
Sider (fra-til)1-4
Antal sider4
ISSN1545-5009
DOI
StatusUdgivet - okt. 2022

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