Epithelioid hemangioendothelioma in children: The European Pediatric Soft Tissue Sarcoma Study Group experience
Publication date
2022-10
Authors
Orbach, Daniel
Van Noesel, Max M.
Brennan, Bernadette
Corradini, Nadège
Alaggio, Rita
Ben Arush, Myriam
Schoot, Reineke A
Berlanga, Pablo
Zanetti, Ilaria
Hjalgrim, Lisa Lyngsie
Editors
Advisors
Supervisors
Document Type
Article
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License
cc_by_nc_nd
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).
Keywords
children, hemangioendothelioma, soft tissue sarcoma, surgery, Hematology, Oncology, Pediatrics, Perinatology, and Child Health, Journal Article
Citation
Orbach, D, Van Noesel, M M, Brennan, B, Corradini, N, Alaggio, R, Ben Arush, M, Schoot, R A, Berlanga, P, Zanetti, I, Hjalgrim, L L, Di Corti, F, Ramirez, G, Casanova, M & Ferrari, A 2022, 'Epithelioid hemangioendothelioma in children : The European Pediatric Soft Tissue Sarcoma Study Group experience', Pediatric Blood & Cancer, vol. 69, no. 10, e29882. https://doi.org/10.1002/pbc.29882