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. 2022 Oct;69(10):e29882.
doi: 10.1002/pbc.29882. Epub 2022 Jul 16.

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

Daniel Orbach  1 , Max M Van Noesel  2   3 , Bernadette Brennan  4 , Nadège Corradini  5 , Rita Alaggio  6 , Myriam Ben Arush  7 , Reineke A Schoot  2   3 , Pablo Berlanga  8 , Ilaria Zanetti  9 , Lisa Lyngsie Hjalgrim  10 , Federica Di Corti  11 , Gema Ramirez  12 , Michela Casanova  13 , Andrea Ferrari  13
Affiliations

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

Daniel Orbach et al. Pediatr Blood Cancer. 2022 Oct.

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.

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References

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