Stacchiotti, S;
Miah, AB;
Frezza, AM;
Messiou, C;
Morosi, C;
Caraceni, A;
Antonescu, CR;
... Gronchi, A; + view all
(2021)
Enithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts.
ESMO OPEN
, 6
(3)
, Article 100170. 10.1016/j.esmoop.2021.100170.
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Abstract
Epithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade sarcoma and it is marked by a high propensity for systemic involvement. No active systemic agents are currently approved specifically for EHE, which is typically refractory to the antitumor drugs used in sarcomas. The degree of uncertainty in selecting the most appropriate therapy for EHE patients and the lack of guidelines on the clinical management of the disease make the adoption of new treatments inconsistent across the world, resulting in suboptimal outcomes for many EHE patients. To address the shortcoming, a global consensus meeting was organized in December 2020 under the umbrella of the European Society for Medical Oncology (ESMO) involving >80 experts from several disciplines from Europe, North America and Asia, together with a patient representative from the EHE Group, a global, disease-specific patient advocacy group, and Sarcoma Patient EuroNet (SPAEN). The meeting was aimed at defining, by consensus, evidence-based best practices for the optimal approach to primary and metastatic EHE. The consensus achieved during that meeting is the subject of the present publication.
| Type: | Article |
|---|---|
| Title: | Enithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1016/j.esmoop.2021.100170 |
| Publisher version: | https://doi.org/10.1016/j.esmoop.2021.100170 |
| Language: | English |
| Additional information: | © 2021 The Author(s). Published by Elsevier Ltd on behalf of European Society for Medical Oncology. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
| Keywords: | sarcoma, epithelioid hemangioendothelioma, diagnosis, treatment, management, guidelines |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute > Research Department of Oncology |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10131128 |
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