Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts.

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State: Public
Version: Final published version
License: CC BY-NC-ND 4.0
Serval ID
serval:BIB_9176DFAEB6D9
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts.
Journal
ESMO open
Author(s)
Stacchiotti S., Miah A.B., Frezza A.M., Messiou C., Morosi C., Caraceni A., Antonescu C.R., Bajpai J., Baldini E., Bauer S., Biagini R., Bielack S., Blay J.Y., Bonvalot S., Boukovinas I., Bovee JVMG, Boye K., Brodowicz T., Callegaro D., De Alava E., Deoras-Sutliff M., Dufresne A., Eriksson M., Errani C., Fedenko A., Ferraresi V., Ferrari A., Fletcher CDM, Garcia Del Muro X., Gelderblom H., Gladdy R.A., Gouin F., Grignani G., Gutkovich J., Haas R., Hindi N., Hohenberger P., Huang P., Joensuu H., Jones R.L., Jungels C., Kasper B., Kawai A., Le Cesne A., Le Grange F., Leithner A., Leonard H., Lopez Pousa A., Martin Broto J., Merimsky O., Merriam P., Miceli R., Mir O., Molinari M., Montemurro M., Oldani G., Palmerini E., Pantaleo M.A., Patel S., Piperno-Neumann S., Raut C.P., Ravi V., Razak ARA, Reichardt P., Rubin B.P., Rutkowski P., Safwat A.A., Sangalli C., Sapisochin G., Sbaraglia M., Scheipl S., Schöffski P., Strauss D., Strauss S.J., Sundby Hall K., Tap W.D., Trama A., Tweddle A., van der Graaf WTA, Van De Sande MAJ, Van Houdt W., van Oortmerssen G., Wagner A.J., Wartenberg M., Wood J., Zaffaroni N., Zimmermann C., Casali P.G., Dei Tos A.P., Gronchi A.
ISSN
2059-7029 (Electronic)
ISSN-L
2059-7029
Publication state
Published
Issued date
06/2021
Peer-reviewed
Oui
Volume
6
Number
3
Pages
100170
Language
english
Notes
Publication types: Journal Article ; Review
Publication Status: ppublish
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.
Keywords
diagnosis, epithelioid hemangioendothelioma, guidelines, management, sarcoma, treatment
Pubmed
Web of science
Open Access
Yes
Create date
15/06/2021 15:05
Last modification date
12/01/2022 7:12
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