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

Stacchiotti, S. ; Miah, A. B. ; Frezza, A. M. ; Messiou, C. ; Morosi, C. ; Caraceni, A. ; Antonescu, C. R. ; Bajpai, J. ; Baldini, E. and Bauer, S. , et al. (2021) In ESMO Open 6(3).
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... (More)

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.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
diagnosis, epithelioid hemangioendothelioma, guidelines, management, sarcoma, treatment
in
ESMO Open
volume
6
issue
3
article number
100170
publisher
BMJ Publishing Group
external identifiers
  • scopus:85108742417
  • pmid:34090171
ISSN
2059-7029
DOI
10.1016/j.esmoop.2021.100170
language
English
LU publication?
yes
additional info
Publisher Copyright: © 2021 The Author(s)
id
14ce555d-70c8-4c79-a869-0b207a20c1c2
date added to LUP
2021-12-20 12:08:38
date last changed
2024-04-20 19:01:48
@article{14ce555d-70c8-4c79-a869-0b207a20c1c2,
  abstract     = {{<p>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 &gt;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.</p>}},
  author       = {{Stacchiotti, S. and Miah, A. B. and Frezza, A. M. and Messiou, C. and Morosi, C. and Caraceni, A. and Antonescu, C. R. and Bajpai, J. and Baldini, E. and Bauer, S. and Biagini, R. and Bielack, S. and Blay, J. Y. and Bonvalot, S. and Boukovinas, I. and Bovee, J. V.M.G. and Boye, K. and Brodowicz, T. and Callegaro, D. and De Alava, E. and Deoras-Sutliff, M. and Dufresne, A. and Eriksson, M. and Errani, C. and Fedenko, A. and Ferraresi, V. and Ferrari, A. and Fletcher, C. D.M. and Garcia del Muro, X. and Gelderblom, H. and Gladdy, R. A. and Gouin, F. and Grignani, G. and Gutkovich, J. and Haas, R. and Hindi, N. and Hohenberger, P. and Huang, P. and Joensuu, H. and Jones, R. L. and Jungels, C. and Kasper, B. and Kawai, A. and Le Cesne, A. and Le Grange, F. and Leithner, A. and Leonard, H. and Lopez Pousa, A. and Martin Broto, J. and Merimsky, O. and Merriam, P. and Miceli, R. and Mir, O. and Molinari, M. and Montemurro, M. and Oldani, G. and Palmerini, E. and Pantaleo, M. A. and Patel, S. and Piperno-Neumann, S. and Raut, C. P. and Ravi, V. and Razak, A. R.A. and Reichardt, P. and Rubin, B. P. and Rutkowski, P. and Safwat, A. A. and Sangalli, C. and Sapisochin, G. and Sbaraglia, M. and Scheipl, S. and Schöffski, P. and Strauss, D. and Strauss, S. J. and Sundby Hall, K. and Tap, W. D. and Trama, A. and Tweddle, A. and van der Graaf, W. T.A. and Van De Sande, M. A.J. and Van Houdt, W. and van Oortmerssen, G. and Wagner, A. J. and Wartenberg, M. and Wood, J. and Zaffaroni, N. and Zimmermann, C. and Casali, P. G. and Dei Tos, A. P. and Gronchi, A.}},
  issn         = {{2059-7029}},
  keywords     = {{diagnosis; epithelioid hemangioendothelioma; guidelines; management; sarcoma; treatment}},
  language     = {{eng}},
  number       = {{3}},
  publisher    = {{BMJ Publishing Group}},
  series       = {{ESMO Open}},
  title        = {{Epithelioid hemangioendothelioma, an ultra-rare cancer : a consensus paper from the community of experts}},
  url          = {{http://dx.doi.org/10.1016/j.esmoop.2021.100170}},
  doi          = {{10.1016/j.esmoop.2021.100170}},
  volume       = {{6}},
  year         = {{2021}},
}