Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts.
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ICR Authors
Authors
Stacchiotti, S
Miah, AB
Frezza, AM
Messiou, C
Morosi, C
Caraceni, A
Antonescu, CR
Bajpai, J
Baldini, E
Bauer, S
Biagini, R
Bielack, S
Blay, JY
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, RA
Gouin, F
Grignani, G
Gutkovich, J
Haas, R
Hindi, N
Hohenberger, P
Huang, P
Joensuu, H
Jones, RL
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, MA
Patel, S
Piperno-Neumann, S
Raut, CP
Ravi, V
Razak, ARA
Reichardt, P
Rubin, BP
Rutkowski, P
Safwat, AA
Sangalli, C
Sapisochin, G
Sbaraglia, M
Scheipl, S
Schöffski, P
Strauss, D
Strauss, SJ
Sundby Hall, K
Tap, WD
Trama, A
Tweddle, A
van der Graaf, WTA
Van De Sande, MAJ
Van Houdt, W
van Oortmerssen, G
Wagner, AJ
Wartenberg, M
Wood, J
Zaffaroni, N
Zimmermann, C
Casali, PG
Dei Tos, AP
Gronchi, A
Miah, AB
Frezza, AM
Messiou, C
Morosi, C
Caraceni, A
Antonescu, CR
Bajpai, J
Baldini, E
Bauer, S
Biagini, R
Bielack, S
Blay, JY
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, RA
Gouin, F
Grignani, G
Gutkovich, J
Haas, R
Hindi, N
Hohenberger, P
Huang, P
Joensuu, H
Jones, RL
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, MA
Patel, S
Piperno-Neumann, S
Raut, CP
Ravi, V
Razak, ARA
Reichardt, P
Rubin, BP
Rutkowski, P
Safwat, AA
Sangalli, C
Sapisochin, G
Sbaraglia, M
Scheipl, S
Schöffski, P
Strauss, D
Strauss, SJ
Sundby Hall, K
Tap, WD
Trama, A
Tweddle, A
van der Graaf, WTA
Van De Sande, MAJ
Van Houdt, W
van Oortmerssen, G
Wagner, AJ
Wartenberg, M
Wood, J
Zaffaroni, N
Zimmermann, C
Casali, PG
Dei Tos, AP
Gronchi, A
Document Type
Journal Article
Date
2021-06-02
Date Accepted
2021-05-11
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.
Citation
ESMO open, 2021, 6 (3), pp. 100170 - ?
Source Title
Publisher
ELSEVIER
ISSN
2059-7029
eISSN
2059-7029
Collections
Research Team
Molecular and Systems Oncology
Molecular and Systems Oncology
Molecular and Systems Oncology