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.

Epithelioid hemangioendothelioma, an ultra-rare cancer : a consensus paper from the community of experts / S. Stacchiotti, A.B. Miah, A.M. Frezza, C. Messiou, C. Morosi, A. Caraceni, C.R. Antonescu, J. Bajpai, E. Baldini, S. Bauer, R. Biagini, S. Bielack, J.Y. Blay, S. Bonvalot, I. Boukovinas, J.V.M.G. Bovee, K. Boye, T. Brodowicz, D. Callegaro, E. De Alava, M. Deoras-Sutliff, A. Dufresne, M. Eriksson, C. Errani, A. Fedenko, V. Ferraresi, A. Ferrari, C.D.M. Fletcher, X. Garcia del Muro, H. Gelderblom, R.A. Gladdy, F. Gouin, G. Grignani, J. Gutkovich, R. Haas, N. Hindi, P. Hohenberger, P. Huang, H. Joensuu, R.L. Jones, C. Jungels, B. Kasper, A. Kawai, A. Le Cesne, F. Le Grange, A. Leithner, H. Leonard, A. Lopez Pousa, J. Martin Broto, O. Merimsky, P. Merriam, R. Miceli, O. Mir, M. Molinari, M. Montemurro, G. Oldani, E. Palmerini, M.A. Pantaleo, S. Patel, S. Piperno-Neumann, C.P. Raut, V. Ravi, A.R.A. Razak, P. Reichardt, B.P. Rubin, P. Rutkowski, A.A. Safwat, C. Sangalli, G. Sapisochin, M. Sbaraglia, S. Scheipl, P. Schoffski, D. Strauss, S.J. Strauss, K. Sundby Hall, W.D. Tap, A. Trama, A. Tweddle, W.T.A. van der Graaf, M.A.J. Van De Sande, W. Van Houdt, G. van Oortmerssen, A.J. Wagner, M. Wartenberg, J. Wood, N. Zaffaroni, C. Zimmermann, P.G. Casali, A.P. Dei Tos, A. Gronchi. - In: ESMO OPEN. - ISSN 2059-7029. - 6:3(2021 Jun), pp. 100170.1-100170.14. [10.1016/j.esmoop.2021.100170]

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

S. Stacchiotti
Primo
;
A. Caraceni;D. Callegaro;M. Molinari;P.G. Casali;
2021

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.
diagnosis; epithelioid hemangioendothelioma; guidelines; management; sarcoma; treatment
Settore MED/06 - Oncologia Medica
Settore MED/09 - Medicina Interna
giu-2021
Article (author)
File in questo prodotto:
File Dimensione Formato  
main2.pdf

accesso aperto

Tipologia: Publisher's version/PDF
Dimensione 2.62 MB
Formato Adobe PDF
2.62 MB Adobe PDF Visualizza/Apri
Pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/909116
Citazioni
  • ???jsp.display-item.citation.pmc??? 43
  • Scopus 64
  • ???jsp.display-item.citation.isi??? 59
social impact