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

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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 , J V M G , 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 , C D M , Del Muro , X G , 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 , Pousa , A L , Broto , J M , 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 , A V , Razak , A R A , Reichardt , P , Rubin , B P , Rutkowski , P , Safwat , A A , Sangalli , C , Sapisochin , G , Sbaraglia , M , Scheipl , S , Schoffski , P , Strauss , D , Strauss , S J , Hall , K S , Tap , W D , Trama , A , Tweddle , A , van der Graaf , W T A , Van de Sande , M A J , 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 2021 , ' Enithelioid hemangioendothelioma, an ultra-rare cancer : a consensus paper from the community of experts ' , ESMO open , vol. 6 , no. 3 , 100170 . https://doi.org/10.1016/j.esmoop.2021.100170

Title: Enithelioid hemangioendothelioma, an ultra-rare cancer : a consensus paper from the community of experts
Author: 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, J. V. M. G.; 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, C. D. M.; Del Muro, X. Garcia; 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.; Pousa, A. Lopez; Broto, J. Martin; 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, Arumugam Veera; Razak, A. R. A.; Reichardt, P.; Rubin, B. P.; Rutkowski, P.; Safwat, A. A.; Sangalli, C.; Sapisochin, G.; Sbaraglia, M.; Scheipl, S.; Schoffski, P.; Strauss, D.; Strauss, S. J.; Hall, K. Sundby; Tap, W. D.; Trama, A.; Tweddle, A.; van der Graaf, W. T. A.; Van de Sande, M. A. J.; 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.
Other contributor: University of Helsinki, University of Helsinki



Date: 2021-06
Language: eng
Number of pages: 14
Belongs to series: ESMO open
ISSN: 2059-7029
DOI: https://doi.org/10.1016/j.esmoop.2021.100170
URI: http://hdl.handle.net/10138/333872
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.
Subject: sarcoma
epithelioid hemangioendothelioma
diagnosis
treatment
management
guidelines
HEPATIC EPITHELIOID HEMANGIOENDOTHELIOMA
OF-THE-LITERATURE
SOFT-TISSUE
LIVER-TRANSPLANTATION
CLINICAL CHARACTERISTICS
RISK STRATIFICATION
IMAGING FINDINGS
VASCULAR TUMORS
PROPOSAL
SARCOMAS
3122 Cancers
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