Portal de Periódicos da CAPES

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

2021; Elsevier BV; Volume: 6; Issue: 3 Linguagem: Inglês

10.1016/j.esmoop.2021.100170

2059-7029

Silvia Stacchiotti, Aisha Miah, Anna Maria Frezza, Christina Messiou, Carlo Morosi, Augusto Caraceni, Cristina R. Antonescu, Jyoti Bajpai, Elizabeth H. Baldini, Sebastian Bauer, R. Biagini, Stefan Bielack, Jean‐Yves Blay, Sylvie Bonvalot, I. Boukovinas, Judith V.M.G. Bovée, Kjetil Boye, Thomas Brodowicz, Dario Callegaro, Enrique de Álava, M. Deoras-Sutliff, Armelle Dufresne, Mikael Eriksson, Costantino Errani, А. А. Феденко, Virginia Ferraresi, Andrea Ferrari, Christopher D.�M. Fletcher, Xavier García del Muro, Hans Gelderblom, Rebecca Gladdy, F. Gouin, Giovanni Grignani, J. Gutkovich, Rick L. Haas, Nadia Hindi, Peter Hohenberger, Paul H. Huang, Heikki Joensuu, Robin L. Jones, Christiane Jungels, Bernd Kasper, Akira Kawai, Axel Le Cesne, Franel le Grange, Andreas Leithner, Hugh Leonard, Antonio López‐Pousa, Javier Martín‐Broto, Ofer Merimsky, Priscilla Merriam, Rosalba Miceli, Olivier Mir, Michele Molinari, Michael Montemurro, Graziano Oldani, Emanuela Palmerini, Maria A. Pantaleo, S. Patel, Sophie Piperno‐Neumann, Chandrajit P. Raut, Vinod Ravi, Albiruni R. Abdul Razak, Peter Reichardt, Brian P. Rubin, Piotr Rutkowski, Akmal Safwat, Claudia Sangalli, Gonzalo Sapisochín, Marta Sbaraglia, S. Scheipl, Patrick Schöffski, D. Strauß, Sandra J. Strauss, Kirsten Sundby Hall, William D. Tap, Annalisa Trama, Andrew Tweddle, Winette T.A. van der Graaf, Michiel A. J. van de Sande, Winan J. van Houdt, G. van Oortmerssen, Andrew J. Wagner, Markus Wartenberg, Jayne Wood, Nadia Zaffaroni, Camilla Zimmermann, Paolo G. Casali, Angelo Paolo Dei Tos, Alessandro Gronchi,

Cardiac tumors and thrombi

•This consensus paper provides key recommendations on the management of epithelioid hemangioendothelioma (EHE).•Recommendations followed a consensus meeting between experts and a representative of the EHE advocacy group and SPAEN.•Authorship includes a multidisciplinary group of experts from different institutions from Europe, North America and Asia. 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 (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.