Epithelioid hemangioendothelioma, an ultra-rare cancer

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 curren...

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Autores: Stacchiotti, Silvia|||0000-0002-1742-8666, Miah, Aisha B., Frezza, Anna Maria|||0000-0003-2335-7224, Messiou, Christina, Morosi, Carlo|||0000-0003-4940-2185, Caraceni, Augusto|||0000-0002-0375-6204, Antonescu, Cristina|||0000-0002-9717-8205, Bajpai, Jyoti, Baldini, Elizabeth Healey, Bauer, Sebastian|||0000-0001-5949-8120, Biagini, Roberto|||0000-0002-6819-2841, Bielack, Stefan S., Blay, Jean-Yves|||0000-0001-7190-120X, Bonvalot, Sylvie, Boukovinas, Ioannis P., Bovee, Judith V.M.G., Boye, Kjetil, Brodowicz, Thomas, Callegaro, Dario|||0000-0002-3392-4002, De Álava, Enrique|||0000-0001-8400-046X, Deoras-Sutliff, M., Dufresne, Armelle, Eriksson, Mikael, Errani, Costantino|||0000-0002-4504-2867, Fedenko, Alexander|||0000-0003-4927-5585, Ferraresi, Virginia|||0000-0001-6419-1668, Ferrari, Andrea|||0000-0002-4724-0517, Fletcher, Christopher D.M., Garcia del Muro, Xavier|||0000-0001-5571-2847, Gelderblom, Hans J.|||0000-0001-9270-8636, Gladdy, Rebecca A., Gouin, François, Grignani, Giovanni|||0000-0001-5515-569X, Gutkovich, J., Haas, Rick LM.|||0000-0003-4834-4331, Hindi, Nadia|||0000-0002-5864-762X, Hohenberger, Peter|||0000-0001-5359-5923, Huang, Paul|||0000-0003-3972-5087, Joensuu, Heikki T., Jones, Robin Lewis, Jungels, Christiane, Kasper, Bernd|||0000-0001-8425-8356, Kawai, Akira, Le Cesne, Axel, Le Grange, Franél, Leithner, Andeas, Leonard, H., López Pousa, Antonio, Martin Broto, Javier, Merimsky, Ofer, Merriam, Priscilla, Miceli, Rosalba|||0000-0003-0954-375X, Mir, Olivier, Molinari, Massimo, Montemurro, Michael, Oldani, Graziano, Palmerini, Emanuela|||0000-0003-3406-6705, Pantaleo, Maria Abbondanza|||0000-0002-0177-6957, Patel, Sapna Pradyuman, Piperno-Neumann, Sophie, Raut, Chandrajit Premanand, Ravi, Vinod, Razak, Albiruni Ryan Abdul, Reichardt, Peter, Rubin, Brian P., Rutkowski, Piotr|||0000-0002-8920-5429, Safwat, Akmal Ahmed, Sangalli, Claudia|||0000-0002-6465-0630, Sapisochin, Gonzalo, Sbaraglia, Marta, Scheipl, Susanne, Schöffski, Patrick, Strauss, Dirk Cornelius, Strauss, Sandra J., Sundby Hall, Kirsten, Tap, William D., Trama, Annalisa, Tweddle, A., van der Graaf, Winette T.A., van de Sande, Michiel|||0000-0002-9156-7656, Van Houdt, Winan|||0000-0002-2303-3468, van Oortmerssen, Gerard, Wagner, Andrew J., Wartenberg, Markus, Wood, J., Zaffaroni, Nadia|||0000-0002-4669-0890, Zimmermann, Camilla|||0000-0003-4889-0244, Casali, Paolo Giovanni|||0000-0003-4056-8023, Dei Tos, Angelo|||0000-0002-1228-8940, Gronchi, Alessandro|||0000-0002-4703-3534
Tipo de recurso: artículo
Fecha de publicación:2021
País:España
Institución:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:271964
Acceso en línea:https://ddd.uab.cat/record/271964
https://dx.doi.org/urn:doi:10.1016/j.esmoop.2021.100170
Access Level:acceso abierto
Palabra clave:Sarcoma
Epithelioid hemangioendothelioma
Diagnosis
Treatment
Management
Guidelines
Descripción
Sumario: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.

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