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

• Published in: ESMO open Vol. 6; no. 3; p. 100170
• Main Authors: 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., Garcia del Muro, X., 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., Lopez Pousa, A., Martin Broto, J., 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, V., Razak, A.R.A., Reichardt, P., Rubin, B.P., Rutkowski, P., Safwat, A.A., Sangalli, C., Sapisochin, G., Sbaraglia, M., Scheipl, S., Schöffski, P., Strauss, D., Strauss, S.J., Sundby Hall, K., 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.
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01.06.2021; Elsevier
• Subjects: Adult; Cancer and Oncology; Cancer och onkologi; Child; Clinical Medicine; Consensus; diagnosis; epithelioid hemangioendothelioma; guidelines; Hemangioendothelioma, Epithelioid - diagnosis; Hemangioendothelioma, Epithelioid - drug therapy; Humans; Klinisk medicin; management; Medical and Health Sciences; Medical Oncology; Medicin och hälsovetenskap; Patient Advocacy; Review; sarcoma; Sarcoma - diagnosis; Sarcoma - drug therapy; treatment; epithelioid hemangioendothelioma; treatment; diagnosis; guidelines; sarcoma; management
• ISSN: 2059-7029; 2059-7029
• DOI: 10.1016/j.esmoop.2021.100170

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. •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.