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. Schöffski; 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

doi:10.1016/j.esmoop.2021.100170

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 AlavaM. 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. Schöffski, 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

Research output: Contribution to journal › Review article › peer-review

58 Scopus citations

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.

Original language	English (US)
Article number	100170
Journal	ESMO Open
Volume	6
Issue number	3
DOIs	https://doi.org/10.1016/j.esmoop.2021.100170
State	Published - Jun 2021

Keywords

diagnosis
epithelioid hemangioendothelioma
guidelines
management
sarcoma
treatment

ASJC Scopus subject areas

Oncology
Cancer Research

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10.1016/j.esmoop.2021.100170

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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., ... Gronchi, A. (2021). Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts. ESMO Open, 6(3), Article 100170. https://doi.org/10.1016/j.esmoop.2021.100170

Stacchiotti, S, Miah, AB, Frezza, AM, Messiou, C, Morosi, C, Caraceni, A, Antonescu, CR, Bajpai, J, Baldini, E, Bauer, S, Biagini, R, Bielack, S, Blay, JY, Bonvalot, S, Boukovinas, I, Bovee, JVMG, 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, CDM, Garcia del Muro, X, Gelderblom, H, Gladdy, RA, Gouin, F, Grignani, G, Gutkovich, J, Haas, R, Hindi, N, Hohenberger, P, Huang, P, Joensuu, H, Jones, RL, 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, MA, Patel, S, Piperno-Neumann, S, Raut, CP, Ravi, V, Razak, ARA, Reichardt, P, Rubin, BP, Rutkowski, P, Safwat, AA, Sangalli, C, Sapisochin, G, Sbaraglia, M, Scheipl, S, Schöffski, P, Strauss, D, Strauss, SJ, Sundby Hall, K, Tap, WD, Trama, A, Tweddle, A, van der Graaf, WTA, Van De Sande, MAJ, Van Houdt, W, van Oortmerssen, G, Wagner, AJ, Wartenberg, M, Wood, J, Zaffaroni, N, Zimmermann, C, Casali, PG, Dei Tos, AP & Gronchi, A 2021, 'Epithelioid 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

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

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.",

keywords = "diagnosis, epithelioid hemangioendothelioma, guidelines, management, sarcoma, treatment",

author = "S. Stacchiotti and Miah, {A. B.} and Frezza, {A. M.} and C. Messiou and C. Morosi and A. Caraceni and Antonescu, {C. R.} and J. Bajpai and E. Baldini and S. Bauer and R. Biagini and S. Bielack and Blay, {J. Y.} and S. Bonvalot and I. Boukovinas and Bovee, {J. V.M.G.} and K. Boye and T. Brodowicz and D. Callegaro and {De Alava}, E. and M. Deoras-Sutliff and A. Dufresne and M. Eriksson and C. Errani and A. Fedenko and V. Ferraresi and A. Ferrari and Fletcher, {C. D.M.} and {Garcia del Muro}, X. and H. Gelderblom and Gladdy, {R. A.} and F. Gouin and G. Grignani and J. Gutkovich and R. Haas and N. Hindi and P. Hohenberger and P. Huang and H. Joensuu and Jones, {R. L.} and C. Jungels and B. Kasper and A. Kawai and {Le Cesne}, A. and {Le Grange}, F. and A. Leithner and H. Leonard and {Lopez Pousa}, A. and {Martin Broto}, J. and O. Merimsky and P. Merriam and R. Miceli and O. Mir and M. Molinari and M. Montemurro and G. Oldani and E. Palmerini and Pantaleo, {M. A.} and S. Patel and S. Piperno-Neumann and Raut, {C. P.} and V. Ravi and Razak, {A. R.A.} and P. Reichardt and Rubin, {B. P.} and P. Rutkowski and Safwat, {A. A.} and C. Sangalli and G. Sapisochin and M. Sbaraglia and S. Scheipl and P. Sch{\"o}ffski and D. Strauss and Strauss, {S. J.} and {Sundby Hall}, K. and Tap, {W. D.} and A. Trama and A. Tweddle and {van der Graaf}, {W. T.A.} and {Van De Sande}, {M. A.J.} and {Van Houdt}, W. and {van Oortmerssen}, G. and Wagner, {A. J.} and M. Wartenberg and J. Wood and N. Zaffaroni and C. Zimmermann and Casali, {P. G.} and {Dei Tos}, {A. P.} and A. Gronchi",

note = "Publisher Copyright: {\textcopyright} 2021 The Author(s)",

year = "2021",

month = jun,

doi = "10.1016/j.esmoop.2021.100170",

language = "English (US)",

volume = "6",

journal = "ESMO Open",

issn = "2059-7029",

publisher = "BMJ Publishing Group",

number = "3",

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TY - JOUR

T1 - Epithelioid hemangioendothelioma, an ultra-rare cancer

T2 - a consensus paper from the community of experts

AU - Stacchiotti, S.

AU - Miah, A. B.

AU - Frezza, A. M.

AU - Messiou, C.

AU - Morosi, C.

AU - Caraceni, A.

AU - Antonescu, C. R.

AU - Bajpai, J.

AU - Baldini, E.

AU - Bauer, S.

AU - Biagini, R.

AU - Bielack, S.

AU - Blay, J. Y.

AU - Bonvalot, S.

AU - Boukovinas, I.

AU - Bovee, J. V.M.G.

AU - Boye, K.

AU - Brodowicz, T.

AU - Callegaro, D.

AU - De Alava, E.

AU - Deoras-Sutliff, M.

AU - Dufresne, A.

AU - Eriksson, M.

AU - Errani, C.

AU - Fedenko, A.

AU - Ferraresi, V.

AU - Ferrari, A.

AU - Fletcher, C. D.M.

AU - Garcia del Muro, X.

AU - Gelderblom, H.

AU - Gladdy, R. A.

AU - Gouin, F.

AU - Grignani, G.

AU - Gutkovich, J.

AU - Haas, R.

AU - Hindi, N.

AU - Hohenberger, P.

AU - Huang, P.

AU - Joensuu, H.

AU - Jones, R. L.

AU - Jungels, C.

AU - Kasper, B.

AU - Kawai, A.

AU - Le Cesne, A.

AU - Le Grange, F.

AU - Leithner, A.

AU - Leonard, H.

AU - Lopez Pousa, A.

AU - Martin Broto, J.

AU - Merimsky, O.

AU - Merriam, P.

AU - Miceli, R.

AU - Mir, O.

AU - Molinari, M.

AU - Montemurro, M.

AU - Oldani, G.

AU - Palmerini, E.

AU - Pantaleo, M. A.

AU - Patel, S.

AU - Piperno-Neumann, S.

AU - Raut, C. P.

AU - Ravi, V.

AU - Razak, A. R.A.

AU - Reichardt, P.

AU - Rubin, B. P.

AU - Rutkowski, P.

AU - Safwat, A. A.

AU - Sangalli, C.

AU - Sapisochin, G.

AU - Sbaraglia, M.

AU - Scheipl, S.

AU - Schöffski, P.

AU - Strauss, D.

AU - Strauss, S. J.

AU - Sundby Hall, K.

AU - Tap, W. D.

AU - Trama, A.

AU - Tweddle, A.

AU - van der Graaf, W. T.A.

AU - Van De Sande, M. A.J.

AU - Van Houdt, W.

AU - van Oortmerssen, G.

AU - Wagner, A. J.

AU - Wartenberg, M.

AU - Wood, J.

AU - Zaffaroni, N.

AU - Zimmermann, C.

AU - Casali, P. G.

AU - Dei Tos, A. P.

AU - Gronchi, A.

PY - 2021/6

Y1 - 2021/6

N2 - 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.

AB - 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.

KW - diagnosis

KW - epithelioid hemangioendothelioma

KW - guidelines

KW - management

KW - sarcoma

KW - treatment

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UR - http://www.scopus.com/inward/citedby.url?scp=85108742417&partnerID=8YFLogxK

U2 - 10.1016/j.esmoop.2021.100170

DO - 10.1016/j.esmoop.2021.100170

M3 - Review article

C2 - 34090171

AN - SCOPUS:85108742417

SN - 2059-7029

VL - 6

JO - ESMO Open

JF - ESMO Open

IS - 3

M1 - 100170

ER -

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

Abstract

Keywords

ASJC Scopus subject areas

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