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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.

SUBMITTER: Stacchiotti S

PROVIDER: S-EPMC8182432 | biostudies-literature | 2021 Jun

REPOSITORIES: biostudies-literature

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

Stacchiotti S S Miah A B AB Frezza A M AM Messiou C C Morosi C C Caraceni A A Antonescu C R CR Bajpai J J Baldini E E Bauer S S Biagini R R Bielack S S Blay J Y JY Bonvalot S S Boukovinas I I Bovee J V M G JVMG Boye K K Brodowicz T T Callegaro D D De Alava E E Deoras-Sutliff M M Dufresne A A Eriksson M M Errani C C Fedenko A A Ferraresi V V Ferrari A A Fletcher C D M CDM Garcia Del Muro X X Gelderblom H H Gladdy R A RA Gouin F F Grignani G G Gutkovich J J Haas R R Hindi N N Hohenberger P P Huang P P Joensuu H H Jones R L RL Jungels C C Kasper B B Kawai A A Le Cesne A A Le Grange F F Leithner A A Leonard H H Lopez Pousa A A Martin Broto J J Merimsky O O Merriam P P Miceli R R Mir O O Molinari M M Montemurro M M Oldani G G Palmerini E E Pantaleo M A MA Patel S S Piperno-Neumann S S Raut C P CP Ravi V V Razak A R A ARA Reichardt P P Rubin B P BP Rutkowski P P Safwat A A AA Sangalli C C Sapisochin G G Sbaraglia M M Scheipl S S Schöffski P P Strauss D D Strauss S J SJ Sundby Hall K K Tap W D WD Trama A A Tweddle A A van der Graaf W T A WTA Van De Sande M A J MAJ Van Houdt W W van Oortmerssen G G Wagner A J AJ Wartenberg M M Wood J J Zaffaroni N N Zimmermann C C Casali P G PG Dei Tos A P AP Gronchi A A

ESMO open 20210602 3

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 l ...[more]

PMID: 34090171

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Project description:IntroductionEpithelioid hemangioendothelioma (EHE) is an ultra-rare sarcoma, marked by distinctive molecular and pathological features and with a variable clinical behavior. Its natural history is still partially understood, reliable prognostic and predictive factors are lacking and many questions are still open on the optimal management. In the context of EURACAN, a prospective registry specifically dedicated to EHE was developed and launched with the aim of providing, through high-quality prospective data collection, a better understanding of this disease.Study designRegistry-based cohort study including only new cases of patients with a pathological and molecularly confirmed diagnosis of EHE.ObjectivesTo improve the understanding of EHE natural history, validate and identify new prognostic and predictive factors, clarify the activity and efficacy of currently available treatment options, describe treatment pattern.MethodsSettings and participantsIt is an hospital-based registry established in centers with expertise in EHE including adult patients with a new pathological and molecularly confirmed diagnosis of EHE starting from the 1st December 2023. The characteristics of each patient in the facility who meets the above-mentioned inclusion criteria will be collected prospectively and longitudinally with follow-up at cancer progression and / or cancer relapse or patient death. It is a secondary use of data which will be collected from the clinical records. The data collected for the registry will not entail further examinations or admissions to the facility and/or additional appointments to those normally provided for routine patient follow-up. VariablesFull details on patients and disease features, treatment and outcome will be collected, according to common clinical practice guidelines developed and shared with all the contributing centers. In addition, data on potential confounders (e.g. comorbidity; functional status etc.) will also be collected. Statistical methodsThe data analyses will include descriptive statistics and analytical analyses. Multivariable Cox's proportional hazards model and Hazard ratios (HR) for all-cause or cause-specific mortality will be used to determine independent predictors of overall survival, recurrence and progression.ResultsThe registry has been joined by 21 sarcoma reference centers across EU and UK, covering 10 countries. Patients' recruitment started in December 2023. The estimated completion date is December 2033 upon agreement on the achievement of all the registry objectives. The already established collaboration and participation of EHE patient's associations involved in the project will help in promoting the registry and fostering accrual.

Dataset Information

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

Publications

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

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