Registry following adults newly diagnosed with epithelioid hemangioendothelioma across Europe
Records adults with a rare soft tissue sarcoma to learn how the disease progresses.
Plain-language title generated from the registry record
Registry title: The Epithelioid Hemangioendothelioma Registry of the European Reference Network on Rare Adult Solid Cancers (EURACAN)
Key facts
- Where
- Porto, PortugalSee the site
Where in Portugal
- Instituto Português Oncologia do Porto Francisco GentilPorto, PortugalNot yet recruiting
Questions to ask the study team
- How often do I need to come in, and for how long each time?
- Are travel costs paid?
- Can I keep my own doctor?
- What happens if I want to stop?
Trial details
- Who runs it
- Fondazione IRCCS Istituto Nazionale dei Tumori, Milano
- Trial code
- NCT06408441
- Record updated
- 3 April 2026
- Original description
Epithelioid 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.Read full original descriptionShow less
The Observational EURACAN Prospective Clinical Registry Dedicated to Epithelioid Hemangioendothelioma: the Protocol of an International and Collaborative Effort on an Ultra-rare Entity
Epithelioid 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.
The study design is a registry-based cohort study including only new cases of patients with a pathological and molecularly confirmed diagnosis of EHE.
The objectives are to 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.
It is an hospital-based registry established in centres 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.
The 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.
The 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.
This registry has been developed with the support of EHE Rare Cancer Charity UK, STATER (Grant Agreement number: 947604, HP-PJ-2019) and EURACAN 2022 (Grant Agreement number: 101085486, EU4H-2022-ERN-IBA) European Health and Digital Executive Agency (HaDEA)
Inclusion: New patients managed by the contributing centers with a pathological EHE diagnosis performed or verified by an expert sarcoma pathologist starting from 1 December 2023 onwards and to be performed within 6 months from the registration; Molecular confirmation of the diagnosis (WWTR1-CAMTA1 or YAP1-TFE3); Adult patients (aged ≥ 18 years).