The Epithelioid Hemangioendothelioma Registry of the European Reference Network on Rare Adult Solid Cancers (EURACAN)

Condition(s) studied

Study summary

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)

Eligibility

Primary outcome measure(s)

• Male to Female (M/F) ratio — 2 years
  Difference between the number of males and females
• Age average — 2 years
  The mathematical average age of all the members of a population (i.e. EHE patients)
• Comorbidity — 2 years
  Proportion of patients with Charlson Comorbidity Index (CCI) score mild (CCI scores of 1-2); moderate (CCI scores of 3-4); and severe (CCI scores ≥5)
• Performance status — 2 years
  Proportion of patients by ECOG performance status grade
• Cumulative incidence of local recurrence — 5 years
  proportion of cancer patients who develop a local recurrence over time
• Cumulative incidence of distant metastasis — 5 years
  proportion of cancer patients who develop distant metastases over time
• Time to local progression — 5 years
  Time elapsed from diagnosis to local progression
• Time to distant metastases — 5 years
  Time elapsed from diagnosis to distant metastasis
• Overall survival — 5 years
  The length of time from either the date of diagnosis or the start of treatment for a disease, such as cancer, that patients diagnosed with the disease are still alive
• Progression free survival — 5 years
  The length of time during and after the treatment of a disease, such as cancer, that a patient lives with the disease but it does not get worse
• Treatment effectiveness — 2 years
  Competitive cumulative incidence of local recurrence and distant metastases
• Disease free survival — 5 years
  The length of time after primary treatment for a cancer ends that the patient survives without any signs or symptoms of that cancer.
• Description of treatment pattern and quality of care — 2 years
  Percentage of patients treated according to Consensus paper recommendations

Trial sites (22)

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