Epithelioid Hemangioendothelioma Registry

Given its incidence of 0.038/100 000/year, Epithelioid Hemangiohendotelioma (EHE) is an ultra-rare sarcoma, with distinctive, well-defined pathological, molecular and clinical features. It belongs to the group of vascular sarcomas and is characterized by WWTR1::CAMTA1 (90%) or YAP1::TFE3 (10%) gene fusions, which represents today a hallmark for diagnosis. EHE potentially arises everywhere in the body and shows a high tendency toward metastatic spread, especially in the lung, liver and bone. The onset is characterized by different presentations, including a unifocal lesion (usually in the soft tissues), locoregional metastases (multiple lesions in a single organ or in a single anatomic compartment) and systemic metastases (multi-organ involvement).

Also, the biological behavior of the disease is unique in sarcomas and variable, with spontaneous regressions reported, patients with untreated stable disease overtime, slowly progressive variants and highly aggressive and rapidly fatal cases. Today, the relative incidence of the different presentations is undefined, the natural history of the different subtypes is poorly understood, and reliable clinical or biological prognostic factors are lacking.

Given the degree of uncertainty on EHE management, a global consensus meeting was organized in December 2020 under the umbrella of the European Society for Medical Oncology (ESMO) involving > 80 multidisciplinary, worldwide experts together with a patient representative, with the aim of defining, by consensus, evidence-based best practices for the optimal approach to primary and metastatic EHE.

EHE registry objectives:

• To describe EHE population and diseases characteristics at diagnosis  
• To define EHE natural history, as a whole and in its variants
• To describe EHE response to different systemic therapies and radiation therapies
• To assess effectiveness of different treatments (surgery, radiation therapy, systemic therapies, ILP, locoregional techniques and combination)
• Identify prognostic and predictive factors including:
         - Age at diagnosis, gender, hormonal status (for females), performance status
         - Disease extent at presentation, disease size (for primary localized tumours) and disease site
         - Signs and symptoms at presentation and during follow up, including tumour-related pain, systemic symptoms (such as temperature, asthenia, anorexia, weight loss, night sweating) and limb edema
         - Anaemia and fibrinogen increase
         - Radiological features (including serosal involvement and / or effusion)
         - Pathological features (including necrosis, mitoses, nuclear pleomorphism)
         - Molecular features (including molecular translocations, WWTR1:CAMTA1 vs TFE3:WWTR1 vs others)

• To describe treatment pattern and adherence to consensus paper recommendations on EHE clinical management (including diagnostic ascertainment, treatment, follow-up)
• To generate hypotheses on potential risk factors: demographics, sex, medical history (allergy, previous malignancies, immune-mediated diseases and previous immunosuppressive therapies, hormonal influence).

Please refer to the checklists developed by the Promoter to be sure to collect all the main information requested in the Case Report Form on REDCap during the visits. The checklists can be used by the clinicians during the first consultation, follow up, pathological review and progression visit. It is for local use only. No check-list must be shared with the Promoter.

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Checklist 1
Checklist 2
Checklist 3
Checklist 4
EHE registry Codebook

Registry parameters

Registy inclusion criteria
Patient protection
Participating centres
Registry report

Registry inclusion criteria

• 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)

Patient protection

Personal data will be recorded and stored in pseudonymised format. in the federated model PHT solution will enable data sharing among multiple organizations without identifiable data leaving the HCP.  

All parties involved in registry development will maintain strict confidentiality to ensure that neither personal privacy nor the privacy of the families of patients participating in the registries is violated. Data will be processed exclusively by authorized personnel participating in development of the EURACAN registry (a data processor will be identified at each HCP involved). Access to computer systems and the premises where they are kept will be controlled by appropriate security measures which comply with privacy regulation requirements. The processing of the personal data of patients taking part in the EURACAN registry, and specifically in relation to consent-related data, will comply with local privacy legislation and the General Data Protection Regulation 2016/679 (GDPR) of the European Union.

The registry protocol will be submitted to the ethics committees (EC) of the HCPs involved. Furthermore, the ECs of the HCP involved will authorize in advance any research carried out using EURACAN registry data.

Informed consent for use of clinical data
Informed consent for future use of biological

Participating centres

The aim of the registry is to collect data on all patients treated or followed up for the ultra-rare cancer Epitelioid Hemangioendothelioma at the HCPs of the ERN EURACAN, at non-EURACAN expert HCPs, or at national and/or regional clinical networks in European and non-European countries. Since December 1st 2023, there are 21 EURACAN HCPs located in 10 European countries, including Great Britain.

Participating centers PDF

Registry report

To access your latest registry report, please click the link below. The report contains important details and updates relevant to your account.

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