A new retrospective study from Charité–Universitätsmedizin Berlin describes 41 people with EHE treated at the center between 1997 and 2022, offering a single-center picture of EHE presentation and outcomes. It was published in the Journal of Cancer Research and Clinical Oncology in July 2026.

In this report, women made up the majority of cases (61%), the most common age at diagnosis was 53, and 63% of people had localized hepatic (liver) EHE. This series captured EHE’s wide range of behavior: while median progression-free survival was 157 months (13 years), a small subset of individuals (three) had rapid, aggressive progression. Ten people (24%) developed pleural and/or peritoneal effusions during the course of their disease. 

Similar to other retrospective reports, molecular testing was inconsistent: about half of people were tested for the CAMTA1 fusion, and of those tested, 67% were positive. Fewer were tested for TFE3 fusion (44%), and of those tested, four were positive (22%). This report highlights a gap in, and the need for, molecular confirmation of  EHE to inform care.

Of the patients included, 49% of individuals had surgery for treatment or diagnostic purposes, 15% of patients were treated with systemic therapy (like chemotherapy, targeted therapy, or antiangiogenic agents), and two (5%) had radiation therapy. Five people underwent liver transplantation, and none reported disease recurrence after a median follow-up of 19 years. The authors reported overall survival in this study was negatively associated with factors such as increased age at diagnosis, metastatic disease, pulmonary localization, pleural involvement, and occurrence of effusion, among others. 

Why this data matters: Because EHE is an ultra-rare sarcoma, it is important to learn from every person diagnosed. Collectively, scientists want to learn more about who is affected by EHE and how the disease changes over time. Because practice patterns can vary across countries and regions, these data help build our growing knowledge about EHE. We are encouraged by the progress and efforts made by these authors to highlight the importance of molecular disease confirmation to identify markers of aggressive disease, to inform personalized treatment plans, and to identify the right population for clinical trials.