A recent study published in the European Journal of Cancer represents the largest collection of data from people with EHE in Canada. Researchers found that EHE can behave very differently from person to person—about one-third of patients were diagnosed by chance and did not need immediate treatment, while others had more aggressive disease. About half of patients had disease that had already spread at diagnosis, most commonly to the liver or lungs. The study also showed that many patients did not receive molecular testing to identify their fusion gene, and only about one-third were treated with systemic therapies such as sirolimus or other targeted drugs.

Overall survival was just over eight years, reinforcing that EHE is often a long-term, chronic condition. Outcomes were generally worse for patients with lung involvement, fluid buildup around the lungs or abdomen, older age, or metastatic disease. The authors emphasize that treatment decisions must be highly individualized—some patients can be safely monitored, while others benefit from therapy based on symptoms and disease behavior.

Why this matters:

This study highlights the importance of national and international collaboration, routine molecular testing, shared patient registries, and prospective studies to improve diagnosis, guide treatment decisions, and accelerate the development of more effective therapies for people living with EHE.