Epithelioid Hemangioendothelioma (EHE)

What is epithelioid hemangioendothelioma?

Epithelioid hemangioendothelioma (EHE) is a very rare vascular tumor or anomaly that arises from the cells lining the blood vessels. In some patients with more aggressive disease, EHE behaves like a low-grade (slow-growing) cancer. In patients with stable tumors, or tumors that shrink without treatment, EHE behaves more like a benign (non-cancerous) vascular tumor.

How we care for epithelioid hemangioendothelioma

Because epithelioid hemangioendotheliomas are so rare, very few doctors have experience diagnosing and treating them. The Vascular Anomalies Center at Boston Children's Hospital, in partnership with the Dana-Farber/Boston Children's Solid Tumor Center, has evaluated more children with EHE than any other hospital in the world.

The VAC's physicians — representing 16 medical and surgical specialties, including radiologists and pathologists that specialize in diagnosing vascular anomalies — draw on those experiences to continually refine the therapies and achieve better long-term outcomes for children with EHE. Together, our team has the experience to make or confirm a diagnosis of EHE and recommend the best available treatment options.

The VAC currently cares for many EHE patients from afar, seeing them in person on an infrequent basis but coordinating closely with providers close to their homes.

Our areas of innovation for epithelioid hemangioendothelioma

Sometimes it’s difficult to determine the best treatment for EHE because it has such a wide spectrum of behavior. In some kids, the tumor remains stable or even goes away without treatment. In others, it grows, appears in other parts of the body — and it can even be fatal.

Understanding which types of therapy work for which kids will help us treat more children more effectively. With that in mind, our researchers and physicians have built an extensive and growing database with information on dozens of children and young adults referred to us for EHE, including their symptoms, the treatments recommended and the outcomes of those treatments. By systematically gathering this kind of information, we continually expand our understanding of the best therapies for patients with EHE. Additionally, we are always developing clinical trials for patients with rare vascular anomalies.