Epithelioid Hemangioendothelioma in Children

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What is epithelioid hemangioendothelioma?

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor or anomaly that’s not widely understood. Some describe it as a type of cancer, and in rare cases it can grow rapidly or spread to other parts of the body. But in most patients, EHE behaves as a benign vascular tumor, with periods of no growth that may last for years.

  • EHE is very rare. Just a few hundred people in the United States are diagnosed with EHE every year, and it is slightly more common in women than men.
  • The tumor arises from the cells lining the blood vessels.
  • It can appear anywhere in the body, but common sites are the liver, lungs and bones.
  • While it most often appears in young adults, it has been seen in children as young as age 8 or 9.
  • The cause is currently unknown, although genetic changes have recently been identified that may lead to insight in how to target therapy against EHE.
  • The tumor behaves differently in different people. In some it can be stable for years, and can even go away without treatment. In others, EHE can grow rapidly, spread and even be fatal.
  • There are several treatments available for epithelioid hemangioendothelioma

Is EHE cancer?

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 Boston Children's Hospital approaches epithelioid hemangioendothelioma

Because epithelioid hemangioendotheliomas are so rare, very few doctors have experience diagnosing and treating it. The Vascular Anomalies Center at Boston Children's, 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 first step in developing a treatment plan is having an accurate diagnosis.  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 takes an interdisciplinary approach to care with every child they see, whether the patient is initially reviewed at our conference or seen in clinic. It's not uncommon for children on their first visit to the clinic to be evaluated by a whole VAC team at the same time. From there, the team works with you to develop and carry out a comprehensive and coordinated care plan that matches your child's specific needs. The team brings the expertise of other Boston Children's departments and services as necessary to provide your child with the best care.

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.

Epithelioid hemangioendothelioma: Reviewed by Cameron Trenor III, MD, © Boston Children's Hospital, 2015

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