Epithelioid hemangioendothelioma Symptoms & Causes

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What are the symptoms of EHE?

The symptoms of EHE depend on the tumor's location, and can differ from patient to patient. Some patients experience no symptoms at all, and find out that they have an EHE when undergoing an imaging test (e.g., MRI, CT scan) for another reason.

EHE tumors can arise anywhere in the body, they are most often found in the liver, lung and bones.

  • Tumors in the liver may cause abdominal pain, weight loss, blood work alterations or an abdominal mass.
  • Lesions in the lungs may cause chronic dry cough, shortness of breath or other problems.
  • EHE in the bone can cause pain or weaken the bone, leading to increased risk of fracture.
  • Skin lesions may be confused with other skin conditions at first. Often, doctors do not consider the diagnosis of EHE involving the skin until after a biopsy.

Keep in mind, this condition is exceedingly rare, and the presence of any of these symptoms does not necessarily mean that your child has EHE.

What causes EHE?

The cause is currently unknown. It is not inherited—therefore, relatives and future children are not at increased risk for developing EHE. However, researchers recently discovered genetic translocations (a problematic connection or fusion of two genes, leading to unregulated gene activity) that could serve as targets future EHE therapies.

When does it appear?

EHE typically appears in young adults, though the tumor may be diagnosed in younger or older adults.

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

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