Epithelioid hemangioendothelioma (EHE) is a rare cancerous tumor that forms in cells lining your blood vessels. Most tumors appear in your liver, lungs or bones. EHE varies in severity. Some tumors are aggressive while others grow slowly and never cause symptoms. Surgery, targeted therapy and watchful waiting are all potential treatments for EHE.
Epithelioid hemangioendothelioma (EHE) is a rare cancer that forms from the cells lining your blood vessels (endothelial cells). Blood vessels, including veins, arteries and capillaries, are located throughout your body. Similarly, EHE can form anywhere in your body. Most often, EHE starts in your liver, lungs or bones. However, EHE can develop in other places, including your head, neck, thyroid, stomach, ovary, prostate, brain, spine, etc.
EHE may present as a single tumor, multiple tumors in a single organ or multiple organs. Often, by the time EHE is diagnosed, tumors have spread to more than one organ.
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Yes. Sarcomas are tumors that may form in soft tissue, including the tissue in blood vessels. EHE begins in endothelial cells (cells that line blood vessels).
EHE is a malignant (cancerous) tumor. Unlike benign tumors, EHE can grow out of control, spread to other parts of your body and damage healthy tissue.
However, EHE is complex because some tumors behave like more aggressive cancers and spread quickly. Other tumors grow slowly. Some tumors even shrink without treatment. Because these tumors behave so differently, it can be challenging to predict how serious a tumor is or how it will respond to treatment.
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Epithelioid hemangioendothelioma is exceptionally rare. Less than 1 in 1 million people get this type of tumor. It’s more common in people aged 20 to 60, but children, teens and people older than 60 can also get EHE. EHE is slightly more common in people assigned female at birth, but researchers aren’t sure why.
Most people with EHE don’t notice any symptoms. When symptoms are present, the most common ones include pain, a mass that you can feel beneath your skin or unexplained weight loss.
Often, symptoms depend on where tumors are located. Signs and symptoms of EHE include:
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Malignant tumors, like EHE, form when a healthy cell transforms into a cancer cell that multiplies out of control.
Researchers have discovered that EHE is caused by a unique genetic mutation. It happens when two chromosomes join abnormally, causing one gene to join to another gene. These genes are WWTR1 (also known as TAZ) and CAMTA1. When WWTR1 joins with CAMTA1, it causes EHE.
Your provider may look for signs of abnormal chromosome attachments to diagnose EHE.
Since EHE doesn’t often cause symptoms, most people learn they have one or more tumors during an imaging procedure unrelated to EHE. Your provider will note where the tumor is located, its size, and whether it’s spread to other parts of your body (metastasized).
The most common procedures used to diagnose EHE include:
Your provider may order other tests, like an ultrasound or a PET scan, to identify tumors and see if they’ve spread.
EHE is so rare that there aren’t standard treatment guidelines. Instead, you’ll likely work with several specialists with experience in treating sarcomas. Your care team may include a cancer specialist (surgical oncologist), an imaging specialist (radiologist) and other specialists with expertise in treating a specific organ or body system (such as an orthopedic surgeon, liver transplant specialist, pathologist, etc.), among others.
The treatment you receive depends on various factors, including the tumor’s size, location, how many tumors you have and whether cancer has spread. Treatments may include:
Research on EHE is ongoing, and new treatments are constantly tested in clinical trials. Depending on the severity of your cancer, your provider may recommend you participate in a clinical trial.
Very little information exists about life expectancy with EHE. First, EHE is so rare that researchers are still collecting data about it. Also, the severity of EHE varies on a case-by-case basis. For some people, EHE progresses aggressively even with treatment. Other people never experience symptoms and live with tumors that never spread for several years. Some tumors shrink without treatment.
Ask your provider about your likely outcomes based on your diagnosis.
A note from Cleveland Clinic
Receiving care from medical professionals you trust is always important, but it’s essential with an epithelioid hemangioendothelioma diagnosis. So much is still unknown about these rare sarcomas. Working with providers who have experience treating sarcomas and complex tumors can make all the difference in your care experience. Treatments may range from adopting a hands-off “wait and see” approach to testing new therapies in a clinical trial. Don’t hesitate to ask questions about how your treatment will likely unfold, including potential risks and benefits.
Last reviewed on 05/20/2022.
Learn more about the Health Library and our editorial process.
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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy