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Epithelioid hemangioendothelioma, also called EHE sarcoma, is a type of sarcoma that starts in cells that line blood vessels. While it can appear anywhere in the body, it is most often found in the liver, lungs and bones.
It is an unpredictable disease. In some people, tumors grow very slowly or even shrink, but in others, EHE sarcoma can be more aggressive and spread quickly. EHE sarcoma is rare, affecting less than one in every million people.
EHE Sarcoma Care Tailored to You
You and your family are our top priority. At Fred Hutch Cancer Center, we offer comprehensive and compassionate care — personalized to you. You'll have access to the latest treatment options, clinical trials and supportive care services.
Symptoms and Signs
Many people with EHE sarcoma do not have any symptoms at first, and the cancer is often discovered by accident during a test for a different problem. When symptoms do show up, they can vary depending on where the tumor is located.
For example, if EHE sarcoma is in the lungs, a person might have a cough or trouble breathing. If they have EHE in the liver, it might cause pain in their abdomen. Common signs are pain, a lump you can feel under your skin or losing weight without trying. Other symptoms can include a fever and extreme tiredness. EHE sarcoma can also cause bones to break if the tumor is growing in a bone.
Causes and Risk Factors
Researchers have found that EHE sarcoma is caused by a genetic change. It happens when two chromosomes in a cell break and swap pieces, which creates a new type of gene that tells cells to grow out of control.
EHE sarcoma is not passed down through families, meaning you cannot inherit it from your biological parents or pass it on to your children.
It is most often found in people between 30 and 50 years old, but it can affect people of any age.
EHE Sarcoma Care Team
At Fred Hutch, we offer comprehensive treatment from a team of experts who specialize in soft tissue sarcomas and bone cancers.
Learn more about sarcoma specialists at Fred Hutch.
Diagnosing EHE Sarcoma
To find out if someone has EHE sarcoma, a physician will perform a physical exam as well as order imaging tests like CT scans, MRIs or PET scans. The pictures will allow them see the size of the tumor and if it has spread.
While scans give important information, the only way to be sure and confirm a tumor is EHE sarcoma is through a biopsy. During a biopsy, a physician takes a small sample of the tumor, and sends it to a lab to be analyzed. At the lab, they may also look for specific genetic markers to confirm the diagnosis is EHE sarcoma. The disease is also staged at this time.
Learn more about diagnosing and staging sarcoma.
Prognosis and Survival
When physicians and scientists talk about cancer survival rates, they typically mean the percentage of people expected to survive their cancer for at least five years after being diagnosed. (The rate doesn’t include the risk of dying during that time for some other reason.) This information appears on a large National Cancer Institute database called SEER, which stands for the Surveillance, Epidemiology, and End Results Program.
However, because EHE sarcoma is so rare, there is no specific SEER data for it. Instead, physicians may look at specialized research to estimate prognosis and survival rates. To better understand your unique situation, talk to your Fred Hutch physician.
Learn more about prognosis and survival of sarcoma.