What is epithelioid hemangioendothelioma?
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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Is epithelioid hemangioendothelioma a sarcoma?
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).
Is epithelioid hemangioendothelioma malignant?
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.
How common is EHE?
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.
FAQs
What causes epithelioid hemangioendothelioma? 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.
How long can you live with epithelioid hemangioendothelioma? ›
Epithelioid hemangioendothelioma tumors affect each person differently. Some people can live with these slow-growing tumors for years with only minor disruption to daily life. Sometimes the tumors even go away on their own. Less commonly, the tumors grow and spread quickly.
What is the survival rate for hemangioendothelioma? ›
The overall 1-, 3-, and 5-year survival rates were 70.8% (95%CI = 48.4–84.9%), 61.2% (95%CI = 38.5–77.7%), and 55.6% (95%CI = 32.8–73.5%), respectively. The age-adjusted incidence rate of epithelioid hemangioendothelioma from 1975 to 2019.
How many people have epithelioid hemangioendothelioma? ›
How common is epithelioid hemangioendothelioma? EHE is very rare, with only one in every one million people diagnosed with this cancer worldwide.
What are the symptoms of epithelioid? ›
Epithelioid Sarcoma Symptoms and Signs
- One or more lumps that can continue to grow.
- Open skin wound above the lump.
- Pain if the tumor forms near a joint or pushes on muscles or nerves.
- Swelling.
Doctors often treat epithelioid mesothelioma with a combination of treatments, which is referred to as multimodal therapy. Early-stage cases typically get aggressive treatment with surgery, chemotherapy and radiation therapy.
Is epithelioid hemangioendothelioma hereditary? ›
What causes epithelioid hemangioendothelioma? The cause of EHE is currently unknown. It is not inherited, so relatives and future children are not at increased risk.
How long can you live with epithelioid sarcoma? ›
Many research studies have reported on epithelioid sarcoma survival rates through the years. But because the disease is so rare, the numbers vary significantly. The five-year survival rate for epithelioid sarcoma ranges from 25% to 92%, depending on which study you read.
What are the clinical features of hemangioendothelioma? ›
Pain is the most common symptom. Other symptoms, including cough and a palpable mass, weight loss and fatigue, have also been reported. Specifically, in cases of hepatic EHE, patients often present right upper quadrant pain, hepatomegaly and weight loss (27).
What is Stage 4 epithelioid hemangioendothelioma sarcoma? ›
Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.
What is the genetic cause of EHE? As is the case with many other cancers, EHE is caused by a genetic malfunction, a specific chromosomal rearrangement or translocation (swapping of genetic material) called the 'WC Fusion. ' This discovery was made by dedicated scientists studying the molecular biology of EHE.
Are epithelioid cells cancerous? ›
Epithelioid sarcoma is a rare type of cancer that starts as a growth of cells in the soft tissue. It can happen anywhere on the body. It often starts under the skin on the finger, hand, forearm, knee or lower leg. Epithelioid sarcoma might cause a small, firm growth or lump under the skin, which is called a nodule.
What is the cause of epithelioid sarcoma? ›
What Causes Epithelioid Sarcoma? Experts do not know what causes epithelioid sarcoma, but the disease is linked with an abnormality in the SMARCB1 gene (sometimes called INI-1). Doctors can order testing of the tumor to identify this abnormality when confirming a diagnosis of epithelioid sarcoma.
What is the origin of hemangioendothelioma? ›
Hemangioendothelioma is a rare tumor arising from the vascular endothelium of soft tissue, skin, bone, and viscera. The disease has an indolent course, with the potential for recurrence, and is often associated with multi-organ involvement.
