You may not have heard this name before. Epithelioid Hemangioendothelioma (EHE) is a very, very rare cancer, occurring in about one in a million people. The name is a bit complicated, so it can be scary. But don't worry. It's important to understand this rare condition simply and clearly. So today, let's talk about exactly what EHE is.
Simply put, what kind of cancer is EHE?
Imagine that the blood vessels ( arteries , veins, and capillaries) throughout our bodies are like a network of roads that stretch across a country. The inside of these roads is lined with a very thin layer of cells. These cells are called endothelial cells in medical science.
Endothelial cancer starts in the cells that line the blood vessels. Since blood vessels are everywhere in our bodies, EHE cancer can develop anywhere in the body. However, these tumors most often start in the liver, lungs, or bones . However, they can also develop in other areas, such as the head, neck, thyroid, stomach, ovaries, prostate, brain, and spinal cord.
Sometimes EHE can appear as a single lump. Other times, it can appear as multiple lumps in the same organ or in multiple organs. Often, when EHE is diagnosed, the cancer has already spread to more than one organ.
Is EHE a type of sarcoma?
Yes, that's right. Sarcoma is a type of cancer that develops in the soft tissues of our body, such as blood vessels. Because EHE cancer begins in the endothelial cells of blood vessels, it is classified as a type of sarcoma.
Is this cancer malignant?
Yes, EHE is malignant , meaning it is a malignant or cancerous condition. Unlike benign tumors, these cancer cells can grow uncontrollably, spread (metastasize) to other parts of the body, and damage healthy tissue.
But here's something to keep in mind. EHE cancer is very complex. Some EHE tumors spread rapidly, like very aggressive cancers. But others grow very slowly, over many years. Surprisingly, there have been reports of these tumors shrinking without any treatment. Because of this variable behavior, it is difficult to predict the severity of this cancer and how it will respond to treatment.
What are the symptoms of EHE?
Many patients with EHE do not experience any symptoms at first. This is why it is often discovered incidentally during tests for another condition. However, if symptoms do occur, they depend on the location of the cancerous lump.
Some common symptoms that can be seen are:
- A lump that can be felt under the skin (this may or may not be painful)
- Weight loss without reason
- Fever and extreme fatigue
Let's take a look at how symptoms vary depending on the location of the cancer.
| Location of the cancer | Possible symptoms |
|---|---|
| Liver | Stomach pain, bloating. |
| Lungs | Difficulty breathing, coughing, coughing up blood, clubbing. |
| Bones | Bone pain, frequent fractures. |
| Spine | Difficulty walking or moving, back pain. |
What causes EHE cancer?
Cancers like EHE develop when a healthy cell transforms into a cancer cell and begins to divide uncontrollably.
In trying to figure out what causes this, researchers have found that it is caused by a specific genetic mutation . Simply put, two chromosomes in our cells are linked together incorrectly, causing two genes to become entangled. These genes are called (WWTR1) (also called (TAZ) ) and (CAMTA1) . When these two genes become entangled together, it causes EHE cancer cells to start forming.
Therefore, your doctor may also test for this abnormal chromosome association when making a diagnosis.
How is the diagnosis made?
As we discussed earlier, EHE is often discovered incidentally. Doctors only notice the lump during an imaging test, such as a CT scan or MRI scan, for another reason. They then look for the location, size, and whether it has spread to other areas.
These are the most commonly used tests to diagnose EHE.
| Test | Simply put, what happens? |
|---|---|
| CT Scan | X-rays are used to take a series of cross-sectional images of the inside of your body. This can help identify EHE tumors in the chest, abdomen, and pelvis. This helps to clearly see what's inside the organs, bones, and muscles. |
| MRI scan (MRI) | A powerful magnet and radio waves are used to make very clear pictures of the inside of the body. This is especially useful for checking for EHE tumors in the liver and bones. Sometimes a whole-body MRI, which covers the entire body from head to toe, can help find tumors that have spread to the bones. |
| Core-Needle Biopsy | This is the most important test to confirm if a lump is EHE after a scan. A small piece of tissue is removed from the lump using a special hollow needle. A specialist doctor called a pathologist examines this piece of tissue under a microscope to determine if there are any cancer cells and what type they are. |
In addition, your doctor may recommend other tests, such as an ultrasound or PET scan, to identify lumps and see if they have spread.
What are the treatments for EHE?
Because EHE is such a rare condition, there are no standard treatment guidelines for it yet. Therefore, you will be treated by a multidisciplinary team of doctors who have experience treating sarcomas. This team may include a surgical oncologist , a radiologist , and, depending on the organ in which the cancer is located (for example, bone), an orthopedic surgeon , or a liver transplant specialist.
The treatment you receive depends on several factors, including the size of the lump, its location, how many lumps there are, and whether the cancer has spread.
Here are some treatment options:
Watchful Waiting
If you have no symptoms and are not a good candidate for surgery, your doctor may decide to not treat you at first and just monitor your condition closely. This is because sometimes the side effects of treatment can outweigh the benefits. As we mentioned earlier, some EHE tumors shrink without treatment or grow very slowly. However, if the condition worsens, your doctor may suggest a different treatment option.
Surgery
The most common treatment for a single tumor is surgery. In this procedure, the surgeon removes the cancerous tumor and a small amount of healthy tissue around it to make sure that no cancer cells are left.
Vascular Embolization
This involves blocking the blood vessels that supply the tumor with blood. Just as a plant dies when you stop watering it, when the blood supply is cut off, the tumor cannot grow or shrinks. This can be done to shrink the tumor before surgery or as the main treatment. Transarterial chemoembolization (TACE) and radioembolization are two such treatments.
Targeted Therapy
This is the most common treatment for advanced EHE. This treatment uses drugs that block the nutrients and oxygen that cancer cells need to grow.
Radiation Therapy
This treatment can be used to destroy any cancer cells that may remain after surgery. Radiation therapy can also be given as the main treatment for someone who cannot have surgery. In this treatment, targeted high-energy beams of radiation are used to destroy cancer cells.
Chemotherapy
Chemotherapy is not commonly used for EHE, but if the cancer is in an advanced stage, it may be recommended in combination with other treatments.
Organ Transplant
If multiple tumors have affected a major organ (most often the liver), the doctor may recommend an organ transplant.
Research into EHE is still ongoing, and new treatments are being tested in clinical trials . Depending on the severity of your condition, your doctor may suggest that you participate in one of these research-based treatments.
How long can you live with EHE?
When you hear this, you may be wondering, 'How long can I live then?' To be honest, it's hard to give a straight answer to that question. Because EHE is a very rare condition, information about it is still being collected.
Also, as we discussed earlier, this cancer doesn't behave the same way in everyone. For some, the cancer can spread rapidly even with treatment. For others, the cancer can live for years without any major symptoms or the tumor spreading. Some tumors shrink without treatment.
Therefore, the best information about your condition and future can only be obtained from your treating doctor. He will consider all your test reports and condition and provide you with the most accurate information.
Questions to ask your doctor
It's normal to have a lot of questions when you learn about such a rare condition. When you go to see your doctor, don't forget to ask these questions.
| Question to ask | Why is this important to know? |
|---|---|
| Where is my cancerous lump? | Symptoms and treatments vary depending on the location. |
| What are the symptoms that can help me know if the lump is growing? | This will help you be notified quickly if there are any changes. |
| What factors affect the prognosis of my condition? | This is important to gain a realistic understanding of your situation. |
| What treatments do you recommend for me? What are their side effects? | You can make a clear decision about treatment and be prepared for side effects. |
| Who are the specialists involved in my treatment team? | Get to know the team that is taking care of you. |
| How often will I have to come for follow-up? | Learn how to keep track of your health. |
It's always important to seek treatment from doctors you trust. It's even more important when it comes to a rare condition like EHE. Since there's still so much we don't know about this, seeking treatment from doctors who have experience treating sarcomas and complex cancers can make a big difference in your recovery journey.
Take-Home Message
- Epithelioid Hemangioendothelioma (EHE) is a very rare type of cancer that begins in the cells that line blood vessels.
- The behavior of this cancer can vary greatly from person to person. Some grow rapidly, while others grow very slowly or shrink without treatment.
- It is often asymptomatic and may be detected incidentally during a scan performed for another reason.
- A biopsy is essential to confirm the diagnosis.
- Treatment methods are personalized to suit each patient and may include observation, surgery, and targeted therapies.
- Since this is a rare condition, it is important to seek treatment from a team of specialists experienced in sarcoma. Ask your doctor questions and get a good understanding of your condition.
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