Epithelioid hemangioendothelioma, or EHE, is a rare and slow-growing vascular sarcoma (bone and soft tissue cancer) that is often associated with blood vessels. It is classified as one of the most aggressive of vascular tumours, with the highest potential for metastasis (the process where cancer cells break away from primary cancer and form tumours in other parts of the body). It is most commonly diagnosed on the liver, lung, bone, or soft tissue, but can develop anywhere in the body.
EHE affects men, women, and children of all age groups. However, it is more commonly diagnosed in females and appears to be more prevalent in young adults. It is often misdiagnosed, and acts in an unpredictable manner. As this type of cancer is incredibly rare, there has been limited research into treatment.
Treatment
Each patient with EHE will present with a unique disease behaviour, with varying stages, locations and symptoms. As such, there is no one treatment method that will work for everyone.
If EHE is detected, it will be staged and graded based on size, metastasis and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumour) indicates the size and depth of the tumour.
- N (nodes) indicates whether the cancer has spread to nearby lymph nodes.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0 – IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and has not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also classified as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade I cancers. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate course of treatment for you.
Treatment options for EHE may include:
- Surgery to remove as much of the tumour as possible.
- Radiation therapy.
- Chemotherapy.
- Watch and wait.
- Clinical trials.
- Palliative care.
For more information on treatment options, please refer to the Rare Cancers Australia treatment options page.
Risk factors
Because of how rare EHE is, there has been limited research into the risk factors of this disease. However, because EHE is caused by a genetic malfunction, it is not classified as hereditary.
Early symptoms
Early symptoms of EHE will vary, as it depends on tumour location. As it is commonly misdiagnosed, patients may present with symptoms several months or, in extreme cases, years before receiving an accurate diagnosis. Some of the common symptoms include:
- Abdominal pain/discomfort.
- Nausea.
- Difficulties breathing.
- Coughing and/or coughing up blood.
- Pain and swelling in the area of the tumour.
- Unexplained weight loss.
- Problems with mobility.
- Skin masses.
- Easily broken bones.
Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.
Diagnosis/diagnosing
EHE is difficult to diagnose. If your doctor suspects you have EHE, they will order a range of diagnostic tests.
Imaging & blood tests
The doctor will take images of your body using magnetic resonance imaging (MRI), a computed tomography scan (CT scan), x-ray, and/or positron emission tomography (PET scan), depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and look for signs of metastasis. Additionally, a blood test may be taken to assess your overall health and help guide treatment decisions.
Biopsy
Once the location(s) of the cancer has been identified, the doctor will perform a biopsy to remove a section of tissue using a needle. The tissue sample will then be analysed for cancer cells.
Prognosis (Certain factors affect the prognosis and treatment options)
While it is not possible to predict the exact course of the disease, your doctor may be able to give you a general idea based on rate and depth of tumour growth, susceptibility to treatment, age, overall fitness and medical history. However, because there are so few cases of EHE, it may be difficult to receive an accurate prognosis. Some patients live a long time, even without treatment, while others who receive treatment may have a more aggressive and metastatic tumours. It is very important to discuss your individual circumstances with your doctor to better understand your prognosis.
References
Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.