Epithelioid hemangioendothelioma, or EHE, is a rare and slow-growing vascular sarcomacancer arising from bones and/or soft tissue that is often associated with bloodthe red bodily fluid that transports oxygen and other nutrients around the body vessels. It is classified as one of the most aggressive of vascular tumours, with the highest potential for metastasiswhen the cancer has spread to other parts of the body, also known as mets. It is most commonly diagnosed on the liver, lung, bone, or soft tissuetissue/the material that joins, holds up or surrounds inside body parts such as fat, muscle, ligaments and lining around joints, 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 cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs 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 cellsthe basic structural and functional unit of all living things look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumoura tissue mass that forms from groups of unhealthy cells) indicates the size and depth of the tumour.
- N (nodea small lump or mass of tissue in your body) indicates whether the cancer has spread to nearby lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid.
- 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 tissuea group of cells that work together to perform a function.
- 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 localisedaffecting only one area of body 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 advancedat a late stage, far along or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells 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 testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, 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:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence to remove as much of the tumour as possible.
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Watch and waitthe close monitoring of a cancer without giving treatment until symptoms appear or worsen.
- Embolisationa treatment that aims to reduce blood flow to the cancer and ultimately reduce cancer growth.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Some of the information regarding treatment was obtained from the Epitheloid Hemangioendothelioma (EHE) page published by National Cancer Institute.
Risk factors
Because of how rare EHE is, there has been limited research into the riskthe possibility that something bad will happen factors of this disease. However, because EHE is caused by a genetic malfunction, it is not classified as hereditary.
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 diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results. Some of the common symptoms include:
- Abdominal pain/discomfort.
- Nauseato feel sick or likely to vomit.
- Dyspneadifficulty breathing, shortness of breath.
- 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.
Some of the information regarding symptoms was obtained from the Epitheloid Hemangioendothelioma (EHE) page published by National Cancer Institute.
Diagnosis
EHE is difficult to diagnose. If your doctor suspects you have EHE, they may order the following tests to confirm the diagnosis and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Imagingtests that create detailed images of areas inside the body tests, potentially including:
- MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology to create detailed images of the body.
- PET (positron emission tomography) scana type of medical imaging that uses radioactive tracers to create detailed images of the body.
- X-raya type of medical imaging that uses x-ray beams to create detailed images of the body .
- Blood teststesting done to measure the levels of certain substances in the blood.
- Biopsyremoval of a section of tissue to analyse for cancer cells.
