Hepatic epithelioid hemangioendothelioma (HEHE) is a very rare type of cancer that develops from epithelial cells in the blood vessels of the liver. Haemangioendothelioma is a term used to describe neoplasms that act in-between a hemangioma (a benignnot cancerous, can grow but will not spread to other body parts growth composed of blood vessel cells) and an angiosarcoma (a malignancy found in the lining of blood vessels).
The liver has many important functions, such as filtering toxins and alcohol, producing bile to dissolve fat, aiding digestion, storing, and releasing sugars and nutrients, protein production to help blood clot and regulating fluid balance in the body. It is the only organ in the body that is able to repair itself if it becomes injured or partially removed.
HEHEs is generally more common in women, and tends to be diagnosed between the ages of 30-40. However, anyone can develop this disease.
Treatment
When cancers are detected, they are 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. However, because of how rare HEHEs are, there is currently no standard staging and grading system for this disease. Instead of staging and grading, your doctor will recommend a treatment plan based on the following factors:
- Cancer location.
- Whether or not the cancer has metastasised.
- Your age.
- General health.
- Your treatment preferences.
Your doctor may also 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 treatment option for you.
Treatment options for HEHE may include:
- Surgery, potentially including:
- Hepatectomycomplete or partial removal of the liver.
- Organ transplant (liver).
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Radiofrequency ablation (RFA)a minimally invasive procedure that uses heat to destroy abnormal tissue and/or cancer cells.
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Immunotherapya treatment that uses a person's immune system to fight cancer.
- 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.
Risk factors
Because of how rare HEHE is, there has been limited research done into the risk factors of this disease.
Symptoms
Early-stage HEHEs are often asymptomatic, and may be found accidentally during a routine check-up. As the tumour progresses some of the following symptoms may appear:
- Abdominal pain, discomfort and/or swelling.
- Fatigue.
- Unexplained weight loss/loss of appetite.
- Nausea and vomiting.
- Jaundiceyellowing of the skin and the whites of the eyes caused by an increase of bile in the blood.
- Fever.
- A hard and potentially painful lump on the upper right side of the abdomen.
Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.
Diagnosis
If your doctor suspects you have a HEHE, 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.
- Imaging tests, potentially including:
- Ultrasounda type of medical imaging that uses soundwaves to create detailed images of the body .
- 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.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Biopsyremoval of a section of tissue to analyse for cancer cells.
