Hepatic Epithelioid Hemangioendothelioma (HEHE)

Hepatic epithelioid hemangioendothelioma (HEHE) is a very rare type of cancerA disease where abnormal cells split without control and spread to other nearby body tissue and/or organs. Cancer cells can also spread to other parts of the body through the bloodstream and lymph systems. that develops from epithelial cells
• the smallest, living parts of the body. Cells work together to form or build the body
• a human is made up of millions of Cells
• Cells reproduce themselves to make sure a body stays working
• sometimes Cells can be abnormal or damaged and these can be cancer cells
in the bloodhello 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, metastasisKnown as secondary cancer, it grows/spreads from the original/ primary cancer. (whether the cancer has spread to other parts of the body) 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 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 treatment option for you.

Treatment options for HEHE may include:

• Surgery, potentially including:
  • Hepatectomy (removal of all or part of the liver).
  • Liver transplant.
• Chemotherapy
  • a chemical drug treatment to kill or slow-growing cancer Cells
  • these drugs are called cytotoxic drugs
  .
• Radiofrequency ablation therapy (RFA).
• Radiation therapy.
• Immunotherapy.
• Clinical trials.
• Palliative careLessening pain without curing the disease..

For more information on the treatment options, please refer to the Rare Cancers Australia treatment options page.

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.
• Jaundice (yellowing of the skin and whites of the eyes).
• Fever.
• A hard and potentially painful lump on the upper right side of the abdomenstomach, stomach area, belly, tummy.

Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.

Diagnosis/diagnosing

If your doctor suspects you have a HEHE, they may order the following tests to confirm the diagnosisUsing medical test results, identify and name a disease and/or condition. and refer you to a specialist for treatment.

Physical examination

Your doctor will collect your overall medical history, as well as your current symptoms. Following this, they may examine your body to check for any abnormalities.

Imaging & blood tests

The doctor will take images of your body using magnetic resonance imagingTaking images/photos of inside body parts using magnet rather than x-ray. (MRITaking images/photos of inside body parts using magnet rather than x-ray.), a computed tomography scan (CT scanA 3-D (three dimensional) x-ray pictures that gives more information than a normal x-ray.), and/or positron emission tomography (PET scanA test that uses a radioactive drug to show a picture of how your tissues and organs are working. Also known as a positron emission tomography scan.), depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and looks for signs of metastasis. Additionally, a blood test may be taken to assessTo measure, look at and learn from. your overall health and help guide treatment decisions.

Biopsy

Once the location(s) of the cancer has been identified, the doctor will perform a biopsyTo take a small piece of body tissue and test it in a laboratory. to remove a section of tissue using a needle. The tissue sample will then be analysed for cancer cells. This can be done by a fine needle aspiration (FNA) or a core needle biopsy (CNB).

References

• NCBI – Hepatic epithelioid hemangioendothelioma: Clinical characteristics, diagnosis, treatment and prognosis (2022)
• NCBI – Hepatic epithelioid hemangioendothelioma: Update on diagnosis and therapy (2020)
• Radiopaedia