Angiosarcoma is a rare cancer that can develop in different parts of the body and may grow and spread rapidly. Following increased public interest in the disease after the death of Omukama Oyo Nyimba Kabamba Iguru Rukidi IV, Dr Fred Okuku, a senior consultant and oncologist at the Uganda Cancer Institute, explains its symptoms, risk factors, diagnosis and treatment.
What is angiosarcoma, and where in the body does it occur?
Angiosarcomas are a type of sarcoma that arises from connective tissues throughout the body. These include muscles, bones, blood vessels, and cartilage, among others. They are broadly categorised as soft tissue sarcomas, to which angiosarcomas belong, and bone sarcomas. A common example of a bone sarcoma is osteogenic sarcoma, or osteosarcoma.
Angiosarcomas account for less than two percent of soft tissue sarcomas. It is an aggressive cancer that originates from blood vessels or lymphatic channels and commonly occurs in the skin and soft tissues, although it can also arise in any organ of the body. As it grows, it tends to invade and spread to neighbouring tissues.
How common is angiosarcoma, and who is most at risk?
It is a rare cancer, and worldwide data estimates its occurrence at between 0.15 and one case per 100,000 people. Angiosarcoma can occur at any age, but it is more common in older adults.
What causes angiosarcoma?
No definite cause has been found, but there are a number of risk factors thought to be associated with the disease. They include environmental carcinogens, non-cancerous growths in the body such as bone infarcts, exposure to non-environmental radiation and genetic disorders such as congenital retinoblastoma, among others.
What are the early signs and symptoms of angiosarcoma?
The signs and symptoms can be vague and are generally dependent on the part of the body affected. If it is the skin, nodules can appear. If it is in deep organs, there may be pressure symptoms such as pain, a feeling of a mass, abdominal pain, anaemia or internal bleeding.
Angiosarcomas can also originate in the heart muscle, in which case symptoms may be related to the heart. Sometimes, symptoms result from the spread of the tumour, which can move rapidly through the blood to organs including the lungs, liver, bones, soft tissues and brain.
Why is this cancer difficult to detect or diagnose in its early stages?
Because this tumour can begin in any tissue of the body and grows very fast, it can be difficult for an individual to know that they have it because the signs can be vague. Once it has spread, however, it can suddenly lead to multiple symptoms.
Is it possible for someone to have angiosarcoma without obvious symptoms in the early stages?
Yes. An individual can have cancer growing in an area where there is a lot of room for expansion. Symptoms will appear later as the tumour grows, and that is when severe pain may begin. A good example is the chest and abdomen.
How is angiosarcoma diagnosed?
Like any cancer, we use a combination of imaging tests to confirm the location of the tumour, based on the complaints that the individual presents. Some patients may fail to pinpoint the area and, in that case, we employ imaging tests such as CT, MRI and PET scans to locate these tumours before taking a tissue sample, or biopsy, to assist in the definitive diagnosis. A biopsy is a must before a cancer diagnosis can be confirmed.
What treatment options are available for someone with angiosarcoma?
The primary treatment for early angiosarcoma, where the tumours are less than 5cm wide, is surgery. The aim is to remove the tumour without leaving residual cancer cells. If this cannot be achieved, as is the case when the cancer has spread to other parts of the body, we employ multiple treatments such as chemotherapy, radiotherapy and, in some cases, targeted therapy and immunotherapy, which is an active area of ongoing research. In most cases, combination treatments are employed in an attempt to cure patients.
Angiosarcoma is described as an aggressive cancer. What does that mean in practical terms?
An aggressive tumour refers to a tumour that grows fast and has a high propensity to spread to other organs. These developments can happen in weeks or months, sometimes with vague symptoms. This aggressive nature can also be documented on the histopathology report as a high Ki-67 index or a higher grade of tumour.
What factors determine how a patient responds to treatment and their overall prognosis?
Response to cancer treatment is generally determined by multiple factors, with the most important being age. When the body is advanced in age, it may not withstand aggressive cancer treatments such as chemotherapy or surgery.
Others include the grade. The higher the grade of the tumour, the more aggressive it is. Another important factor that corresponds to prognosis is stage, which is the same as the volume of disease. It is generally categorised as stage one to four, with stage four representing a high volume of disease in the body.
Stages three and four are described as advanced cancer, with poor prognosis or outcomes.
Generally, medically speaking, very few people can be cured of stage four cancer. The goal of care in stage four is generally to improve symptoms and prolong survival while optimising quality of life. This sometimes increases the cost of cancer treatment, resulting in what we describe as financial toxicity to the family.
Can angiosarcoma be prevented, or are there steps people can take to reduce their risk?
Since the causes of angiosarcomas are unknown, it is difficult to prevent them because there is no direct cause. The risk factors are multifactorial. For example, cigarette smoking is an established risk factor for 11 different cancers, the most common being lung cancer. Smoking cessation can lead to a reduction in the risk of these cancers.
There are, however, general strategies that may help cut the risk of developing cancers, such as eating a healthy and balanced diet, quitting smoking and alcohol consumption, regular exercise, and reducing high sugar and fat intake. These and many others can help cut the risk of developing cancer.
Individuals with a family history of cancer are normally considered high risk, and regular checks at cancer-based hospitals are advised.
There has recently been increased public interest in angiosarcoma following the death of Omukama Oyo Nyimba Kabamba Iguru Rukidi IV. What would you want the public to understand about the disease beyond this particular case?
It was a shock to us in the cancer field to learn about this, and we commiserate with the family. The country should know that cancer is a unique disease and very challenging to diagnose and treat. Countries all over the world, however advanced, are grappling with a huge number of cancer patients.
The disease requires a high level of advancement in personnel and equipment, as well as an educated population that understands its signs and symptoms.
Many Ugandans are unaware of the kind of investment the Government of Uganda has already made in this area and continues to make. Only those who interface with the system may know something about it. The commitment from government is there, and Ugandans should support these efforts.
Advice
What message would you give Ugandans about recognising and seeking care for symptoms that could potentially be linked to cancer?
With cancer, sometimes there may be no obvious signs or symptoms. The only signs may be unexplained weight loss, bleeding from any part of the body or persistent low energy.
Do not ignore symptoms that persist or do not respond to treatment. Visit an established hospital and ask to be seen by a qualified and registered health practitioner.
Although checking your symptoms on Google may help you understand them, it is important to seek advice from a qualified health professional and, where possible, a specialist in the area related to your symptoms.