Angiosarcomas are rare types of sarcomas (cancers arising from bone or soft tissue) that develop in the lining of blood vessels (haemangiosarcoma) or lymph vessels (lymphangiosarcomas) throughout the body. They are most commonly found in the skin, but can also be found in the breast, liver, spleen, bone or heart.
Angiosarcomas are more common in men, and are often diagnosed over the age of 70. However, anyone can develop this disease.
Types of Angiosarcoma
There are several different types of angiosarcoma, which are classified by the types of cells they develop from.
Cutaneous Angiosarcoma
Cutaneous angiosarcomas are the most common form of angiosarcoma, and they develop in the skin. These types of angiosarcomas are generally subcategorised by the presence or absence of lymphoedema.
Cutaneous Angiosarcoma with associated Lymphoedema
Cutaneous angiosarcoma with associated lymphoedema, also known as Stewart-Treves syndrome, is the development of a lymphangiosarcoma as a rare complication of long-term or chronic lymphoedema. Lymphoedema is a condition that causes a part of the body to swell, most often a limb. It is caused by a blockage in the lymphatic system, a network of tissues and organs that help our bodies fight infection and disease.
In many cases, cutaneous angiosarcoma with associated lymphoedema occurs after a radical mastectomy (removal of the breast(s)) and lymphadenectomy (removal of affected lymph nodes) to treat breast cancer. It is often aggressive, and may not carry as good of a prognosis as other types of cancer.
Cutaneous Angiosarcoma without associated Lymphoedema
Cutaneous angiosarcoma without associated lymphoedema, also known as Wilson-Jones angiosarcoma, senile angiosarcoma, or malignant angioendothelioma, is the most common subtype of cutaneous angiosarcoma. It develops as a haemangiosarcoma, and is generally found in the head and neck. These cancers can often be misdiagnosed, are often aggressive, and may not have as good of a prognosis as other types of angiosarcoma.
Angiosarcoma of the Breast
Breast angiosarcomas are very rare forms of angiosarcoma that develop in the breast. Unlike most other types of angiosarcoma, this type develops most commonly in women between the ages of 30 and 40. Breast angiosarcomas are often more aggressive than other types of breast cancers, and may not have as good of a prognosis as other breast cancers.
Angiosarcoma of Deep Soft Tissue
Angiosarcomas of deep soft tissue are a relatively common type of angiosarcoma, but are often difficult to diagnose. They often appear as a mass in the limbs, or in/around organs such as the liver, spleen, or heart. The symptoms of these tumours often vary based on tumour location, and generally occur equally in men and women. Angiosarcomas of deep soft tissues are often aggressive, and can have varied prognoses based on tumour location.
Radiation-induced Angiosarcoma
Radiation-induced angiosarcomas are a rare subtype of angiosarcoma that develop as a complication of previous radiation treatment. In many cases, they can develop in the abdomen following radiation for gynaecological malignancies, or in the breast following breast-sparring surgery to treat breast cancer. Radiation-induced angiosarcoma can develop several years after treatment, and may be diagnosed at a later stage of disease. It can be aggressive, and may not have as good of a prognosis as other types of angiosarcoma.
Angiosarcoma Associated with Foreign Material
Angiosarcomas associated with foreign material are a very rare subtype of angiosarcoma that are associated with foreign material that have been in the body for extended periods of time. This may include materials implanted accidentally, such as shrapnel, or material implanted for medical purposes, such as dialysis shunts made of Dacron (a type of polyester fibre), or metal for orthopaedic procedures. These tumours behave similarly to angiosarcomas of deep soft tissue, and are often aggressive.
Treatment
If an angiosarcoma is detected, it will be staged and graded based on size, metastasis (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.
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 had 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 known 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 tumours. 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 treatment option for you.
Treatment is dependent on several factors, including location, age, stage of disease and overall health.
Treatment for angiosarcomas may include:
- Surgery, potentially including:
- Local excision (removal of tumour and a healthy margin of tissue around it).
- Mastectomy (complete removal of one or both breasts).
- Hepatectomy (partial or complete removal of the liver).
- Splenectomy (removal of part or all of the spleen).
- Radiation therapy.
- Chemotherapy.
- Clinical trials.
- Palliative care.
For more information on the treatment options, please refer to the Rare Cancers Australia treatment options page.
Risk factors
While the cause of angiosarcomas remain unknown, the following factors may increase the likelihood of developing the disease:
- Previous radiation therapy for other conditions.
- Having lymphoedema.
- Exposure to certain chemicals, potentially including:
- Vinyl chloride.
- Arsenic.
- Thorium dioxide.
- Having certain genetic conditions, such as:
- Having genetic mutations in the BRCA1 and/or BRCA2 genes.
Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.
Early symptoms
The symptoms of an angiosarcoma will generally vary based on location, and may be asymptomatic in the early stages of disease.
Symptoms of Cutaneous Angiosarcoma
Symptoms of a cutaneous angiosarcoma may include:
- Persistent bruises.
- Bruises with an unusual appearance, potentially including ulcerated, plaque-like, or nodular bruises.
- Unhealed ulcerations.
- Bleeding or painful lesions.
- Growing bruises.
- Bruises appearing red in colour.
- Swelling around the skin lesion.
Symptoms of Angiosarcoma of the Breast
Symptoms of angiosarcoma of the breast may include:
- A painless lump in the breast.
- Unexplained breast enlargement.
- Blue-ish skin discolouration of the breast.
- Change in size or shape of the breast.
- A change in size or shape of the nipple.
- A rash or bruising of the nipple.
- Swelling around the armpit.
Symptoms of Angiosarcoma in Deep Soft Tissue
Symptoms of angiosarcoma in deep soft tissue will often vary with location.
General Symptoms
General symptoms of an angiosarcoma in deep soft tissue may include:
- A rapidly growing lump in the limbs or abdomen.
- Internal bleeding.
- Anaemia, with symptoms potentially including:
- Fatigue.
- Dizziness.
- Weakness.
- Shortness of breath.
- Paleness.
- Haematomas.
- Enlarged lymph nodes.
Angiosarcoma in the Liver
In addition to the symptoms above, people with an angiosarcoma in the liver may also experience:
- Fatigue.
- Unexplained weight loss/loss of appetite.
- Pain in the upper right abdomen.
Angiosarcoma in the Spleen
In addition to the symptoms above, people with an angiosarcoma in the spleen may also experience:
- Fever.
- Fatigue.
- Unexplained weight loss/loss of appetite.
- Pain in the upper right abdomen.
- Pain on the left side of the abdomen.
- Chest pain.
Angiosarcoma in the Lungs
In addition to the symptoms above, people with an angiosarcoma in the lungs may also experience:
- Chest pain.
- Blood in sputum.
- Unexplained weight loss/loss of appetite.
- Cough.
- Difficulties breathing.
Symptoms of Radiation-Induced Angiosarcoma
Symptoms of radiation-induced angiosarcoma vary by location. Patients with radiation-induced angiosarcoma may experience the symptoms of deep soft tissue sarcoma, and/or breast angiosarcoma, depending on where exactly the cancer is located.
Symptoms of Angiosarcomas Associated with Foreign Material
Because of how rare this subtype is, the symptoms of angiosarcomas associated with foreign materials have not been adequately researched.
Not everyone with the symptoms above will have cancer, but see your GP if you are concerned.
Diagnosis/diagnosing
If your doctor suspects you have an angiosarcoma, they may order the following tests to confirm the diagnosis 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 imaging (MRI), a computed tomography scan (CT scan), x-rays, bone scan 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 looks 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 of the cancer has been identified, the doctor will perform a biopsy to remove a section of tissue using a needle. In most cases, this will be done by a core-needle biopsy, which involves a larger, hollow needle being inserted into a small incision above the area to be biopsied. Once a sample has been removed, it will be sent to a lab and 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 the rate and depth of tumour growth, susceptibility to treatment, age, overall fitness, and medical history. Generally, early-stage angiosarcomas have a better prognosis and survival rates. However, if the cancer is advanced and has spread, the prognosis may not be as good and there may be a higher risk of cancer recurrence. It is very important to discuss your individual circumstances with your doctor to better understand your prognosis.
References