Angiosarcomas are rare types of sarcomas (cancers arising from bone or soft tissuetissue/the material that joins, holds up or surrounds inside body parts such as fat, muscle, ligaments and lining around joints) that develop in the lining of bloodthe red bodily fluid that transports oxygen and other nutrients around the body vessels (haemangiosarcoma) or lymph vesselsa network/chain of thin tubes that moves lymph fluid around the body (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 cellsthe basic structural and functional unit of all living things 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 lymphoedemaabnormal swelling in the body (usually in the arms or legs) that is caused by a buildup of lymph fluid.
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 chronica long-lasting disease that changes slowly over time 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 systema network of tissues and organs that help our bodies fight infection and disease, a network of tissues and organs that help our bodies fight infectiona condition where harmful pathogens, such as bacteria, viruses or parasites, have entered the body and disease.
In many cases, cutaneous angiosarcoma with associated lymphoedema occurs after a radical mastectomycomplete or partial removal of one or both breasts and lymphadenectomysurgical removal of lymph node(s) to treat breast cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs. It is often aggressive, and may not carry as good of a prognosisto predict how a disease/condition may progress and what the outcome might be as other types of cancer.
Cutaneous Angiosarcoma without associated Lymphoedema
Cutaneous angiosarcoma without associated lymphoedema, also known as Wilson-Jones angiosarcoma, senile angiosarcoma, or malignantcancerous, may grow and spread to other areas of the body 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 tissuea group of cells that work together to perform a function are a relatively common type of angiosarcoma, but are often difficult to diagnose. They often appear as a massa growth of cells that come together to make a lump, may or may not be cancer in the limbs, or in/around organs such as the liver, spleen, or heart. The symptoms of these tumours often vary based on tumoura tissue mass that forms from groups of unhealthy cells 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 abdomenstomach, stomach area, belly following radiation for gynaecological malignancies, or in the breast following breast-sparring surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence 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 dialysisa procedure that removes excess fluid and/or waste from the blood in people whose kidneys aren't functioning properly 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, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancer cells 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 (tumour) 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 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 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 known 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 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 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 is dependent on several factors, including location, age, stage of disease and overall health.
Treatment for angiosarcomas may include:
- Surgery, potentially including:
- Wide local excisionremoval of cancerous tissue and a margin of healthy tissue around it to prevent cancer recurrence.
- Unilateralaffecting one side or bilateralaffecting both sides mastectomy.
- Hepatectomycomplete or partial removal of the liver.
- Splenectomyremoval of the spleen.
- 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.
- 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
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:
- Neurofibromatosis.
- Klippel-Trenaunay syndrome.
- Maffucci syndrome.
- Having genetic mutations in the BRCA1 and/or BRCA2 genes.
Not everyone with these riskthe possibility that something bad will happen 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.
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.
- Anaemiaa condition where there aren't enough red blood cells in the blood, causing fatigue, weakness and pale skin and affecting how the body responds to infection, with symptoms potentially including:
- Fatiguea state of extreme tiredness or exhaustion, can be physical or mental.
- Dizziness.
- Weakness.
- Dyspneadifficulty breathing, shortness of breath.
- Paleness.
- Haematomas.
- Lymphadenopathyswollen lymph nodes/glands, also known as adenopathy.
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 sputuma mixture of saliva and mucus coughed up from the respiratory tract.
- Unexplained weight loss/loss of appetite.
- Cough.
- Dyspnea.
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 sarcomacancer arising from bones and/or soft tissue, 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
If your doctor suspects you have an angiosarcoma, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results 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.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Biopsyremoval of a section of tissue to analyse for cancer cells.
