Alveolar soft part sarcoma is a very rare type of soft tissue sarcoma.
Alveolar soft part sarcoma, also known as “ASPS”, is a very rare sarcoma that can occur anywhere throughout the body. But, it often occurs in the arms and legs.
ASPS is thought to be caused when certain chromosomes (the parts within cells that contain DNA) are broken apart and put back together in the wrong way. Researchers are still trying to find out why this happens and whether this causes ASPS.
There are an average of 6 cases of ASPS diagnosed every year in England.
ASPS makes up 0.15% of all soft tissue sarcomas.
For more information on these statistics, you can visit our soft tissue sarcoma data hub.
Signs and symptoms
Symptoms of ASPS can vary depending on the size and location of your tumour. You may experience all, some or none of these symptoms before you are diagnosed with ASPS:
- A lump or swelling in the soft tissue of the body under the skin, often in the arms or legs
- This lump is often slow-growing, soft and painless
A specialist doctor will diagnose ASPS through a series of tests. These may include:
- Physical examination – looking at and feeling any lump
- A scan – taking pictures of the inside of the body using scans such as CT or MRI scans
- A biopsy – taking and testing a sample of the tumour. Using this biopsy, a doctor will look to see if the biopsy looks like ASPS and whether it contains the chromosome error
To read more about these scans and tests, click here.
- The first treatment method for ASPS is surgery. The surgeon will remove the tumour and will aim to take out an area of normal tissue too – this is known as “taking a margin”. This is to make sure that all of the cancer has been removed
- ASPS mostly affects the arms and legs, and surgeons will do limb-sparing surgery so that the limb can continue to work well
- Although very rare, if the cancer has spread throughout the limb, then the surgeons may have to perform a partial or full amputation to stop the cancer
- This treatment uses high-energy radiation to destroy cancer cells
- It can be used either before or after surgery
- When used before surgery, radiotherapy aims to make the tumour smaller so that it can be more easily removed
- When used after surgery, radiotherapy aims to kill off any remaining cancer cells that have not been removed by surgery
- This treatment uses anti-cancer drugs to destroy cancer cells. It is sometimes used in patients that have a high risk of the cancer coming back or if it has spread to other parts of the body, also known as “metastasis”
- Unfortunately, chemotherapy is not currently recommended for ASPS. This is because research has found that current chemotherapy is not effective for the treatment of ASPS
Targeted and experimental therapies
- As chemotherapy does not currently work well for ASPS, researchers are looking for better treatments. In ASPS, researchers have been learning more about treatments known as “targeted therapies” and “immunotherapies”
Targeted therapy is a treatment that targets specific characteristics within cancer cells in order to stop them from growing and spreading.
Immunotherapy is a treatment that helps the person’s own immune system to fight and kill cancer cells.
- Researchers have found that some targeted therapies and immunotherapies worked well in clinical trials of ASPS. But, more research and trials are needed before these treatments are approved
- You may be offered an opportunity to take part in a trial to investigate new diagnosis methods, drugs or treatments. Some studies also look at the care and well-being of patients. Your doctor or nurse can give you more information on opportunities for you to take part in a clinical trial
For all sarcoma clinical trials based in the UK that are currently recruiting, you can check our Clinical Trials Hub.
After treatment, you will have regular follow-up appointments for several years. You should receive a follow-up schedule from your sarcoma clinical nurse specialist. The follow-ups will usually include:
- A chance to discuss symptoms
- An examination to look for any signs of ASPS returning. This may include scans such as CT or MRI scans
- A chest x-ray to rule out any secondary cancers occurring in the lungs
ASPS can reappear in the same area after the treatment of a previous tumour; this is called a “local recurrence”.
If the cancer does reappear, it is important to get treated as quickly as possible. This could involve further surgery and/or radiotherapy; your treatment will be assessed on an individual basis. It is useful to check for recurrences yourself through self-examination: your doctor or sarcoma clinical nurse specialist can tell you what to look for.
If you are worried about your cancer returning, please contact your doctor or nurse. They may decide to bring forward the date of your follow-up appointment to investigate your concerns.
What if my cancer spreads to another part of my body?
A recurrence of ASPS may be accompanied by cancer in other parts of the body. This is called “metastasis” or “secondary cancer”. Some people are diagnosed with sarcoma because their metastases have been discovered before their primary ASPS tumour. In ASPS patients, these secondary cancers may appear in the lungs, which is why a chest x-ray is taken at follow-up appointments.
Secondary cancers may also appear in the liver, bone, brain and lymph nodes. Treatment for secondary cancer may involve surgery, radiotherapy or other therapy as appropriate; your treatment will be assessed on an individual basis.