Spindle cell sarcoma is a type of undifferentiated sarcoma. It can occur anywhere throughout the body, but it most commonly occurs in the arms and legs.
Spindle cell sarcoma can occur at any age but more commonly affects middle-aged and older people. It affects slightly more males than females.
Spindle cell sarcoma is a “diagnosis of exclusion”. This means that the tumour has no identifiable characteristics, other than the shape of its cells, for the doctors to be able to diagnose it as a specific type of sarcoma.
A diagnosis of spindle cell sarcoma is based on how it looks under a microscope. As the name suggests, the cells look long and narrow. This differs to the most common type of undifferentiated sarcoma, known as undifferentiated pleomorphic sarcoma, or UPS. UPS is made up of a mix of cells with different sizes and shapes.
The cause of most spindle cell sarcomas is unknown. But, exposure to radiation has been shown to cause some spindle cell sarcomas. Researchers are still trying to learn more about the causes of spindle cell sarcoma.
There are an average of 173 cases of spindle cell sarcoma diagnosed every year in England.
Spindle cell sarcoma makes up 4.39% of all soft tissue sarcomas, and about 0.06% of all cancers.
Although spindle cell can affect anyone of any age, the median age at diagnosis is 69 years old.
There are slightly more males than females diagnosed with spindle cell sarcoma.
For more information on these statistics, you can visit our soft tissue sarcoma data hub.
Signs and symptoms
Symptoms of spindle cell sarcoma 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 spindle cell sarcoma:
- A lump or swelling within the soft tissue of the body, which can occur anywhere in the body but most often in the arms and legs
- This lump is often fast-growing and painful
A specialist doctor will diagnose spindle cell sarcoma 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 ultrasound, x-ray, CT, MRI or PET scans
- A biopsy – taking and testing a sample of the tumour. Using a biopsy, a doctor will look to see if the biopsy looks like a specific sarcoma subtype. If it doesn’t, then the doctor will look to see whether the cells are spindle-shaped. If they are, the doctor will diagnose the cancer as spindle cell sarcoma.
To read more about these scans and tests, click here.
- The first treatment method for spindle cell sarcoma 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
- When spindle cell sarcoma affects the arms and legs, 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”.
Targeted and experimental therapies
- In spindle cell sarcoma, researchers have been learning more about treatments known as “targeted therapies” and “immunotherapies”
- Targeted therapy: a treatment that targets specific characteristics within cancer cells in order to stop them from growing and spreading
- Immunotherapy: 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 spindle cell sarcoma. 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.
- There are trials currently recruiting patients with spindle cell sarcoma. For more information, please follow this link.
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 spindle cell sarcoma returning. This may include scans such as CT, PET or MRI scans
- A chest x-ray to rule out any secondary cancers occurring in the lungs
Spindle cell sarcoma 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 spindle cell sarcoma 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 spindle cell sarcoma tumour. In spindle cell sarcoma 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 lymph nodes and bones. Treatment for secondary cancer may involve surgery, radiotherapy or other therapy as appropriate; your treatment will be assessed on an individual basis.
If you or a loved one has been diagnosed with sarcoma, you may be feeling scared and uncertain about the future. However, it’s important to remember that there is hope, and that many people with sarcoma are able to live long, fulfilling lives after their diagnosis.
Survival depends on a number of different factors. Doctors usually use large sets of data to determine the outlook for a particular type of cancer. However, sarcoma is rare, which means survival is harder to estimate than for other, more common cancers.
Here are some survival statistics for spindle cell sarcoma:
- 75% of people diagnosed with spindle cell sarcoma in England survive their disease for one year or more (2013-2017).
- 50% of people diagnosed with spindle cell sarcoma in England survive their disease for five years or more (2013-2017).
It’s important to keep in mind that survival rates are just one factor to consider when it comes to your prognosis. Your individual prognosis will depend on many factors, including your age, overall health, and the stage and grade of your cancer.
About these statistics
The terms 1 year survival and 5 year survival don’t mean that you will only live for 1 or 5 years.
The Office for National Statistics (ONS) and researchers collect information. They watch what happens to people with cancer in the years after their diagnosis. 5 years is a common time point to measure survival. But some people live much longer than this.
5 year survival is the number of people who have not died from their cancer within 5 years after diagnosis.
Last reviewed: 2 February 2023
Next review due: 2 February 2026