MPNSTs, also known as neurofibrosarcomas, are a type of soft tissue sarcoma that develops in the cells that cover nerves.
They can occur anywhere throughout the body.
There are an average of 72 cases of MNPST diagnosed every year in England.
MPNST makes up around 1.83% of all soft tissue sarcomas, and about 0.02% of all cancers.
Although MPNST can affect anyone of any age, the median age at diagnosis is 49 years old.
For more information on these statistics, you can visit our soft tissue sarcoma data hub.
Signs and symptoms
Symptoms of 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 a sarcoma:
A lump or swelling in the soft tissue of the body under the skin, often on the arm, leg or trunk
- increasing in size
- is larger than 5cm
- usually painful, but not always
- Physical examination – looking at and feeling any lump
- A scan – taking pictures of the inside of the body using ultrasound, x-ray, CT, EUS, PET or MRI
- A biopsy – taking and testing a tissue sample
To read more about scans and tests, click here.
In a lot of cases, surgery is the first treatment method used for sarcoma – sometimes with additional radiotherapy or chemotherapy. The surgeon will remove the tumour and will aim to take out an area of normal tissue around it too; this is known as taking a margin. It allows any cancer cells that are not visible to the naked eye to be removed along with the tumour which can reduce the risk of the cancer coming back.
This treatment uses high-energy radiation beams to destroy cancer cells. It is used either before or after surgery. When used before surgery it aims to reduce the size of the tumour so it can be operated on and removed. Radiotherapy is also very effective when given after surgery. This is particularly so for intermediate and high grade tumours and when the margins are quite close. In this case, the aim is to kill off any local cancer cells which remain in the area of the tumour. Your doctor will advise which is best for you.
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.
You may be offered an opportunity to take part in a study 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.
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 usual practice will include:
- A chance to discuss symptoms
- An examination to look for any signs of the sarcoma returning. This may include an MRI or ultrasound if required after examination
- A chest x-ray to rule out any secondary cancers occurring in the lungs
Sarcoma cancer 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 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 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 sarcoma tumour. In 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 liver or brain. Treatment for secondary cancer may involve surgery, radiotherapy or chemotherapy as appropriate; your treatment will be assessed on an individual basis.