MPNSTs, also known as neurofibrosarcomas, develop in the cells that cover nerves. They can occur anywhere throughout the body. MPNSTs occur mainly in adults.
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 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
Types of diagnostic scans
The earlier sarcoma is diagnosed the better the chances of successful treatment. Sarcomas are usually found by a patient when a lump appears on the leg, arm or trunk. They can also be found during an investigation of other symptoms or during a routine operation.
A specialist doctor will diagnose 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 ultrasound, x-ray, CT, EUS, PET or MRI
- A biopsy – taking and testing a tissue sample
Types of diagnostic scans and tests
Uses x-radiation to take images of dense tissues inside the body such as bones or tumours.
A scan that uses sound waves to create images from within the body.
The Computer Tomography (CT) scan takes a number of x-rays to make a 3D image of an affected area.
The Endoscopic Ultrasound Scan (EUS) uses a tube-like instrument called an endoscope with an ultrasound scanner attached. This is put inside the body to look inside the gut to investigate GIST tumours.
The Positron Emission Tomography (PET) scan shows up changes in tissues that use glucose as their main source of energy – for example the brain or heart muscle. It involves an injection of a very small amount of a radioactive drug into the body. The drug travels to places where glucose is used for energy and shows up cancers because they use glucose in a different way from normal tissue.
Magnetic Resonance Imaging (MRI) uses magnets to create an image of the tissues of the body.
Examination of a tissue sample by a pathologist under a microscope to identify disease.
Laboratory analysis of a blood sample.
Understanding the diagnosis
Identifying the stage and grade of a cancer means your doctor can advise on the best course of treatment for you. It also describes the cancer in a common language which is useful when your doctor is discussing your case with other doctors or healthcare professionals.
The stage of cancer is measured by how much it has grown or spread which can be seen on the results of your tests and scans. The results from a biopsy can tell what grade the cancer is.
- Low-grade means the cancer cells are slow-growing, look quite similar to normal cells, are less aggressive, and are less likely to spread
- Intermediate-grade means the cancer cells are growing slightly faster and look more abnormal
- High-grade means the cancer cells are fast growing, look very abnormal, are more aggressive and are more likely to spread
- Stage 1 means the cancer is low grade, small (less than 5cm) and has not spread to other parts of the body
- Stage 2 means the cancer is of any grade, usually larger than stage one but has not spread to other parts of the body
- Stage 3 means a high grade cancer that has not spread to other parts of the body
- Stage 4 means a cancer of any grade or size that has spread to any other part of the body
The National Institute for Health and Care Excellence (NICE) recommends that anyone with sarcoma should be referred to a specialist sarcoma team for diagnosis and treatment.
Your case will be managed by a team of experts from a wide range of health care professions called a multidisciplinary team (MDT). Your MDT will include your key worker or sarcoma clinical nurse specialist, surgeon and other healthcare professionals involved in your care. They will support you throughout your treatment to ensure you get the right treatment as and when you need it.
Types of treatment
The type of treatment you receive will depend on the stage and grade of your cancer. Your MDT will discuss your case and your doctor will talk you through your options so you are included in deciding what treatment is best for you.
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.
Emotional and practical support
TALKING ABOUT IT
There is no right or wrong way to feel or react to diagnosis; you may feel angry, sad or anxious about the future. You may also have concerns for how the news will affect your loved ones. You may find comfort in talking about your concerns with family members or friends.
TALKING TO OTHERS AFFECTED BY SARCOMA
If you do not feel comfortable discussing your concerns with family or friends, it may help to talk to other people who have been affected by sarcoma. There are a number of sarcoma support groups supported by Sarcoma UK around the country. These offer valuable support and information to patients, carers and family members, and provide the opportunity to meet with other people in the same situation.
Most groups are run by patients and carers working together with local sarcoma clinical nurse specialists or doctors. A typical meeting may include a talk from an invited speaker on a related topic, discussion and questions, informal chat with other group members, and refreshments.
There is also online support available for people affected by all types of sarcoma. Online support provides members with the opportunity to get in touch with other sarcoma patients or carers to discuss their concerns over a new diagnosis, treatment options or worries about the future.
TALKING TO A PROFESSIONAL
You may find it helpful to talk to your sarcoma clinical nurse specialist or doctor about your diagnosis; they will be able to answer any questions you may have about your condition. They may also be able to put you in touch with a counsellor for additional support, providing you with a safe, confidential place to talk about your concerns. Your GP will have access to local counselling services who can provide support to people with cancer.
Patients who are being treated for cancer can apply for a medical exemption (MedEx) certificate which will allow you to get free prescriptions. You can get an application form from your doctor and it will need to be countersigned by your GP or consultant. Prescriptions are free for all patients in Scotland, Wales and Northern Ireland.
Your sarcoma clinical nurse specialist should be able to advise you on the types of benefits you can claim or any special funding you can apply for. Your local Citizens Advice Bureau can also give you benefits information and many branches can help you fill out application forms. Macmillan Cancer Support have a number of benefit advisors who offer financial advice and support to cancer patients including advice about returning to work following cancer treatment.
Date published: August 2015
Next review: August 2017