What is soft tissue sarcoma?
Soft tissue sarcoma is a type of cancer that forms in the soft tissues of the body.
Cancer starts when cells begin to grow out of control. Cells in nearly any part of the body can become cancer and spread to other areas.
Soft tissue is the name for all the supporting tissues in the body, apart from the bones. They include fat, muscle, nerves, tendons, deep skin tissue, lymph vessels, blood vessels and tissue around joints.
These tissues support and connect all the organs and structures of the body. They are known as connective tissue. A soft tissue sarcoma is a rare type of cancer that generally forms as a painless lump (tumour) in any one of these soft tissues. It can develop anywhere in the body, but most commonly in the thigh, shoulder, arm, pelvis and abdomen.
Malignant (cancerous) tumours have the potential to spread to other parts of the body through the blood stream or lymph vessels and form another tumour at a new site. This new tumour is known as secondary cancer or metastasis.
Types of soft tissue sarcoma
There are more than 70 types of soft tissue sarcoma. They are named after the abnormal cells that make up the sarcoma, that is the type of cell where the cancer first started to grow.
The most common type in adults is undifferentiated pleomorphic sarcoma (UPS), which is made up of many abnormal spindle-shaped cells. It is aggressive so it often returns or spreads after treatment.
Other types of soft tissue sarcoma in adults
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Liposarcoma |
from fat cells |
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Angiosarcoma |
from blood and lymph vessels |
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Malignant peripheral nerve sheath tumour (MPNST or PNST) |
from the lining of nerve cells |
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Gastro-intestinal stromal sarcoma (GIST) |
from nerve cells in the gut (treated differently from other types of soft tissue sarcoma) |
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Stromal sarcoma |
from supporting tissues |
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Kaposi sarcoma |
from skin cells |
Other types of soft tissue sarcoma in children and young adults:
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Rhabdomyosarcoma |
from muscle cells |
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Ewing sarcoma |
(may also start in the bone) |
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Synovial sarcoma |
from cells around joints and tendons |
More information on the different types of soft tissue sarcoma is available from the Australia and New Zealand Sarcoma Association (ANZSA) and Rare Cancers Australia.
How common is soft tissue sarcoma?
Soft tissue sarcoma is rare. Around 1600 Australians are diagnosed with a soft tissue sarcoma each year (about 6 cases per 100,000 people). It is slightly more likely to be diagnosed in men than women. While soft tissue sarcoma can develop at any age, it is more likely to be found in people over 55 years of age.
What are the symptoms and the risk factors?
Soft tissue sarcoma usually doesn’t cause symptoms in its early stages.
As it grows over a period of months, you may develop a painless lump. You may begin to have pain as the lump grows and presses on nerves and muscles. Other symptoms will depend on where in the body the sarcoma is. Sometimes a soft tissue sarcoma may be mistaken for a benign fatty lump (lipoma) or bruise (haematoma). This can delay tests that would help make the right diagnosis.
One rare type of skin cancer, dermatofibrosarcoma protuberans (DFSP), can show as a flat, slightly raised or even depressed area of skin that may be violet, reddish brown or skin-coloured making it particularly hard to diagnose. While the original tumour is not technically a sarcoma, a small number of people with DFSP have ‘fibrosarcomatous transformation’ where the tumour may progress and show areas of fibrosarcoma, a type of soft tissue sarcoma from supportive tissue. Visit DermNet NZ for more information.
Most people who develop a painless lump do not have a sarcoma. However, you should see your doctor if you notice the lump is getting bigger, is the size of a golf ball or larger, or is painful or tender.
What are the risk factors?
The causes of most sarcomas are not known. However, there are several risk factors:
Radiation therapy – There is a very small risk for people who have had radiation therapy (also known as radiotherapy) to treat other types of cancer. The risk is higher for people who had high doses of radiation therapy at a very young age. Most people who have had radiation therapy in the past will not develop soft tissue sarcoma.
Genetic factors – Some rare, inherited conditions can put people more at risk of soft tissue sarcoma. These rare, genetic conditions include von Recklinghausen disease (also known as neurofibromatosis or NF), Li–Fraumeni syndrome (also known as p53) and retinoblastoma (also known as Rb). Most people know if one of these very rare conditions runs in their family, and if so, that they may pass it to their children. If you do not know of this condition in your family, then it is very unlikely that it is present. A small number of people develop sarcoma due to genetic changes that happen during their lifetime, rather than inheriting a faulty gene.
Chemicals – Some sarcomas may be linked to being exposed to chemicals including vinyl chloride (used to make plastic) and some high-dose herbicides (weedkillers).
Other – Long-term lymphoedema in the body, for example in the legs or arms (swelling from a build-up of lymph fluid) has been linked with angiosarcoma.
How is a soft tissue sarcoma diagnosed?
If your doctor thinks that you may have a soft tissue sarcoma, they will take your medical history, perform a physical examination (including feeling any lumps) and carry out certain tests. These tests are needed to rule out other conditions.
If the results suggest that you may have sarcoma, your doctor will refer you to a specialist who will carry out more tests. These may include:
Blood tests
Blood tests including a full blood count to measure your white blood cells, red blood cells, platelets.
X-rays
A painless scan called an x-ray may be taken of your chest to check your lungs for signs of cancer.
CT (computerised tomography) or MRI (magnetic resonance imaging) scans
Special machines are used to scan and create pictures of the inside of your body. Before the scan you may have an injection of dye (called contrast) into one of your veins, which makes the pictures clearer. During the scan, you will need to lie still on an examination table.
For a CT scan the table moves in and out of the scanner which is large and round like a doughnut; the scan itself takes about 10 minutes.
For an MRI scan the table slides into a large metal tube that is open at both ends; the scan takes a little longer, about 30–90 minutes to perform. Both scans are painless.
PET (positron emission tomography) scan
Before the scan you will be injected with a small amount of radioactive glucose (sugar) solution. Many cancer cells will show up brighter on the scan. You will be asked to sit quietly for 30–90 minutes to allow the glucose to move around your body, and the scan itself will take around 30 minutes to perform.
Biopsy
A biopsy is the removal of some tissue from the affected area for examination under a microscope. The biopsy may be done in one of two ways. In a core needle biopsy, a local anaesthetic is used to numb the area, then a thin needle is inserted into the tumour under ultrasound or CT guidance. An open or surgical biopsy is done under general anaesthesia. The surgeon will cut through the skin to expose the affected area and take a tissue sample.
A biopsy is the only sure way to diagnose a soft tissue sarcoma and should only be carried out by a specialist who does them regularly and has special expertise in the area of sarcoma. It is important that the biopsy is done properly, to reduce the chance of the cancer spreading. It could also affect the type of treatment that you may be able to have.
Finding a specialist
Visit The Australia and New Zealand Sarcoma Association (ANZSA) for a directory of specialists in sarcoma care and treatment.
Rare Cancers Australia also have a directory of health professionals and cancer services across Australia.
Treatment for soft tissue sarcoma
You will be cared for by a multi-disciplinary team (MDT) of health professionals during your treatment for soft tissue sarcoma.
The team may include a surgeon, radiation oncologist (to prescribe and coordinate a course of radiation therapy), medical oncologist (to prescribe and coordinate a course of systemic therapy which includes chemotherapy), paediatric oncologist, nurse and allied health professionals such as a dietitian, social worker, psychologist or counsellor, physiotherapist and occupational therapist.
Discussion with your doctor will help you decide on the best treatment for your sarcoma depending on:
- the type of sarcoma you have
- where it is in your body
- whether or not the cancer has spread (stage of disease)
- your age, fitness and general health
- your preferences.
The main treatments include surgery, chemotherapy and radiation therapy. These can be given alone or in combination. This is called multi-modality treatment.
Surgery
Surgery is the main treatment for most types of soft tissue sarcoma. Surgery usually involves removing the cancer and some healthy tissue around the cancer. This is called a wide local excision. The healthy tissue is removed to help reduce the risk of the cancer coming back in that area.
The type of operation depends on where the sarcoma is. Most sarcomas are in the arm or leg. This usually means having limb-sparing surgery; amputation is less common. If the sarcoma is in another part of the body, for example the chest or abdomen, surgery will depend on where the cancer is and how big it is. Your surgeon will discuss the type of operation you may need.
The most important thing is that you have your surgery done by an experienced surgeon working in a specialist centre for sarcoma.
Support programs
If you need major surgery like amputation, you may find it helps to speak with a social worker or counsellor. You might also find it beneficial to talk to others who have been through a similar experience. A range of support programs are offered by Cancer Council including telephone, online, and peer to peer support groups. Call 13 11 20 and speak to a cancer health professional about what might suit you best.
Radiation therapy
Radiation therapy (also known as radiotherapy) uses high energy rays to destroy cancer cells. Sarcoma is known to be very sensitive to radiation therapy.
It may be used:
- before surgery, to destroy the cancer cells and create a ‘rind’ around the cancer, which helps the surgeon to perform good surgery
- if the cancer is too big to remove with surgery
- if the cancer has spread to other parts of the body
- after surgery, to destroy any remaining cancer cells and stop the cancer coming back
- if the cancer is in a place in the body that is too hard to reach using surgery.
Radiation therapy can shrink the cancer down to a smaller size. This may help to make surgery easier. Sometimes chemotherapy is given as well as radiation therapy, to help make the treatment more effective. This depends on the type of sarcoma.
A course of radiation therapy needs to be carefully planned. During your first consultation session you will meet with a radiation oncologist who will arrange a planning session. At the planning session (known as CT planning or simulation) you will need to lie still on an examination table and have a CT scan in the same position you will be placed in for treatment.
The information from the planning session will be used by your specialist to work out the treatment area and how to deliver the right dose of radiation. Radiation therapists will then deliver the course of radiation therapy as set out in the treatment plan.
Radiation therapy does not hurt and is usually given in small doses over a period of time to minimise side effects.
Chemotherapy
Chemotherapy (sometimes just called “chemo”) is the use of drugs to kill or slow the growth of cancer cells. You may have one chemotherapy drug, or a combination of drugs. This is because different drugs can destroy or shrink cancer cells in different ways.
Your treatment will depend on your situation and the type of sarcoma you have. It may also be used to help stop a sarcoma coming back after surgery. Your medical oncologist will discuss options with you.
Most often chemotherapy is given through a drip into a vein (intravenously) or as a tablet that is swallowed. It can also be given as a cream or as injections into different parts of the body. Having a needle inserted for intravenous chemotherapy feels like having blood taken. If you have a temporary tube (cannula) in your hand or arm only the initial injection may be uncomfortable. Your medical oncologist may recommend a central venous access device (also known as a ‘central line’). This is put into a vein in your neck or chest under local or general anaesthetic and stays in throughout your treatment so you don’t have to have lots of needles. You can also have your blood taken from this line.
Chemotherapy is commonly given in treatment cycles which may be daily, weekly or monthly. For example, one cycle may last three weeks where you have the drug over a few hours, followed by a rest period before starting another cycle. The length of the cycle and number of cycles depends on the chemotherapy drugs being given.
Targeted therapy
Targeted therapy is a cancer treatment that uses drugs to target specific genes and proteins that are involved in the growth and survival of cancer cells. It uses drugs that work in a different way to chemotherapy drugs. While chemotherapy affects all rapidly dividing cells and works by killing cancerous cells (cytotoxic), targeted therapy attacks specific molecules within cells and often works by blocking cell growth (cytostatic). People with soft tissue sarcomas may be offered targeted therapies such as pazopanib (Votrient) to slow the growth of the tumour.
Clinical trials
Your doctor or nurse may suggest you take part in a clinical trial. Doctors run clinical trials to test new or modified treatments and ways of diagnosing disease to see if they are better than current methods. For example, if you join a randomised trial for a new treatment, you will be chosen at random to receive either the best existing treatment or the modified new treatment. Over the years, trials have improved treatments and led to better outcomes for people diagnosed with cancer.
You may find it helpful to talk to your specialist, clinical trials nurse or GP, or to get a second opinion. If you decide to take part in a clinical trial, you can withdraw at any time.
For more information, visit Australian Cancer Trials or the Australia and New Zealand Sarcoma Association (ANZSA).
For information on gastro-intestinal stromal sarcoma (GIST) clinical trials contact the Australasian Gastro-Intestinal Trials Group (AGITG).
Download our booklet ‘Understanding Clinical Trials and Research’
Involvement in research
You may wish to be involved in research in addition to clinical trials, or perhaps because you and your doctor can’t find a clinical trial suitable for your needs. Some research studies enable your cancer to be studied in more detail in the laboratory. These studies will require samples of your cancer tissue and other tissues, such as your blood, and your clinical data to be collected. You would need to read a patient information and consent form to decide whether you wish to take part in this kind of research prior to providing your consent.
Information about these types of research studies may be available at your treatment centre or from websites such as the Australian New Zealand Clinical Trials Registry which lists appropriate studies. For more information, visit:
Complementary therapies and integrative oncology
Complementary therapies are designed to be used alongside conventional medical treatments (such as surgery, radiation therapy and chemotherapy) and can increase your sense of control, decrease stress and anxiety, and improve your mood. Some Australian cancer centres have developed “integrative oncology” services where evidence-based complementary therapies are combined with conventional treatments to improve both wellbeing and clinical outcomes.
Some complementary therapies and their clinically proven benefits are listed below:
acupuncture – reduces chemotherapy-induced nausea and vomiting; improves quality of life
aromatherapy – improves sleep and quality of life
art therapy, music therapy – reduce anxiety and stress; manage fatigue; aid expression of feelings
counselling, support groups – help reduce distress, anxiety and depression; improve quality of life
hypnotherapy – reduces pain, anxiety, nausea and vomiting
massage – improves quality of life; reduces anxiety, depression, pain and nausea
meditation, relaxation, mindfulness – reduce stress and anxiety; improve coping and quality of life
qi gong – reduces anxiety and fatigue; improves quality of life
spiritual practices – help reduce stress; instil peace; improve ability to manage challenges
tai chi – reduces anxiety and stress; improves strength, flexibility and quality of life
yoga – reduces anxiety and stress; improves general wellbeing and quality of life.
Let your doctor know about any therapies you are using or thinking about trying, as some may not be safe or evidence-based.
Download our booklet ‘Understanding Complementary Therapies’
Alternative therapies are therapies used instead of conventional medical treatments. These are unlikely to be scientifically tested and may prevent successful treatment of the cancer. Cancer Council does not recommend the use of alternative therapies as a cancer treatment.
Side effects of treatment
All treatments can have side effects. The type of side effects that you may have will depend on the type of treatment and where in your body the cancer is. Some people have very few side effects and others have more. Your specialist team will discuss all possible side effects, both short and long-term (including those that have a late effect and may not start immediately), with you before your treatment begins. One issue that is important to discuss before you undergo treatment is fertility, particularly if you want to have children in the future.
Common side effects may include:
Surgery – Nausea and vomiting from the anaesthetic, bleeding, damage to nearby tissue and organs (including nerves), drug reactions, pain, infection after surgery, blood clots, weak muscles (atrophy), lymphoedema.
Radiation therapy – Fatigue, loss of appetite, nausea, bowel issues such as diarrhoea, abdominal cramps and excess wind, bladder issues, hair loss, dry mouth, skin problems, lymphoedema, loss of fertility, early menopause.
Chemotherapy – Fatigue, loss of appetite, nausea, bowel issues such as constipation or diarrhoea, hair loss, mouth sores, skin and nail problems, increased chance of infections, loss of fertility, early menopause.
Nutrition and exercise
If you have been diagnosed with soft tissue sarcoma, both the cancer and treatment will place extra demands on your body. Research suggests that eating well and exercising can greatly benefit people during and after cancer treatment.
Eating well and being active can help you cope with some of the common side effects of cancer treatment, speed up recovery, improve sleep and help improve quality of life by giving you more energy, keeping your muscles strong, helping you maintain a healthy body weight and boosting your mood.
You can discuss individual nutrition and exercise plans with health professionals such as dietitians, exercise physiologists and physiotherapists.
Download our booklet ‘Nutrition for People Living with Cancer’
Download our booklet ‘Exercise for People Living with Cancer’
Life after treatment
Once your treatment has finished, you will have regular check-ups to confirm that the cancer hasn’t come back.
Ongoing surveillance for sarcoma involves a schedule of ongoing scans and physical examinations. Let your doctor know immediately of any health problems between visits.
Some cancer centres work with patients to develop a “survivorship care plan” which includes a summary of your treatment, sets out a clear schedule for follow-up care, lists any symptoms to watch out for, possible long-term side effects, identifies any medical or emotional problems that may develop and suggests ways to adopt a healthy lifestyle. Maintaining a healthy body weight, eating well and being physically active are all important.
If you don’t have a care plan, ask your specialist for a written summary of your cancer and treatment and make sure a copy is given to your GP and other health care providers.
What if the cancer returns?
For some people soft tissue sarcoma does come back after treatment, which is known as a recurrence. This is most likely to happen within the first five years after treatment. If the cancer does come back, treatment will depend on where the cancer has returned in your body and may include a mix of surgery, chemotherapy and radiation therapy.
In some cases of advanced cancer, treatment will focus on managing any symptoms, such as pain, and improving your quality of life without trying to cure the disease. This is called palliative treatment. Palliative care can be provided in the home, in a hospital, in a palliative care unit or hospice.
When cancer is no longer responding to treatment, it can be difficult to think about how you want to be cared for towards the end of life. But it’s essential to talk about what you want with family and health professionals, so they know what is important to you.
Dealing with feelings of sadness
If you have continued feelings of sadness, have trouble getting up in the morning or have lost motivation to do things that previously gave you pleasure, you may be experiencing depression. This is quite common among people who have had cancer.
Talk to your GP, as counselling or medication—even for a short time—may help. Some people are able to get a Medicare rebate for sessions with a psychologist. Ask your doctor if you are eligible. Cancer Council SA operates a free cancer counselling program. Call Cancer Council 13 11 20 for more information.
For information about coping with depression and anxiety, visit Beyond Blue or call them on 1300 22 4636. For 24-hour crisis support, visit Lifeline or call 13 11 14.
