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Soft Tissue Sarcoma

Soft tissue sarcoma refers to cancer which develops in soft tissue or connective tissue. These are the tissues that connect and support all organs and structures of the body.

Last modified: January 24, 2022

Quick facts about soft tissue sarcomaQuick facts

  • Soft tissue sarcoma can occur anywhere in the body from soft tissues such as muscle, fat, cartilage, nerves, tendons, lining of joints, lymph vessels or blood vessels. It is most commonly found in the thigh, shoulder, retroperitoneum (back of abdomen) and pelvis
  • There are more than 70 types of soft tissue sarcoma
  • Soft tissue sarcoma is a rare type of cancer. It was estimated that in 2021, Australians had a 1 in 149 (0.67%) chance of being diagnosed with sarcoma by the age of 85
  • Soft tissue sarcoma accounted for 1.3% of all Australian cancer diagnoses in 2021 

Types of soft tissue sarcomaTypes of soft tissue sarcoma

There are more than 50 types of soft tissue sarcomas.

Common types include:

Undifferentiated pleomorphic sarcoma (UPS) Leiomyosarcoma Liposarcoma Fibrosarcoma Rhabdomyosarcoma Synovial sarcoma Gastrointestinal stromal tumour (GIST) Malignant peripheral nerve sheath tumour (MPNST)

Undifferentiated pleomorphic sarcoma (UPS)

Previously referred to as malignant fibrous histiocytoma, UPS is an aggressive type of soft tissue sarcoma, usually occurring in the arms, legs or abdomen.

Leiomyosarcoma

This is one of the most common types of soft tissue sarcoma and is a smooth muscle tumour. They are most commonly found in the uterus in women but can also occur in bladder or stomach.

Liposarcoma

Found in fatty tissue, liposarcomas can develop anywhere in the body but are most commonly found in the retroperitoneum and the thighs. There are many subtypes of liposarcomas including well differentiated, dedifferentiated, myxoid and pleomorphic.

Fibrosarcoma

A type of tumour that grows in fibrous tissue, most commonly in extremities, chest, abdomen, head or neck.

Rhabdomyosarcoma

This type of soft tissue sarcoma is found in the skeletal muscle cells and can develop anywhere in the body. This is the most common soft tissue sarcoma in children. 

Synovial sarcoma

Synovial sarcomas refer to soft tissue sarcomas with unknown origins. They are most commonly detected in knee or ankle joints, but may also be found in other joints throughout the body including the hips or shoulders.

Gastrointestinal stromal tumour (GIST)

A common type of soft tissue sarcoma that originates from the digestive tract, commonly in the stomach. 

Malignant peripheral nerve sheath tumour (MPNST)

A soft tissue tumour that originates from the lining of the nerve cells. This can occur anywhere in the body but commonly in the arms, legs and trunk. 

Signs and symptoms of soft tissue sarcoma Signs and symptoms

As signs and symptoms for soft tissue sarcoma may be similar to other conditions in some cases, it’s important to see your GP or healthcare professional if you experience any of the symptoms below. Discussing anything concerning with your doctor as soon as possible can help give you peace of mind and offer the best chance of successful treatment if you receive a soft tissue sarcoma diagnosis.

Soft tissue sarcoma can be difficult to detect as they may not cause any symptoms at early stages. The type of symptoms you experience will also depend on which part of the body the sarcoma develops in.

These may include:  

  • Growing lump under the skin, which may be painless or painful if it is pressing on surrounding muscles or nerves

  • Trouble breathing

  • Pain

Stages of soft tissue sarcoma Stages

The TNM system is used to stage soft tissue sarcoma, and it helps doctors understand what your cancer looks like. The TNM stands for:

  • Tumour (T) – Describes the size of the tumour and the degree to which it has affected surrounding tissue. The tumour can be graded as T1 (describing a tumour 5cm or less), T2 (greater than 5cm but less than 10 cm), T3 (greater than 10 cm but less than 15 cm) or T4 (greater than 15cm)
  • Nodes (N) – Describes whether the tumour has spread to nearby lymph nodes. Nodes can be graded from N0, where there has been no spread, through to N1, where the cancer has spread the lymph nodes
  • Metastasis (M) – Describes whether the cancer has spread to other areas of the body. Metastasis can be graded from M0, where there has been no spread through to M1, where the cancer has spread to other organs of the body and produced additional tumours

Soft tissue sarcomas are also given a grade (G) from G1 to G3. Soft tissue sarcomas with a higher grade are more aggressive and have a higher likelihood of growing and spreading compared to those with a lower grade.

The TNM information, along with the grade, helps determine the stage of your soft tissue sarcoma using the guidelines below. 

Staging of soft tissue sarcoma in the arms, legs and trunk

Stage IA soft tissue sarcoma in the arms, legs and trunk Stage IB soft tissue sarcoma in the arms, legs and trunk Stage II soft tissue sarcoma in the arms, legs and trunk Stage IIIA soft tissue sarcoma in the arms, legs and trunk Stage IIIB soft tissue sarcoma in the arms, legs and trunk Stage IV soft tissue sarcoma in the arms, legs and trunk

Stage IA

The tumour is 5cm or smaller and is low grade. 

Stage IB

The tumour has grown larger than 5cm and is low grade.

Stage II

The tumour is 5cm or smaller and is mid or high grade.

Stage IIIA

The tumour is between 5cm to 10cm and is mid or high grade.

Stage IIIB

The tumour is larger than 10cm and is mid or high grade.

Stage IV

The tumour may be any size or grade that has spread to the nodes and/or other parts of the body.  

Staging of soft tissue sarcoma in the retroperitoneum

Stage IA soft tissue sarcoma in the retroperitoneum Stage IB soft tissue sarcoma in the retroperitoneum Stage II soft tissue sarcoma in the retroperitoneum Stage IIIA soft tissue sarcoma in the retroperitoneum Stage IIIB soft tissue sarcoma in the retroperitoneum Stage IV soft tissue sarcoma in the retroperitoneum

Stage IA

The tumour is 5 cm or smaller and is low grade.

Stage IB

The tumour has grown larger than 5cm and is low grade.

Stage II

The tumour is 5 cm or smaller and is mid or high grade.

Stage IIIA

The tumour is between 5cm to 10cm and is mid or high grade.

Stage IIIB

The tumour is larger than 10cm and is mid or high grade and may or may not have spread to the nearby lymph nodes.

Stage IV

The tumour may be any size and may or may not have spread to the lymph nodes, however it has spread to other parts of the body.

Treatment for soft tissue sarcoma

There are many different types of treatment for soft tissue sarcoma. Your treatment will depend on you and your cancer.

Learn more

Frequently asked questions about soft tissue sarcoma FAQs

Is soft tissue sarcoma hereditary?

Soft tissue sarcoma can be hereditary, which means the risk of developing soft tissue sarcoma may be inherited from your parents. You may also inherit other genetic syndromes that can increase your likelihood of soft tissue sarcoma, such as:

  • Retinoblastoma
  • Li-Fraumeni syndrome
  • Familial adenomatous polyposis
  • Neurofibromatosis
  • Tuberous sclerosis
  • Werner syndrome
What causes soft tissue sarcoma?

It’s not known what causes soft tissue sarcoma, however there are some factors which are thought to increase your risk of developing it including previous radiation therapy, genetics, chemical exposure and your past medical history.

What are the risk factors for soft tissue sarcoma?

Risk factors that have been associated with developing soft tissue sarcoma include:

  • Gender – There is a slightly higher rate of soft tissue sarcoma detected in men compared to women
  • Exposure – You have an increased risk of developing soft tissue sarcoma if you are exposed to certain chemicals (including vinyl chloride, dioxin and herbicides) or radiation. People who have been treated with radiation therapy for a previous cancer have a slightly higher risk of developing sarcoma. The sarcoma often starts in the part of the body that was treated with radiation, with an average time of about 10 years between the radiation therapy and the diagnosis of a sarcoma
  • Genetics – People with familial history of soft tissue sarcoma or certain inherited syndromes have a higher risk of sarcoma
  • Lymphoedema – Soft tissue sarcomas can sometimes form in the area where lymphoedema has developed

 

How common is soft tissue sarcoma?

Soft tissue sarcoma is a rare type of cancer. In 2021, it was estimated that there would be around 2,100 new cases of soft tissue sarcoma in Australia, accounting for less than 1.5% off all new cancer diagnoses nationally.

How is soft tissue sarcoma diagnosed?

If your doctor suspects you may have soft tissue sarcoma based on your medical history and any symptoms, they will refer you to a specialist to confirm your diagnosis. There are many different tests that are used by specialists to diagnose soft tissue sarcoma. This may include blood tests, x-ray, CT scan, MRI, PET scan and biopsy which can help to identify the type of cancer you have, its grade and how far it has spread.

Are there clinical trials available for soft tissue sarcoma that I can participate in?

Icon offers clinical trials across a wide range of cancer types and treatments. If you would like more information on participating in a clinical trial, please speak with your doctor who will be able to find a trial that might be right for you and your cancer. 

See current clinical trials delivered by Icon Cancer Centre.

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ReferencesReferences

For a full list of references, click here.
  1. Cancer Research UK. (2021, April 28). Types of soft tissue sarcomas. Cancer Research UK. Retrieved January 10, 2022, from http://www.cancerresearchuk.org/about-cancer/soft-tissue-sarcoma/types
  2. Cancer Council. (2021, February). Soft tissue sarcoma. Cancer Council. Retrieved January 10, 2022, from http://www.cancer.org.au/cancer-information/types-of-cancer/rare-cancers/soft-tissue-sarcoma 
  3. Cancer Australia. (2019, December 18). Soft tissue sarcoma statistics in Australia. Cancer Australia. Retrieved January 10, 2022, from http://www.canceraustralia.gov.au/cancer-types/sarcoma/statistics 
  4. Mayo Foundation for Medical Education and Research. (2020, December 2). Soft tissue sarcoma. Mayo Clinic. Retrieved January 10, 2022, from http://www.mayoclinic.org/diseases-conditions/soft-tissue-sarcoma/symptoms-causes/syc-20377725   
  5. American Cancer Society. (2018, April 8). What is a soft tissue sarcoma? American Cancer Society. Retrieved January 10, 2022, from http://www.cancer.org/cancer/soft-tissue-sarcoma/about/soft-tissue-sarcoma.html 
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