About different types of soft tissue sarcoma

There are different types of soft tissue sarcoma. We describe the most common types in this section. Your doctor or specialist nurse can tell you more about which type you have.

A sarcoma is not named after the part of the body where it started. Instead, it is named after the type of cell it looks like.

Some sarcoma starts from very early, undeveloped cells called stem cells. As they grow, they start to look like a particular tissue.

Fat tissue tumours

Sarcoma that starts in fat cells is called liposarcoma. It can grow anywhere in the body. The most common places are the thigh and the area in the back of the tummy, called the retroperitoneum.

Liposarcoma is more common in people aged 50 to 65. Sometimes it grows very slowly, taking many years to develop. Or it may grow more quickly.

Muscle tissue tumours

There are 2 types of muscle tissue in the body, called smooth and skeletal muscle.

Smooth muscle sarcoma

Leiomyosarcoma (LMS) is one of the more common types of sarcoma. It develops in smooth muscle. Smooth muscle makes up the walls of internal organs, such as the womb (uterus), stomach, bowel and blood vessels. The muscle makes these organs contract (shorten) without our control.

This sarcoma can develop anywhere in the body. It is most common in the tummy, retroperitoneum and pelvic area. Sometimes it starts in the deep, soft tissues of the arms or legs.Leiomyosarcoma usually develops in people over 50.

Skeletal muscle sarcoma

Rhabdomyosarcoma (RMS) develops in skeletal muscles. These are the active muscles in our arms and legs, and other parts of the body.

Skeletal muscle sarcoma is most common in the head and neck. But it can also develop in organs such as the bladder, vagina or arms or legs. Rhabdomyosarcoma is most common in children.

Peripheral nerve tumours (nerve tissue tumours)

The body’s nervous system is divided into the following 2 parts:

  • The central nervous system, which is made up of the brain and spinal cord.
  • The peripheral nervous system, which is made up of the nerves that carry messages between the brain, the spinal cord and the rest of the body.

Sarcoma of the peripheral nerves starts in the cells that cover the nerves. It is called a malignant peripheral nerve sheath tumour (MPNST). 

It can develop anywhere in the body and there are different types. It is usually diagnosed in people aged 30 to 50 who have a rare genetic disorder called neurofibromatosis. This may also be called von Recklinghausen’s disease.

Fibrous tissue tumours

Fibrous tissues join the inner structures of the body. For example, they join muscles to bones. This tissue is made up of cells called fibrocytes. A sarcoma of the fibrous tissue is called a fibroblastic sarcoma. There are different types:

  • Undifferentiated pleomorphic sarcoma (UPS)

    In an undifferentiated pleomorphic sarcoma or UPS, it is not clear which type of cell the sarcoma started in. UPS used to be called a malignant fibrous histiocytoma (MFH). They usually develop in the legs and arms but can affect any part of the body. UPS is more common in people aged 60 to 70. This type of sarcoma is known as undifferentiated pleomorphic sarcoma (UPS). It used to be called a malignant fibrous histiocytoma (MFH). Undifferentiated pleomorphic sarcomas are more commonly in people aged 60 to 70.

  • Myxofibrosarcoma

    This is a common type of soft tissue sarcoma. It usually develops in the arms, legs and central part of the body (the trunk). It is more common in older people.

  • Fibrosarcoma

    This is a rare type of sarcoma which usually develops in the arms, legs, central part of the body (trunk) or head and neck. But it can also develop deeper in the body. It can happen at any age, but is more common in middle-aged and older people.

  • Dermatofibrosarcoma protuberans (DFSP)

    This is a rare, slow-growing tumour. It often starts in the skin. It is usually diagnosed in people aged 20 to 30. 

  • Fibromatosis

    This is sometimes called a desmoid tumour. It is a benign tumour. Benign means the tumour is not cancer. Desmoid tumours do not usually spread to other parts of the body. But they can spread locally into nearby tissues and may cause serious damage. Because of this, they may be treated in the same way as a cancerous sarcoma.

Blood and lymph vessel tumours (angiosarcoma)

Blood and lymph vessel tumours (angiosarcoma)

Sarcoma that starts from the cells in the walls of blood or lymph vessels is called angiosarcoma. There are 2 types:

  • haemangiosarcoma develops from blood vessels
  • lymphangiosarcoma develops from lymph vessels.

Angiosarcoma sometimes develops in a part of the body that has been treated with radiotherapy many years before.

Other types of sarcoma

Synovial sarcoma

This tumour can develop anywhere in the body. It most commonly affects the arms and legs. It is usually diagnosed in people aged 15 to 35 but can develop at any age, including childhood.

Gastrointestinal stromal tumour (GIST)

This is the most common type of soft tissue sarcoma to develop in the digestive system. The digestive system is often called the gastrointestinal (GI) tract. It is made up of the gullet, stomach and bowel. GISTs start in nerve cells that make the muscles of the digestive system tighten and relax. They behave differently from other types of sarcoma and are treated differently.

Kaposi’s sarcoma (KS)

Kaposi’s sarcoma develops differently from other sarcomas. It starts from cells in the skin. Coloured patches or lumps can develop in the skin or mouth. Lymph nodes or internal organs such as the lungs, liver or spleen are rarely affected. It is linked with human herpes virus (HHV8).

There are different types of KS. Certain types can affect people with a weakened immune system. This includes people with HIV and people who have had an organ transplant. Other types of KS can happen in parts of the world where human herpes virus (HHV8) is more common.

Kaposi’s sarcoma is treated differently from other types of soft tissue sarcoma.

Soft tissue Ewing sarcoma

Ewing sarcoma is a rare cancer that usually develops in the bones. Sometimes Ewing sarcoma starts in the soft tissues of the body. This is called soft tissue Ewing sarcoma. Ewing sarcoma is most common in teenagers.

Soft tissue Ewing sarcoma behaves differently to other types of soft tissue sarcoma. It is treated in the same way as a Ewing sarcoma that starts in the bone.

Rarer types of soft tissue sarcoma

There are other much rarer types of sarcoma that we have not explained here. These include:

  • inflammatory myofibroblastic tumours (IMT)
  • alveolar soft part sarcoma
  • epithelioid sarcoma
  • clear cell sarcoma
  • desmoplastic small round cell tumours
  • extraskeletal myxoid chondrosarcoma
  • solitary fibrous tumours.

It can be difficult to get information about the rarer types of sarcoma. Your doctor and specialist nurse can give you more information and answer any questions you have. You can also speak to the nurses on the Macmillan Support Line.

About our information

  • References

    Below is a sample of the sources used in our soft tissue sarcoma information. If you would like more information about the sources we use, please contact us at cancerinformationteam@macmillan.org.uk

    Gronchi A, Miah AB et al. Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical practice guidelines for diagnosis, treatment and follow-up. Annals of Oncology, 2021; 32, 11, 1348-1365 [accessed May 2022].

    Casali PG, Blay JY et al. Gastrointestinal stromal tumours: ESMO-EURACAN-GENTURIS Clinical practice guidelines for diagnosis, treatment and follow-up. Annals of Oncology, 2022; 33,1, 20-33 [accessed May 2022].

  • Reviewers

    This information has been written, revised and edited by Macmillan Cancer Support’s Cancer Information Development team. It has been reviewed by expert medical and health professionals and people living with cancer. It has been approved by senior medical editor Fiona Cowie, Consultant Clinical Oncologist.

    Our cancer information has been awarded the PIF TICK. Created by the Patient Information Forum, this quality mark shows we meet PIF’s 10 criteria for trustworthy health information.

The language we use

We want everyone affected by cancer to feel our information is written for them.

We want our information to be as clear as possible. To do this, we try to:

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Date reviewed

Reviewed: 01 August 2022
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Next review: 01 August 2025
Trusted Information Creator - Patient Information Forum
Trusted Information Creator - Patient Information Forum

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