About soft tissue sarcomas

About soft tissue sarcomas  1 About soft tissue sarcomas This information is from the booklet Understanding soft tissue sarcomas. You may find the fu...
Author: Dana Blair
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About soft tissue sarcomas  1

About soft tissue sarcomas This information is from the booklet Understanding soft tissue sarcomas. You may find the full booklet helpful. We can send you a free copy – see page 6.

Contents • What are soft tissue sarcomas? • Types of soft tissue sarcoma • Soft tissue sarcomas in children • More information and support

What are soft tissue sarcomas? Sarcomas are rare. About 3,300 people a year in the UK are diagnosed with a soft tissue sarcoma. Soft tissue sarcomas are cancers that develop from cells in the soft, connective tissues of the body. They can occur in soft tissues such as fat, muscle, nerves, fibrous tissues, blood vessels or in any of the other tissues that support, surround and protect the organs of the body. Soft tissue sarcomas can develop in any part of the body. Most of them develop in the arms and legs, but they can also develop in specific organs such as the womb (uterus), stomach, skin and small bowel. Less commonly, they may occur in the head and neck. Some types of sarcoma occur in children, teenagers and young adults, but generally they are more likely to develop in people over the age of 30.

Bone sarcomas Some sarcomas, such as osteosarcoma, start in the bone. These grow and develop differently and are treated differently from soft tissue sarcomas. Occasionally, it is hard to tell whether a sarcoma has started in soft tissue or bone. There are some types of sarcoma, such as Ewing’s tumours, that can begin in either the bone or soft tissue.

We have separate information about cancer that starts in the bone.

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Types of soft tissue sarcoma There are many types of soft tissue sarcoma and they tend to grow and develop differently. The most common types are described in this section. Your doctor or specialist nurse can tell you more about which type you have. Each type of sarcoma is named after the type of cell it started from, rather than the part of the body where it started to grow.

Fat tissue tumours Sarcomas that start in the body’s fat cells are called liposarcomas. They can grow anywhere in the body and most commonly affect people aged 50–65. Some grow very slowly, taking many years to develop, whereas others grow more quickly.

Muscle tissue tumours There are two different types of muscle tissue in the body: smooth and skeletal.

Smooth muscle sarcomas

Smooth muscle forms the walls of internal organs such as the womb (uterus), stomach, intestine and blood vessels. The muscle causes these organs to contract, which happens without our control. Smooth muscle is also called involuntary muscle. Sarcomas that develop in smooth muscle are called leiomyosarcomas. They are one of the more common types of sarcoma and can occur anywhere in the body, especially in the back of the abdominal area (retroperitoneum). Leiomyosarcomas are less often found in the deep, soft tissues of the legs or arms. They tend to occur in adults, particularly in older people.

Skeletal muscle sarcomas

Skeletal muscles are the active muscles that we control in our arms and legs or other parts of the body. They are voluntary muscles and sometimes called striated muscles. This is because the cells look stripy when examined under a microscope. Sarcomas that grow in these voluntary muscles of the body are called rhabdomyosarcomas. They occur mostly in the head and neck, but also in organs such as the bladder, vagina andthe arms or legs. Rhabdomyosarcomas are more commonly diagnosed in children than in adults.

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Peripheral nerve tumours The body’s nervous system is divided into two parts: •• the central nervous system, which is made up of the brain and spinal cord •• the peripheral nervous system, which consists of all the nerves that run throughout the body. Sarcomas of the peripheral nerves develop in the cells that cover the nerves. They are known as malignant peripheral nerve sheath tumours (MPNST) and can occur anywhere in the body. There are different types of MPNSTs. They most commonly occur in people who have a rare genetic disorder called neurofibromatosis (von Recklinghausen’s disease).

Fibrous tissue tumours Fibrous tissues join together 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 fibrosarcoma. They are most commonly found on the arms, legs, chest or tummy, but can occur deeper in the body. They can develop at any age but are more commonly seen in people aged 20–60. Most people first notice them as a painless, firm lump.


This is a type of fibrosarcoma. It was previously called malignant fibrous histiocytoma (MFH). It is not clear which type of cell it starts from. Pleomorphic sarcomas can affect any part of the body, but most commonly occur in the arms or legs.


These tumours are sometimes known as Desmoid tumours. They are classified as benign tumours, not cancer. They are sometimes said to be halfway between a fibrosarcoma and a benign fibroma (a non-cancerous tumour of fibrous tissue). Desmoid tumours don’t tend to spread to other parts of the body, but they can spread into nearby tissues. This means they are usually treated in a similar way to cancerous sarcomas.

Blood and lymph vessel tumours Sarcomas that start from the cells that make up the walls of blood or lymph vessels are called angiosarcomas. Haemangiosarcomas develop from blood vessels and lymphangiosarcomas develop from the lymph vessels. Angiosarcomas sometimes occur in a part of the body that has been treated with radiotherapy many years before.

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Other types of sarcoma Gastrointestinal stromal tumours (GIST)

These are the most common type of soft tissue sarcoma. They develop in nerve cells in the walls of the digestive system. The digestive system is often called the gastrointestinal (GI) tract. This type of tumour may also be called GI stromal sarcoma. GISTs behave differently from other types of sarcoma and are treated very differently.

Kaposi’s sarcoma (KS)

Although Kaposi’s sarcoma is a type of sarcoma, it develops differently from other sarcomas. It starts from cells in the skin. Coloured patches or lumps can develop in the skin, mouth, lymph nodes or internal organs such as the lung, liver or spleen. Kaposi’s sarcoma can affect people with a weakened immune system, including people with HIV and AIDS. Other types can affect people of Jewish, Italian and West African origin. Kaposi’s sarcoma is treated differently from other types of soft tissue sarcoma.

Ewing’s sarcoma

Ewing’s sarcoma is named after the surgeon who first described it. It is more common in teenagers and young adults, but it can occur at any age. In young children, it is more common than osteosarcoma. Any bone can be affected, but the most common sites are the pelvis, thigh bone (femur) and shin bone (tibia). It is also possible for Ewing’s sarcoma to start in the soft tissues of the body. This is called extraosseous Ewing’s sarcoma (extra means outside, osseous means bone), or soft tissue Ewing’s sarcoma.


Osteosarcoma is the most common type of primary bone cancer. It is most common in teenagers, young adults and adults in their 60s, but people of any age can be affected. It can occur in any bone, but is most likely to develop around the knee, in the thigh bone (femur), in the shin bone (tibia) or in the upper arm (humerus).


Chondrosarcoma is usually a slow-growing tumour and is most common in middle-aged people. The cancer starts in cartilage cells, although it can also grow inside a bone or on its surface. The most common places for it to develop are the upper arm (humerus) or thigh bone (femur). But it can develop in other bones such as the ribs, pelvis or shoulder blade (scapula).

Spindle cell sarcoma of the bone

Spindle cell sarcoma is a rare type of bone cancer. It is similar to osteosarcoma, but normally occurs in adults over the age of 40. It is extremely rare in people under 20. When spindle cell sarcoma is in a bone, it is treated in a similar way to osteosarcoma. You may hear words like leiomyosarcoma or malignant fibrous histiocytoma (MFH) used to describe spindle cell sarcoma of the soft tissue.

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Angiosarcoma can occur at any age. It usually affects the soft tissues but, rarely, it can develop in bone. Angiosarcomas can affect any bone. They can develop in more than one bone at the same time, or in more than one place in a single bone.

We have more information about all the types of soft tissue sarcoma mentioned in this section.

Rarer types of sarcoma

There are other much rarer types of sarcoma than those we have already listed. These include: •• alveolar soft part sarcoma •• dermatofibrosarcoma protuberans (DFSP) •• desmoplastic small round cell tumour •• epithelioid sarcoma •• extraskeletal myxoid chondrosarcoma •• giant cell fibroblastoma (GCF) •• mesenchymal chondrosarcoma •• clear cell sarcoma.

Our cancer support specialists on 0808 808 00 00 can give you information about these rarer types of sarcoma.

Soft tissue sarcomas in children Soft tissue sarcomas can occur in children, particularly some types of rhabdomyosarcoma. Their symptoms and treatment may differ from those in an adult with the same sarcoma.

We can send you a booklet called A parent’s guide to children’s cancer, which discusses the treatment of children’s cancers. We also have information about some types of sarcoma that occur in children.

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More information and support More than one in three of us will get cancer. For most of us it will be the toughest fight we ever face. And the feelings of isolation and loneliness that so many people experience make it even harder. But you don’t have to go through it alone. The Macmillan team is with you every step of the way. Visit macmillan.org.uk or call us on 0808 808 00 00, Monday to Friday, 9am to 8pm. You can order a copy of Understanding soft tissue sarcomas or any other cancer information, from be.macmillan.org.uk or by calling us. Hard of hearing? Use textphone 0808 808 0121, or Text Relay. Speak another language? We have telephone interpreters. We provide information in a range of languages and formats. Visit macmillan.org.uk/otherformats or call us.

We make every effort to ensure that the information we provide is accurate and up to date but it should not be relied upon as a substitute for specialist professional advice tailored to your situation. So far as is permitted by law, Macmillan does not accept liability in relation to the use of any information contained in this publication, or third-party information or websites included or referred to in it. © Macmillan Cancer Support 2015. Registered charity in England and Wales (261017), Scotland (SC039907) and the Isle of Man (604). Registered office 89 Albert Embankment, London, SE1 7UQ Revised in September 2015. Next planned review in 2018.