Types of soft tissue sarcoma

Soft tissue sarcomas can develop from cells in the soft, connective tissues of the body. There are many different types and they tend to grow and develop differently. Knowing the type helps decide on the best treatment for you. 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. These types of sarcoma include:

  • Fat tissue tumours
  • Muscle tissue tumours
  • Peripheral nerve tumours
  • Fibrous tissue tumours
  • Blood and lymph vessel tumours.

Other types of sarcoma include:

  • Gastrointestinal stromal tumours (GISTs) – the most common type
  • Kaposi's sarcoma (KS)
  • Ewing’s sarcoma
  • Osteosarcoma
  • Chondrosarcoma
  • Spindle cell sarcoma of the bone
  • Angiosarcoma.

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

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 and the arms or legs.

Rhabdomyosarcomas are more commonly diagnosed in children than in adults.


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.
The nervous system
The nervous system

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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.

Pleomorphic

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.

Fibromatosis

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 vessels 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.


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

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

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.

Angiosarcoma

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.


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.


Soft tissue sarcomas in children

Back to Understanding soft tissue sarcomas

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