A soft tissue sarcoma is a malignant (cancerous) tumor that develops in mesenchymal tissue. Mesenchymal tissues encompass all the muscle, connective tissues, and bones of the body.
Soft tissue sarcomas can invade surrounding tissue and can metastasize (spread) to other organs of the body, forming secondary tumors. The cells of secondary tumors are similar to those of the primary (original) cancer. Secondary tumors are referred to as "metastatic soft tissue sarcoma" because they are part of the same cancer and are not a new disease. The most common site of spread is to the lungs.
Sarcoma is cancer of the connective tissue such as muscle, cartilage, blood vessels, nerves and bones. Soft tissue (non-bone) sarcomas are rare, but they can occur in many parts of the body such as muscle or fat of the extremities or the trunk. They can also occur in the abdomen, pelvis or chest. There are many types of soft tissue sarcomas, but most are treated in the same way. Some common soft tissue sarcomas include fibrosarcoma, liposarcoma, malignant fibrous histiocytoma, rhabdomyosarcoma and synovial sarcoma.
Types of Soft Tissue Sarcomas
There are many types of soft tissue sarcomas. Some of these sarcomas include:
- Alveolar rhabdomyosarcoma
- Alveolar soft part sarcoma
- Clear cell sarcoma of soft tissue
- Dedifferentiated liposarcoma
- Dermatofibrosarcoma protuberans
- Desmoplastic small round cell tumor
- Embryonal rhabdomyosarcoma
- Epithelioid fibrosarcoma
- Epithelioid hemangioendothelioma
- Epithelioid sarcoma
- Extrarenal rhabdoid tumor
- Gastrointestinal stromal tumor
- Infantile fibrosarcoma
- Inflammatory myofibroblastic tumor
- Kaposi sarcoma
- Malignant fibrous histiocytoma
- Malignant mesenchymoma
- Malignant peripheral nerve sheath tumor
- Myxoid liposarcoma
- Myxoinflammatory fibroblastic sarcoma
- Neoplasms with perivascular epithelioid cell differentiation (PEComa)
- PEComa (neoplasm with perivascular epithelioid cell differentation)
- Pleomorphic liposarcoma
- Pleomorphic rhabdomyosarcoma
- Pleomorphic sarcoma
- Round cell liposarcoma
- Solitary fibrous tumor
- Synovial sarcoma
Where in the body are soft tissue sarcomas more likely to develop?
Soft tissue sarcomas can arise almost anywhere in the body. About 43 percent occur in the extremities (e.g., arms, legs) 34 percent occur in and around the internal organs (e.g., uterus, heart) 10 percent occur in the trunk (e.g., chest, back) and 13 percent occur in other locations . In very rare cases, these tumors develop in the gastrointestinal tract. A small percentage of these are GISTs. Malignant GISTs occur most commonly in the stomach and small intestine.
What are the possible causes of soft tissue sarcomas?
Although most soft tissue sarcomas do not have a clearly defined cause, researchers have identified several factors that increase the likelihood of developing these tumors. External radiation therapy is the most well-established risk factor for soft tissue sarcomas. Patients treated with radiation therapy for cancers of the retina, breast, cervix, ovary, testes, or lymphatic system have a much higher chance of developing soft tissue sarcomas than the general population. The risk appears to be related to the dose of radiation. To limit this risk, radiation treatment for cancer is planned to ensure that the greatest amount of radiation is delivered to diseased tissue while surrounding healthy tissue is protected as much as possible.
Another risk factor for soft tissue sarcomas is exposure to certain chemicals in the workplace, including vinyl chloride, arsenic, herbicides such as phenoxyacetic acids, and wood preservatives that contain chlorophenols. Chronic lymphedema (a condition in which excess fluid collects in the tissue and causes swelling) following radiation to, or surgical removal of, lymph nodes is also a risk factor.
Symptoms of soft tissue sarcomas
Sarcomas often don’t cause any symptoms for a long time. They can start in any part of the body and the symptoms will depend on the part of the body that is affected.
If the sarcoma is in an arm or a leg, the most common symptom is an uncomfortable swelling in the affected limb. Occasionally, this swelling may be painful or tender, but it may also be painless.
If the sarcoma is in the central part of the body (the trunk), the symptoms will depend on which organ in the body is affected.
- a sarcoma in a lung may cause a cough and breathlessness
- a sarcoma in the abdomen could cause abdominal pain, vomiting and constipation
- a sarcoma affecting the womb may cause bleeding from the vagina and pain in the lower part of the abdomen.
Things to look out for include:
- any lump, especially if it is increasing in size and is bigger than 5cm (2in)
- any lump that is painful or tender
- any lump that is deep in the body (ie not just under the skin)
- any lump that has come back after being surgically removed.
As sarcomas are rare cancers, you should always be referred for treatment at a specialist sarcoma unit where a team of specialist doctors and others work together.
This is known as a multidisciplinary team (MDT) and may include:
- a surgeon
- a clinical oncologist – a doctor who treats cancer with radiotherapy
- a medical oncologist – a doctor who treats cancer with chemotherapy
- a pathologist – a doctor who specialises in how disease affects the body
- a radiologist – a doctor who analyses x-rays and scans
- a specialist nurse who gives information and support to people with sarcoma.
- a dietitian
- occupational therapist
- psychologist or counsellor.
In order to plan the best treatment for you, your doctors will take into account your age, general health, and the type, stage and grade of the sarcoma.
Types of Treatment
Surgery is the most common treatment. If the soft tissue sarcoma is small and it is possible to remove it completely, surgery may be used on its own.
For larger sarcomas, and where there may be a possibility of cancer cells being left behind, radiotherapy is usually used as well as surgery. Radiotherapy may be given before the operation to shrink the tumour and make it easier to remove, or afterwards to try to destroy any cancer cells that may not have been removed.
Sometimes radiotherapy is used on its own or in combination with chemotherapy |to treat more advanced sarcomas that can’t be removed.
Chemotherapy may sometimes be given before surgery, to shrink the tumour. It is sometimes given after surgery, to try to destroy any cancer cells that have not been removed or which may have spread to other parts of the body.
For gastrointestinal stromal tumours a drug called Imatinib may be used before or after surgery.
It often helps to make a list of the questions you want to ask your doctor, and to take a close friend or relative with you.
Benefits and disadvantages of treatment
Many people are frightened at the idea of having cancer treatments, because of the potential side effects that can occur. Although many of the treatments can cause side effects, these can often be well controlled with medicines. Some people ask what would happen if they did not have any treatment. Treatment can be given for different reasons and the potential benefits will vary depending upon the individual situation.
In people with early stage sarcoma, surgery is often done with the aim of curing the cancer, and additional treatments may also be given to reduce the risks of it coming back.
Advanced-stage sarcoma (metastatic)
If the cancer is at a more advanced stage, the treatment may only be able to control it – leading to an improvement in symptoms and a better quality of life. However, for some people the treatment will have no effect upon the cancer and they will get the side effects with little benefit.
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