NORD gratefully acknowledges Charles Forscher, MD, Co-Medical Director, Sarcoma Program, Samuel Oschin Cancer Center, Cedars-Sinai Medical Center, for assistance in the preparation of this report.
Sarcomas are malignant (cancerous) tumors that arise from cells that make up the connective tissues, which are the tissues that connect, support, and surround structures and organs in the body. They can be broadly broken down into bone, visceral and soft tissue forms. Soft tissues are tissues of the body that have not hardened or calcified like bone has. Soft tissues are found all over the body and include muscles, tendons, ligaments, nerves, fat, blood vessels, and lymph vessels. Soft tissue sarcomas can arise from fat, muscle, nerve, tendons, and blood and lymph vessel tissue. For some sarcomas the tissue of origin is uncertain such as pleiomorphic undifferentiated sarcoma. Synovial sarcoma is a misnomer as it does not arise from synovial tissue (as was originally thought). Sarcomas can occur almost anywhere in the body, but the most common areas are the arms and legs, the back of the abdomen (retroperitoneum) and head and neck. They can affect men or women, adults or children. These tumors are diverse with significantly different signs and symptoms, progression, and often different treatment regimens. The exact, underlying cause of these tumors is not fully understood. Most likely, complex genetic and environmental factors play a role in their development. As researchers learn more about the genetic factors associated with these disorders, newer, targeted therapies may become available.
Soft tissue sarcomas are a form of cancer. Cancer is characterized by abnormal, uncontrolled growth of cells that invades surrounding tissue and can sometimes spread (metastasize) to distant areas of the body via the bloodstream, the lymphatic system, or other means. Different forms of cancer, including soft tissue sarcomas, may be classified based upon the cell type involved, the specific nature of the malignancy, and the disease’s clinical course. According to the World Health Organization classification, there are more than 100 different histologic subtypes of soft tissue sarcomas.
This report is a general overview of soft tissue sarcomas as a group. NORD has individual reports on several types of soft tissue sarcoma, including: alveolar soft part sarcoma; desmoid tumors; extraosseous Ewing sarcoma; gastrointestinal stromal tumors (GIST); leiomyosarcoma; leiomyosarcoma of the inferior vena cava; liposarcoma; mesenchymal chondrosarcoma; pleuropulmonary blastoma; and uterine leiomyosarcoma. These forms of soft tissue sarcoma are not discussed in this report. Individual reports go into greater detail concerning the signs and symptoms and treatment options for these rare cancers. (For more information, choose the specific name as the search term for the Rare Disease Database.)
The signs and symptoms of soft tissue sarcomas can vary greatly from one person to another. Specific findings depend on numerous factors including the specific subtype, the exact location of the tumor, the extent of the tumor into nearby tissue or organs, the specific organs involved, and whether the disease has remained localized or spread to other areas of the body (metastasized).
Often, soft tissue sarcomas will not be associated with any noticeable symptoms early in the course of the disease. At some point, affected individuals may notice a slow-growing, painless mass in the affected area. Tumors can become very large and cause pain or symptoms related to pressing against (compression of) nearby organs, nerves, or structures in the body. Large tumors in the arms or legs can lead to a sensation of numbness, burning or tingling in the hands or feet (paresthesia). Tumors in the stomach or abdomen can cause abdominal pain, or blood in the stool. Swelling (edema) can occur in areas where tumors push up against lymph vessels. Tumors near the skin can damage the skin, causing open sores (ulceration).
Sometimes, soft tissue sarcomas can cause nonspecific symptoms. These are symptoms that are common to many different disorders and conditions. These symptoms include nausea, vomiting, loss of appetite, unintended weight loss, unexplained fatigue, and a general feeling of poor health (malaise).
According to the World Health Organization, there are more than 100 histologic subtypes of soft tissue sarcoma. Several of the better-known subtypes are briefly discussed below.
This form of cancer starts in cells that make up the walls of blood vessels. When it affects lymphatic tissue, it is called a lymphangiosarcoma. The lymphatic system is part of the immune system and helps to protect the body against infection and disease. It consists of tubular channels (lymph vessels) that drain a watery fluid known as lymph from different areas of the body into the bloodstream.
Angiosarcoma can occur in almost any area of the body and is often aggressive with spread to other areas. The skin of the head and neck is a commonly affected site. Angiosarcoma of soft tissue can present in the lower extremities, the space behind the abdominal cavity (retroperitoneum) or the soft tissue of the chest (mediastinum). Angiosarcoma of the breast can present as either a mass within the breast or as a cutaneous angiosarcoma seen as a rare late effect of radiation for breast cancer.
In most instances, there are no obvious symptoms early in their development. Angiosarcoma are prone to bleeding (hemorrhaging). When angiosarcoma affects the skin, there may be reddish or purplish discoloration that looks like a bruise. A lesion may form that is prone to bleeding and swelling. When angiosarcoma affects an organ, symptoms depend upon the exact size and location of the tumor. Prior radiation exposure may play a role in the development of secondary angiosarcoma. Recent investigations have demonstrated differing genetic patterns between primary and secondary angiosarcoma.
A rare form of angiosarcoma, called lymphangiosarcoma or Stewart-Treves syndrome, develops in people with long-standing primary or secondary lymphedema.
Angiosarcoma may also present as a primary tumor in bone.
Clear cell sarcoma
This form accounts for less than 1% of all soft tissue sarcomas. These tumors arise in tendons, the tough, flexible tissue that attaches muscle to bone. They usually develop in the arms and legs, specifically the foot and ankle region. They usually develop as a slow-growing lump or mass that may or may not be tender or painful. Eventually, they tumor spreads to nearby tissue. They are most common in young adults. Under a microscope, the cells of these tumors resemble those of a skin cancer called melanoma, and sometimes distinguishing these two cancers can be difficult.
This form of soft tissue sarcoma arises in the inner layer of skin called the dermis. They may present as a slow-growing bump (nodule) or as a spot of hardened skin or a deep-seated pimple. They may involve nearby tissue, but usually do not spread to other areas of the body. These tumors may recur after surgery. The risk of distant spread is increased when these tumors undergo fibrosarcomatous transformation.
Desmoplastic small round cell tumor
This is an extremely rare form of soft tissue sarcoma that usually arises in the abdomen or pelvis, although it can develop elsewhere in the body. It is aggressive and often spreads to other areas of the body, particularly the lungs or liver. These tumors can cause pain and a mass can usually be felt in the abdomen. This form most commonly affects Caucasian men between 10 and 30 years of age, but can also affect young women and young children. These tumors can recur after treatment.
This form usually develops in the soft tissue just underneath the skin, most often in the fingers, hand, forearms, and lower legs or feet. These tumors can potentially form almost anywhere in the body. They often begin as a small growth or bump, and are usually painless. Sometimes, there are open sores on the skin above the tumor that may be mistaken for areas of infection. Eventually, multiple growths or bumps may develop. Epithelioid sarcoma can move into nearby tissue and spread to other areas of the body. This type of sarcoma has a tendency to spread to lymph nodes. It often recurs after treatment.
Malignant peripheral nerve sheath tumors
These tumors arise in the protective lining that covers the nerves of the peripheral nervous system. These are the nerves outside of the brain and spinal cord. They appear as a lump or mass and can develop throughout the body. They can cause pain, weakness or difficulty moving the affected body part. When they press up against (compress) nerves, they can cause a sensation of burning, tingling, or numbness in certain areas. They tend to recur after treatment. They may be associated with a rare genetic disorder called neurofibromatosis type 1 but can also occur without a genetic predisposition.
This form of soft tissue sarcoma is usually found most often in the arms or legs. It begins as a slow-growing bump or bumps and can move into nearby tissue or spread to other areas of the body. They are usually painless. These tumors often recur after treatment. They usually develop in people over the age of 50 and occur slightly more often in men than women. It is one of the most common forms of sarcoma found in adult patients.
One of the most common types of soft tissue sarcoma, these tumors usually develop from cells which have features related to striated muscle cells. Striated muscle tissue is the muscle that is attached to the skeleton by tendons and is part of the voluntary muscle system. These are the muscles that are controlled by a person’s conscious thought as opposed to muscles that act involuntary like the heart or muscles found within organs like the bladder or intestines.
Rhabdomyosarcomas arise from cells within the developing fetus called rhabdomyoblasts. These are immature cells that grow into the cells that help to form skeletal and striated muscle. These tumors can develop anywhere in the body, even areas of the body where there isn’t skeletal muscle tissue. The head and neck area, the urinary system including the bladder, the arms and legs, and the reproductive system including the vagina, uterus, and testes are common sites of rhabdomyosarcoma development. The signs and symptoms depend on where the tumor is located.
Rhabdomyosarcomas are the most common form of soft tissue sarcoma in children. They are far more common in children, but can sometimes occur in adults. Adults tend to have a faster growing form of the disorder.
Solitary fibrous tumor
These are individual, slow-growing tumors. Although they can develop almost anywhere in the body, they most often arise in the lining of the lungs (pleura), pelvis, or the dura, which is the thick, outer layer of the three membranes (meninges) that cover and protect the brain and spinal cord. These tumors tend to grow very slowly and go not cause any symptoms until they are very large. Most are noncancerous (benign), but malignant (cancerous) tumors can develop.
This form of soft tissue sarcoma usually develops in the arms or legs, often near a joint but rarely, if ever, within a joint. It can also develop in the trunk or abdomen. They usually present as a small growth or lump and are sometimes associated with pain. Eventually, a synovial sarcoma can cause limitation in the movement of the nearby joint. Doctors do not know what type of cell causes a synovial sarcoma. There are called synovial sarcomas because the cells of the tumor were felt to resemble cells of the synovium, which is a layer of connective tissue that lines joints. They most often occur in young adults. These tumors sometimes recur after treatment and are capable of metastasis.
Undifferentiated pleomorphic sarcoma
This is an aggressive form of soft tissue sarcoma that can develop in soft tissue or bone in any part of the body. These tumors tend to affect older adults in their 50s or older. They most often develop in the lower legs (especially the thighs), the upper arms, behind the membrane that lines the abdomen and covers the abdominal organs (retroperitoneum), and the main organs of the body, especially those of the abdomen (viscera). In childhood, they most often form in the head or neck. They usually grow quickly and often spread to other areas of the body, especially the lungs. They often cause pain or swelling in the affected area. They can recur after treatment. This form of soft tissue sarcoma was once referred to as malignant fibrous histiocytoma.
As with many forms of cancer, the exact, underlying cause of soft tissue sarcoma is not fully understood. In most affected individuals, this cancer is thought to occur randomly, for no specific reason (sporadically). Sporadic occurrence of cancer is thought to occur because of multiple factors acting together. This can include genetic and environmental factors. Current research suggests that abnormalities in DNA (deoxyribonucleic acid), which is the carrier of the body’s genetic code, are the underlying basis that causes cells to become cancerous (malignant). The specific cell that becomes cancerous differs depending upon the specific subtype of soft tissue sarcoma. In most instances, these changes in DNA are acquired during life and are not inherited, nor do soft tissue sarcomas tend to run in families, unless affected individuals also have a genetic cancer-predisposition syndrome.
Changes (variations) in certain genes have been noted to occur in greater frequency in individuals with soft tissue sarcoma than in people without this form of cancer. Many soft tissue sarcomas are associated with reciprocal translocations. Reciprocal translocations occur when regions of chromosomes break off and “trade places.” This results in the shifting of genetic material and an altered set of chromosomes. These translocations can involve oncogenes. Genes normally produce (encode) proteins that have several functions in the body. Oncogenes control cell growth. Reciprocal translocations can sometimes result in the abnormal fusion of two genes. This abnormal fusion results in a “fusion” gene that produces an abnormal protein product. Researchers believe that this abnormal protein product may contribute to the growth and spread of cancer in such instances.
Some forms of soft tissue sarcoma are associated with a variation (mutation) in a single oncogene, which is believed to drive the growth of cancer. An altered (mutation) oncogene may produce a protein that is ineffective, overproduced, or underproduced. Usually, oncogenes are activated or “turned on” when they are not supposed to allowing for the growth and replication of cells.
Some forms of soft tissue sarcomas have a complex karyotype. Karyotype refers to the number and appearance of chromosomes in a person. Chromosomes, which are present in the nucleus of human cells, carry the genetic information for each individual. Human body cells normally have 46 chromosomes. Pairs of human chromosomes are numbered from 1 through 22 and the sex chromosomes are designated X and Y. Males have one X and one Y chromosome and females have two X chromosomes. A complex karyotype means that there are a few to several variations to the chromosome makeup (e.g., translocations, etc.).
Some sarcomas have neither chromosomal translocations nor complex karyotypes. Rather they have amplification of a gene such as MDM2 in dedifferentiated liposarcoma, deletions of genes or loss of gene function such as INI1 in epithelioid sarcoma.
The underlying, genetic factors associated with soft tissue sarcoma are very complex and more research is necessary for doctors to figure out the all of the genetic interactions that contribute to the development of these tumors. Although the causes and genetic aspects of soft tissue sarcomas are not fully understood, several risk factors have been identified. Risk factors are anything that increases a person’s risk of developing a disease. Having a risk factor does not mean a person will definitely develop that disease, and people who do not have any risk factors can still develop the disease.
Environmental factors that have been associated with soft tissue sarcomas include radiation therapy (often to treat a different form of cancer) and exposure to certain chemicals including vinyl chloride, arsenic, and thorium dioxide. In certain forms, damage to the lymphatic system, specifically long-standing lymphedema (swelling of the lymph vessels because of the abnormal accumulation of fluid), is a risk factor.
Soft tissue sarcomas occur with greater frequency in people or families that have rare genetic cancer-predisposing syndromes such as hereditary retinoblastoma, Li-Fraumeni syndrome, neurofibromatosis type I, familial adenomatous polyposis (FAP), tuberous sclerosis, nevoid basal cell carcinoma syndrome (Gorlin syndrome) or Werner syndrome. These disorders are associated with specific genes that are shown to increase the risk of specific forms of cancer. These disorders can be inherited. People who have these disorders are more likely to develop a soft tissue sarcoma than the general population as a whole. Recent data has suggested genetics may play a greater role in the risk of developing a sarcoma than previously felt.
Soft tissue sarcomas affect men and women and individuals of any age and every racial and ethnic group. This is a large group of tumors, but overall, they are still classified as rare disorders. Estimates to their frequency varies. The American Cancer Society estimates that soft tissue sarcomas account for about 2% of all cancers in the United States. However, because rare diseases often go misdiagnosed or undiagnosed, determining their true frequency in the general population is difficult.
A diagnosis of a soft tissue sarcoma is based upon identification of characteristic symptoms, a detailed patient and family history, a thorough clinical evaluation and a variety of specialized tests. A physical examination can reveal a mass or growth that can be painless.
Clinical Testing and Workup
A biopsy is the only way to definitively tell if a mass or tumor is a sarcoma. A biopsy is test where a piece of tissue is taken from the tumor and examined under a microscope. This allows a special doctor called a pathologist to see the specific cells and characteristics of the tissue sample. Doctors can then distinguish a soft tissue sarcoma from other types of benign and cancerous tumors as well as determine the specific subtype of soft tissue sarcoma that is present. Additional testing of the tumor may involve specialized pathology techniques such as immunohistochemistry. Some tumors will be tested for specific genetic characteristics by either fluorescent in-situ hybridization (FISH) analysis or next generation sequencing.
Many doctors and medical centers recommend a core needle biopsy. A core needle biopsy is generally considered to be more accurate than fine needle aspiration. During a core needle biopsy, a wide, hollow needle is inserted into the mass or growth to take a piece of tissue. The needle is moved into and out of the tumor by a spring. A cylindrical-shaped piece of tissue called a core is removed. Several cores are taken.
Fine needle aspiration is another type of biopsy. It involves a thinner, hollow needle, which is inserted into the tumor to remove tissue. The needle is attached to a syringe, which is used to draw out (aspirate) a sample of tissue and fluid from the mass or tumor.
Imaging techniques may be used before or after a biopsy is taken. Imaging techniques will be used to evaluate the size, placement, and extension of the tumor (e.g. into the soft tissue or surrounding areas), to determine whether the tumor has spread (metastasized) to the lungs or other areas of the body, and to serve as an aid for future surgical procedures. Conventional x-rays (plain radiographs) of the lungs are often recommended because the lungs are the most common site of spread (metastasis) with soft tissue sarcomas. Some treatment centers recommend all newly-diagnosed individuals with soft tissue sarcoma in the arms and legs or trunk receive a chest x-ray or chest CT scan.
Specialized imaging techniques are also used to help with the diagnosis or assessment of soft tissue sarcoma. These techniques can include magnetic resonance imaging (MRI), computerized tomography (CT scan), and ultrasound. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues including bone marrow and soft tissue. Sometimes, CT scans may be used for people who cannot have (or tolerate) an MRI. During CT scanning, a computer and x-rays are used to create a film showing cross-sectional images of certain tissue structures. An ultrasound uses reflected sound waves to create pictures of internal organs and other structures and are effective at detecting small areas of cancer that are near the surface of the body (superficially located). An ultrasound may also be used to determine whether fluid is present in an unidentified mass.
Another advanced imaging technique known as positron emission tomography or PET scan may also be used. During a PET scan, a radioactive sugar is injected into the body. This sugar will collect in areas of the body where there is a higher demand for energy. Tumors require a lot of energy to keep growing and spreading, and will soak up the radioactive sugar. When the x-ray (scan) is taken, areas that take up the radioactive sugar including soft tissue sarcomas may show up as bright spots on the film. A PET scan is often used to help show whether a soft tissue sarcoma has spread or how well it is responding to treatment.
The therapeutic management of individuals with soft tissue sarcoma may require the coordinated efforts of a team of medical professionals, such as physicians who specialize in the diagnosis and treatment of cancer in children (pediatric oncologists), oncologists, physicians who specialize in the treatment of cancer with radiation (radiation oncologists), physicians who specialize in examining tissues and cells to find disease and determine what disease is present (pathologists), surgeons who specialize in removing cancer by surgery (oncology surgeon or orthopedic oncology surgeon), nurses who specialize in the car and treatment of cancer (oncology nurses), psychiatrists, nutritionists, and other healthcare specialists. Psychosocial support for the entire family is essential as well.
Specific therapeutic procedures and interventions may vary, depending upon numerous factors, such as disease stage; tumor size and specific location; specific soft tissue sarcoma subtype; the presence or absence of certain symptoms; an individual’s age and general health; and/or other elements. Decisions concerning the use of surgery, particular drug regimens, and/or other treatments should be made by physicians and other members of the health care team in careful consultation with the patient based upon the specifics of his or her case; a thorough discussion of the potential benefits and risks, including possible side effects and long-term effects; patient preference; and other appropriate factors.
Several organizations have released guidelines for the surveillance, screening and treatment of soft tissue sarcoma. The National Comprehensive Cancer Network (NCCN) guidelines are one of the most commonly used and the organization provides a booklet geared toward patient explaining these guidelines. A PDF of this booklet can be found at: https://www.nccn.org/patients/guidelines/sarcoma/.
It is recommended that individuals with soft tissue sarcoma be seen by a physician or team of physicians with experience with these disorders. In the United States, there are Sarcoma Centers, which are medical centers and hospitals that specialize in the diagnosis and treatment of people with soft tissue sarcoma. At a Sarcoma Centers, patients will be seen by a medical team of specialists with experience with these types of tumors.
The three main treatments used for soft tissue sarcoma are surgery, chemotherapy, and radiation therapy. Surgery involves removing (resecting) the entire tumor and a small amount of surrounding healthy tissue to ensure all of the cancer is eliminated. Tumors affecting the arms and legs are treated by limb-sparing surgery, if possible, which is designed to remove the tumor, but preserve the function of the limb as well as the physical appearance of the limb.
Chemotherapy is the use of certain medications to slow down or stop the growth of cancer cells. Cancers cells grow and divide rapidly, which makes them susceptible to chemotherapy medications. In soft tissue sarcoma, chemotherapy may be used to treat affected individuals. Sometimes, it is used along with a primary form of treatment, usually surgery. This is called neoadjuvant or adjuvant therapy. When used along with surgery, chemotherapy may be given before surgery (neoadjuvant) to shrink a tumor or following surgery (adjuvant) to eliminate any remaining cancer cells and lessen the risk of a recurrence. Sometimes, chemotherapy may be given before and after surgery. Different combinations of medications may be used; this is called a chemotherapy regimen. When chemotherapy is given, the specific chemotherapy regimen used can vary. Different medical centers may have their own preferences as to the best way to approach treatment and what chemotherapeutic regimen is best for each individual.
Radiation therapy is the use of high doses of radiation to kill cancer cells and shrink tumors. Radiation therapy preferentially destroys or injures rapidly-dividing cells, primarily cancerous cells. Radiation is passed through affected tissue to destroy cancer cells while minimizing exposure and damage to normal cells. Radiation therapy works to destroy cancer cells by depositing energy that damages the cells’ genetic material, preventing or slowing their growth and replication. Radiation therapy has been shown to decrease the local recurrence rate when added to surgery in either the pre-operative or post-operative setting.
The U.S. Food and Drug Administration (FDA) has approved several therapies for the treatment of specific subtypes of soft tissue sarcoma or for several different forms, collectively. These therapies include:
Olaratumab (Lartruvo®), which is approved in combination with doxorubicin for the treatment of adults with soft tissue sarcoma with a histologic subtype that can be treated with an anthracycline-containing chemotherapy regimen and is also not able to be treated by radiotherapy or surgery.
Pazopanib (Votrient®), which is approved for the treatment of individuals with advanced soft tissue sarcoma who have received prior chemotherapy (excludes liposarcoma).
Dactinomycin has been approved for the treatment of childhood rhabdomyosarcoma. The drug vincristine sulfate in conjunction with other chemotherapeutic agents has also been approved for rhabdomyosarcoma.
The drug imatinib mesylate (Gleevec®) is approved to treat adults with dermatofibrosarcoma protuberans that cannot be treated by surgery (unresectable), has recurred, or has spread (metastasized). Gleevec is also approved for gastrointestinal stromal tumors (GIST).
Trabectedin (Yondelis®) is approved for use in advanced liposarcoma or leiomyosarcoma. Eribulin (Halaven®) is approved for use in advanced liposarcoma.
Sunitinib (Sutent®) is approved as a second line agent for gastrointestinal stromal tumor (GIST). Regorafenib (Stivarga®) is approved as a third line agent for gastrointestinal stromal tumor (GIST).
Targeted therapies are being explored as potential treatments for individuals with soft tissue sarcoma. Targeted therapies are drugs and other substances that prevent the growth and spread of cancer by blocking or inhibiting certain specific molecules (often proteins) that are involved in the development of specific cancers. Generally, targeted therapies are less toxic than other treatments for cancer. Several research studies are ongoing to test various targeted therapies for specific subtypes of soft tissue sarcoma. More research is necessary to determine what genetic factors (e.g. altered genes) play a role in the development of the specific subtypes of soft tissue sarcoma and what types of targeted therapies may be possible to treat these tumors.
Immunotherapy is the most recent addition to therapy for cancer. It is designed to enlist the body’s immune system to act against the sarcoma. Trials incorporating immune agents in sarcoma are in the process of attempting to best define the use of these agents in sarcoma.
Information on current clinical trials is posted on the Internet at https://clinicaltrials.gov/. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Toll-free: (800) 411-1222
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Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
For information about clinical trials sponsored by private sources, contact:
For information about clinical trials conducted in Europe, contact:
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