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What is a soft tissue sarcoma?
A sarcoma is a type of cancer that develops from certain tissues, like bone or muscle. There are 2 main types of sarcoma: bone sarcomas and soft tissue sarcomas. Soft tissue sarcomas can develop from soft tissues like fat, muscle, nerves, fibrous tissues, blood vessels, or deep skin tissues. They can be found in any part of the body. Most of them develop in the arms or legs. They can also be found in the trunk, head and neck area, internal organs, and the area in back of the abdominal cavity (known as the retroperitoneum). Sarcomas are not common tumors, and most cancers are the type of tumors called carcinomas.
There are many types of soft tissue tumors, and not all of them are cancerous. When a tumor is not cancerous, it is called benign. When the term sarcoma is part of the name of a disease, it means the tumor is malignant (cancer). There are about 50 different types of soft tissue sarcomas (not all are listed here).
This document is about soft tissue sarcomas in adults. Sarcomas occurring in bone, such as osteosarcomas and Ewing tumors are discussed in separate documents. Rhabdomyosarcoma, the most common type of soft tissue sarcoma seen in children, is also discussed in another document.
Fat tissue tumors
Benign fat tissue tumors : Lipomas are benign tumors of fat tissue. They are the most common benign soft tissue tumor. Most are found just under the skin, but they can develop anywhere in the body.
Lipoblastomas are benign fat tumors that occur in infants and young children.
Hibernomas, like lipomas, are also benign fat tissue tumors. They are much less common than lipomas.
Cancerous fat tissue tumors : Liposarcomas are malignant tumors of fat tissue. They can develop anywhere in the body, but they most often develop in the thigh, behind the knee, and inside the back of the abdomen. They occur mostly in adults between 50 and 65 years old.
Muscle tissue tumors : There are 2 types of muscle: smooth and skeletal.
Smooth muscle is found in internal organs such as stomach, intestines, blood vessels, or uterus (womb) and causes them to contract. These muscles are involuntary — we don’t control their movement.
Skeletal muscle is sometimes called striated (because stripes can be seen inside the cells under the microscope). This is the type of muscle that lets us move our arms and legs and other body parts when we want them to move— this is called voluntary movement.
Benign muscle tumors : Leiomyomas are benign tumors of smooth muscle (or involuntary muscle). Leiomyomas can start from the walls of blood vessels, so they can develop almost anywhere in the body. They can be found in both men and women, but the most common place to find a leiomyoma is in the walls of the uterus. They are often called fibroids.
Rhabdomyomas are rare benign tumors of skeletal muscle.
Malignant muscle tumors : Leiomyosarcomas are malignant tumors of smooth muscle. Like leiomyomas, they can grow almost anywhere in the body. They are most often found in the retroperitoneum (area in back of the abdominal cavity), the internal organs, and blood vessels. These tumors are less often found in the deep soft tissues of the legs or arms. They tend to occur in adults, particularly the elderly. Leiomyosarcomas of the uterus are discussed in detail in our document, Uterine Sarcoma.
Rhabdomyosarcomas are malignant tumors of skeletal muscle. These tumors commonly grow in the arms or legs, but they can also begin in the head and neck area and in reproductive and urinary organs like the vagina or bladder. Children are affected much more often than adults. For more information, see our document, Rhabdomyosarcoma.
Peripheral nerve tissue tumors : The brain and spinal cord are parts of the central nervous system. The nerves that run throughout the body are part of the peripheral nervous system. Tumors can start in these peripheral nerves.
Benign nerve tumors : Neurofibromas, schwannomas (neurilemmomas), and neuromas are all benign tumors of nerves. These tumors can occur almost anywhere in the body. Neurofibromas are very common in people with an inherited condition calledneurofibromatosis (also called von Recklinghausen disease). Sometimes neurofibromas of very large nerves (like those in the upper arms or neck) can become malignant.
Malignant nerve tumors : Neurofibrosarcomas, malignant schwannomas, and neurogenic sarcomas are malignant tumors of the cells that surround a nerve. These are also called malignant peripheral nerve sheath tumors.
Gastrointestinal stromal tumor (GIST) is a type of sarcoma that develops in the digestive tract. It starts in the cells that control the muscles lining the stomach and intestine. These muscles propel food through the digestive tract. GISTs are not discussed further in this document, but are covered in detail in our document, Gastrointestinal Stromal Tumor (GIST).
Joint tissue tumors : Our joints are surrounded by a capsule made of a tough tissue called synovium. This tissue produces a fluid that lubricates the joint surfaces so that they move smoothly. Tumors of joints can start in the synovium.
Benign joint tumors : Nodular tenosynovitis is a benign tumor of joint tissue. It is most common in the hands and is more common in women than in men.
Malignant joint tumors : Synovial sarcoma is a malignant tumor of the tissue around joints (the synovium). The most common locations are the knee and ankle. Other sites are the shoulder and hip. This tumor is more common in children and young adults, but it can occur in older people.
Blood and lymph vessels tumors :
Benign vessel tumors : Hemangiomas are benign tumors of blood vessels. They are rather common and can affect the skin or internal organs. They are sometimes present at birth, and some disappear without treatment.
Lymphangiomas are benign lymph vessel tumors that are usually present at birth. Lymph is a fluid that circulates in every tissue of the body, ending up in the venous system. It carries waste products from tissues and immune system cells.
Glomus tumors are benign tumors that are found around blood vessels (perivascular). They usually are found under the skin of the fingers.
Intermediate vessel tumors : Hemangiopericytoma is another tumor of perivascular tissue. This tumor can be either benign or malignant. It most often starts in the legs, pelvis, and retroperitoneum (the back of the abdominal cavity). It is most common in adults. This type of tumor usually doesn't spread to distant sites, but it does tend to come back in or near the same place that it started, even if it was removed completely at surgery.
Hemangioendothelioma is a blood vessel tumor that is considered a low-grade cancer (meaning it grows slowly and is slow to spread). It does grow into nearby tissues and sometimes can spread to distant parts of the body (metastasize). It may start in soft tissues or in internal organs, such as the liver or lungs.
Malignant vessel tumors : Angiosarcoma is a malignant tumor that can develop either from blood vessels (hemangiosarcomas) or from lymph vessels (lymphangiosarcomas). These tumors are linked to radiation exposure — they sometimes start in a part of the body that has been treated with radiation. Angiosarcomas are sometimes seen in the breast after radiation therapy for breast cancer, and in limbs that are chronically swollen because lymph circulation is blocked (lymphedema).
Kaposi sarcoma is a cancer formed by cells similar to those lining blood or lymph vessels. In the past, Kaposi sarcoma was an uncommon cancer mostly seen in older people with no apparent immune system problems. But it is now seen more often in people with suppressed immune systems (from HIV infection and in organ transplant patients). It is not discussed further in this document, but is covered in detail in our document, Kaposi Sarcoma.
Fibrous tissue tumors : Fibrous tissue forms tendons and ligaments and covers bones as well as other organs in the body.
Benign fibrous tumors
These include:
Fibromas
Elastofibromas
Superficial fibromatosis
Fibrous histiocytomas
Intermediate fibrous tumors : Fibromatosis is the name given to fibrous tissue tumor with features in between fibrosarcoma and benign tumors such as fibromas and superficial fibromatosis. They tend to grow slowly but, often, steadily. These tumors are also called desmoid tumors, as well as the more scientific name musculoaponeurotic fibromatosis. They do not spread to distant sites, but they do cause problems by growing into nearby tissues. They can sometimes be fatal. Some doctors consider them a type of low-grade fibrosarcoma; but others believe they are a unique type of fibrous tissue tumors. Certain hormones, particularly estrogen, make some desmoid tumors grow. Anti-estrogen drugs are sometimes useful in treating desmoids that cannot be completely removed by surgery.
Dermatofibrosarcoma protuberans is a slow-growing cancer of the fibrous tissue beneath the skin, usually in the trunk or limbs. It grows into nearby tissues but rarely spreads to distant sites.
Malignant fibrous tumors : Fibrosarcoma is cancer of fibrous tissue. It usually affects the legs, arms, or trunk. It is most common in people between the ages of 20 and 60, but can occur at any age, even in infancy.
Uncertain tissue type tumors : Doctors look at tumor tissue under the microscope and do other tests and can usually find similarities between most sarcomas and certain types of normal soft tissues. But some sarcomas have not been linked to a specific type of normal soft tissue.
Benign uncertain tissue type tumors: Myxoma is a benign tumor that usually is located in muscles but does not start from muscle cells. The cells of a myxoma produce mucus-like material, a feature that distinguishes this tumor. It almost always occurs in adults.
Granular cell tumors are usually benign tumors in adults that occur often in the tongue but can be found almost anywhere in the body.
PEComa is a family of tumors made up of abnormal cells called perivascular epithelial cells. Although most of these tumors are benign, some rare PEComas are malignant (cancer). The most common PEComas are angiomyolipoma and lymphangioleiomyomatosis (LAM). Angiomyolipoma is a benign tumor that most often affects the kidney. LAM is a disease of women in which the tumor cells grow into the lung tissue and interfere with lung function.
Malignant uncertain tissue type tumors : Malignant mesenchymoma is a rare type of sarcoma that contains some areas showing features of fibrosarcoma and other areas with features of at least 2 other types of sarcoma.
Alveolar soft-part sarcoma is a rare cancer that mostly affects young adults. These tumors most commonly occur in legs.
Epithelioid sarcoma most often develops in tissues under the skin of the hands, forearms, feet, or lower legs. Adolescents and young adults are often affected.
Clear cell sarcoma is a rare cancer that often develops in tendons of the arms or legs. Under the microscope, it shares some features with malignant melanoma, a type of cancer that develops from pigment-producing skin cells. How cancers with these features start in parts of the body other than the skin is not known.
Desmoplastic small round cell tumor is a rare sarcoma of adolescents and young adults, found most often in the abdomen. Its name means that it is formed by small, round cancer cells surrounded by scar-like tissue.
Pleomorphic undifferentiated sarcoma, also known as malignant fibrous histiocytoma (MFH), is most often found in the arms or legs. Less often, it can start inside the back of the abdomen. This sarcoma is most common in older adults. Although it mostly tends to grow locally, it can spread to distant sites.
Spindle cell tumor and spindle cell sarcoma are named based on the long, narrow appearance of the cells under the microscope). A spindle cell tumor is a tumor with cells that look like these. Spindle cell tumor is not a specific diagnosis or a specific type of cancer. The tumor may be a sarcoma, or it can be sarcomatoid — meaning another type of tumor (like a carcinoma) that looks like a sarcoma under the microscope.
Other types of sarcoma
There are other types of tumors called soft tissue sarcomas, but these are all quite rare.
Tumor-like conditions of soft tissue
Some conditions of soft tissues are caused by inflammation or injury and can form a mass that looks like a soft tissue tumor. Unlike a true tumor, they do not come from a single abnormal cell, they have limited capacity to grow or spread to nearby tissues, and never spread through the bloodstream or lymph system. Nodular fasciitis and myositis ossificans are 2 examples which involve tissues under the skin and muscle tissues, respectively.
What are the risk factors for soft tissue sarcomas?
A risk factor is anything that changes your chance of getting a disease like cancer. Different cancers have different risk factors. For example, unprotected exposure to strong sunlight is a risk factor for skin cancer. Smoking is a risk factor for cancers of the lung, and many other cancers. But risk factors don’t tell us everything. Having a risk factor, or even several, doesn’t mean that you will get the cancer. Also, many people get cancer without having a risk factor.
Scientists have found a few risk factors that make a person more likely to develop soft tissue sarcomas.
Radiation exposure : Patients might develop sarcomas from radiation given to treat other cancers, like breast cancer or lymphoma. The sarcoma often starts in the area of the body that had been treated with radiation. The average time between radiation exposure and diagnosis of a sarcoma is about 10 years. Radiation exposure accounts for less than 5% of sarcomas.
Radiation therapy techniques have improved steadily over several decades. Treatments now target the cancers more precisely, and more is known about selecting radiation doses. These advances are expected to reduce the number of cancers caused by radiation therapy. But because these cancers take so long to develop, the results of these changes may not be seen for a long time. Still, radiation therapy is used only when its benefits (improved survival rate and relief of symptoms) outweigh the risk of cancer and other complications.
Genetic syndromes : Certain inherited conditions increase a person's risk of developing soft tissue sarcomas.
Neurofibromatosis : Neurofibromatosis is a disease that usually runs in families and is characterized by many neurofibromas (benign tumors that form in nerves under the skin and in other parts of the body). It is also known as von Recklinghausen disease. It is caused by a defect (mutation) in a gene called NF1. About 5% of people with neurofibromatosis will develop a malignant peripheral nerve sheath tumor in a neurofibroma.
Gardner syndrome : Gardner syndrome is a disease caused by defects in the gene APC. People with this syndrome get many polyps in the colon (and intestines) and have a high risk of getting colon cancer. It also causes musculoaponeurotic fibromatosis (also called desmoid tumors). Some experts consider desmoid tumors a slow-growing (low-grade) type of fibrosarcoma.
Li-Fraumeni syndrome : Li-Fraumeni syndrome is caused by inherited defects in the gene TP53. People affected by this syndrome have a high risk of cancer, such as breast cancer, brain tumors, and sarcomas. People with this syndrome are sensitive to the cancer-causing effects of radiation − if their cancer is treated with radiation, they have a very high chance of developing a new cancer in the part of the body that received the radiation.
Retinoblastoma : Retinoblastoma is an eye cancer in children that can be caused by defects in the gene RB1. Children with one of these gene defects also have an increased risk of developing bone or soft tissue sarcomas, especially if treated for cancer with radiation.
Werner syndrome : Werner syndrome is caused by defects in the gene RECQL2. Children with this syndrome have problems like those seen in the elderly. These include cataracts, skin changes, and clogged heart arteries (arteriosclerosis) which can lead to heart attacks. They also have an increased risk of cancer, including soft tissue sarcomas.
Gorlin syndrome : Gorlin syndrome, also called nevoid basal cell carcinoma syndrome, is caused by defects in the PTC gene. People with this syndrome have a high risk of developing many basal cell skin cancers. They also have an increased risk of getting fibrosarcoma and rhabdomyosarcoma.
Tuberous sclerosis : Tuberous sclerosis can be caused by a defect in the TSC1 gene. It can also be caused by a defect in another gene:TSC2. People with this syndrome often have seizures and learning problems. They get benign tumors in many different organs. They also get kidney problems, often along with a kidney tumor called angiomyolipoma. People with tuberous sclerosis have an increased risk of getting rhabdomyosarcoma.
Damaged lymph system : Lymph is a clear fluid containing immune system cells that is carried throughout the body by a series of lymph vessels. These vessels connect lymph nodes (small bean-shaped collections of immune system cells). When lymph nodes have been removed by surgery or damaged by radiation therapy, lymph fluid can build up. This is calledlymphedema. In some parts of the world, severe lymphedema (a condition called elephantiasis) is sometimes caused by infection with a parasite that blocks lymph vessels.
Lymphangiosarcoma (a malignant tumor that develops in lymph vessels) is a very rare complication of chronic lymphedema.
Chemicals : Exposure to vinyl chloride (a chemical used in making plastics) is a risk factor for developing sarcomas of the liver, but it has not been proven to cause soft tissue sarcomas. Arsenic has also been linked to a type of liver sarcoma but not soft tissue sarcoma. Exposure to dioxin and to herbicides that contain phenoxyacetic acid at high doses (such as might occur to people who work on farms) may also be risk factors, but this is not known for certain. There is no evidence that herbicides (weed killers) or insecticides, at levels encountered by the general public, cause sarcomas.
Injury : An injury is not a risk factor for developing sarcomas. But this issue has caused some confusion in the past. One reason is that injury may produce a swelling that resembles a tumor but is not a true tumor. Also, when you are injured, the pain may draw your attention to the injured area. The area may be examined closely, and x-rays or other imaging studies may be obtained. This can make it more likely that any sarcoma that is present will be discovered, even though it may have been present for some time.
Do we know what causes soft tissue sarcomas?
Scientists still don't know exactly what causes most cases of soft tissue sarcoma, but they have found several risk factors that can make a person more likely to develop these cancers. And research has shown that some of these risk factors affect the DNA of cells in the soft tissues.
Researchers have made great progress in understanding how certain changes in DNA can cause normal cells to become cancerous. Our DNA carries the instructions for nearly everything our cells do. We usually look like our parents because they are the source of our DNA. However, DNA affects more than the way we look.
The DNA is divided into units called genes. Genes carry the recipes for making proteins, the molecules that determine all cell functions. Some genes contain instructions for proteins that control when our cells grow and divide.
Certain genes that promote cell division are called oncogenes. Others that slow down cell division or cause cells to die at the right time are called tumor suppressor genes. Cancers can be caused by DNA mutations (defects) that turn on oncogenes or turn off tumor suppressor genes.
Several familial cancer syndromes have been found in which inherited DNA mutations cause a very high risk of developing breast, colon, kidney, eye, or other cancers. In some of these, there is also an increased risk of developing soft tissue sarcomas. Researchers have characterized many of these DNA changes in the past few years.
Some inherited conditions that increase a person's risk of developing soft tissue sarcoma were noted in the section on risk factors. They are caused by defects (mutations) in genes that can be inherited from a parent. These gene defects can be found through genetic testing.
DNA mutations in soft tissue sarcoma are common. They are usually acquired during life rather than having been inherited before birth. Acquired mutations may result from exposure to radiation or cancer-causing chemicals. In most sarcomas, they occur for no apparent reason.
Researchers still do not know why most soft tissue sarcomas develop in people who have no apparent risk factors.
Can soft tissue sarcomas be prevented?
The only way to prevent some soft tissue sarcomas is to avoid exposure to risk factors whenever possible. Most sarcomas, however, develop in people with no known risk factors, so there is no way known at this time to prevent most cases. And for people receiving radiation therapy, there is usually little choice.
Can soft tissue sarcomas be found early?
People who have a strong family history of sarcomas or who have had other cancers when they were young, might wish to discuss the benefits and disadvantages of genetic testing with their doctor. The genetic testing results should always be explained by a genetic counselor or a specially trained doctor who can interpret the results and advise high-risk patients about early cancer detection.
Families with a history of certain inherited conditions caused by mutated tumor suppressor genes have an increased risk of developing soft tissue sarcomas. The mutated genes can be detected by genetic testing, so family members should discuss this option with their doctors. They should let their doctor know about any lumps or growths right away.
No screening tests and exams are recommended for people who have no family history of sarcoma or other sarcoma risk factors. For these people, the best approach to early detection is to notify their health care professional of any unexplained lumps or growths or other symptoms that may be caused by a soft tissue sarcoma.
How are soft tissue sarcomas diagnosed?
If you have symptoms or other reasons that suggest you may have a sarcoma, the doctor will use one or more methods to find out if the disease is really present.
Signs and symptoms of soft tissue sarcomas
When sarcomas start on the arms or legs, most people simply notice a lump that has grown over a period of time (weeks to months). It can be painful, but generally, it doesn't hurt. More than half of sarcomas begin in an arm or leg.
When sarcomas grow in the retroperitoneum (the back wall inside the abdomen), the symptoms they cause more often come from other problems. Sometimes the tumors cause pain. They may also cause blockage or bleeding of the stomach or bowels. They may grow large enough for the tumor to be felt in the abdomen. About 20% of sarcomas begin in the abdomen (stomach) area.
Sarcomas can also begin on the outside of the chest or abdomen (about 10%) or in the head or neck area (around 10%).
If you have any of the following problems, see a doctor right away:
A new lump or a lump that is growing anywhere on your body
Abdominal pain that is getting worse
Blood in your stool or vomit
Black, tarry stools (when bleeding happens in the stomach or bowels, the blood can turn black as it is digested, and it may make the stool look very black and tarry)
Since symptoms of soft tissue sarcomas often do not appear until the disease is advanced, only about 50% of soft tissue sarcomas are found in the early stages, before they have spread.
Medical history and physical exam : Your doctor will take a complete medical history to check for any risk factors and to ask you about symptoms, as well as any other medical conditions. They will also examine you for signs of sarcomas and other health problems.
Biopsy : A biopsy is a test where a sample of tissue is removed from a tumor to see if it is a type of cancer. The piece of tissue is looked at under a microscope and, some other tests may be done on the sample as well. A physical exam may suggest that a tumor is a sarcoma, but a biopsy is the only way to be certain that it is a sarcoma and not another type of cancer or a benign disease.
Several types of biopsies are used to diagnose sarcomas. Doctors with experience in these tumors will choose one, based on the size and location of the tumor. Most experts prefer a fine needle aspiration or a core needle biopsy as the first step.
Fine needle aspiration (FNA) biopsy : In FNA, the doctor uses a very thin needle and a syringe to withdraw small fragments of tissue from the tumor mass. The doctor can often aim the needle while feeling a mass near the surface of the body. If the tumor is too deep to feel, the doctor can guide the needle while viewing a computed tomography (CT) scan. The main advantage of FNA is that it can be used to biopsy tumors deep in the body without surgery. The disadvantage is that the thin needle may not remove enough tissue to make a precise diagnosis.
FNA is often useful in showing that a mass first thought to be a sarcoma (found on physical exam or imaging tests) is really another type of cancer, a benign tumor, an infection, or some other disease. But if FNA results suggest a sarcoma, another type of biopsy will usually be done to remove enough tissue to confirm that diagnosis. After a sarcoma is diagnosed, FNA is most useful in determining whether additional tumors in other organs are metastases.
Core needle biopsy : Core needle biopsies use a needle that is larger than the FNA needle. Sometimes this needle is called a Tru-Cutneedle. It removes a cylindrical piece of tissue about 1/16 inch across and 1/2 inch long. It usually removes enough tissue to see if a sarcoma is present. Like FNA, CT scans can be used to guide the needle into tumors of internal organs.
Surgical biopsy : In a surgical biopsy, the entire tumor or a piece of the tumor is removed during an operation. There are 2 types of surgical biopsies, excisional and incisional. In an excisional biopsy, the surgeon removes the entire tumor. In an incisional biopsy, only a piece of a large tumor is removed. An incisional biopsy almost always removes enough tissue to diagnose the exact type and grade of sarcoma. If the tumor is near the skin surface, this is a simple operation that can be done with local or regional anesthesia (numbing medication given near the mass or into a nerve). But if the tumor is deep inside the body, general anesthesia is used (the patient is asleep).
If a tumor is rather small, near the surface of the body, and not located near critical tissues (such as important nerves or large blood vessels), the doctor may choose to remove the entire mass and a margin of normal tissue in an excisional biopsy. This surgery combines the biopsy and the treatment into one operation, so it should only be done by a surgeon with experience in treating sarcomas.
If the tumor is large, then an incisional biopsy is needed. Only a surgeon experienced in sarcoma treatment should perform this procedure. That is because the incision needs to be planned so that the resulting wound can be completely removed later on when the entire tumor is removed.
Proper biopsy technique is a very important part of successfully treating soft tissue sarcomas. An improper biopsy can lead to tumor spread and problems removing the tumor later on. An incisional biopsy in the wrong place or an excision without wide enough margins can make it harder to completely remove a sarcoma later on. To prevent these problems, these 2 types of biopsies should only be done by a surgeon experienced in treating sarcomas. It is best that an incisional biopsy be done by the same surgeon who will later remove the entire tumor (if a sarcoma is found).
Testing biopsy samples : The tissue removed will be looked at under the microscope to see if cancer is present. If cancer is present, the doctor will try to determine what kind it is (sarcoma or carcinoma.
Grading: If a sarcoma is present, the biopsy will determine what type it is and its grade. The grade of a sarcoma is based on the way the cancer cells look under the microscope. In grading a cancer, the pathologist (a doctor who specializes in diagnosing diseases microscopically) considers 3 factors: how closely the tumor resembles normal tissue (differentiation), how many of the cells appear to be dividing, and how much of the tumor is made up of dying tissue.
These factors are scored, and then the scores are added to determine the grade of the tumor. The sarcomas that have cells that look more normal and have fewer cells dividing are generally placed in a low-grade category. Low-grade tumors tend to be slow growing, slower to spread, and often have a better outlook (prognosis) than higher-grade tumors. Certain types of sarcoma are automatically given higher scores for differentiation. This affects the overall score so much that they are never considered low grade. Examples of these include synovial sarcoma and embryonal sarcoma.
The grade is partly used to determine the stage of a sarcoma. The official staging system actually divides sarcomas into 3 grades (1 to 3). The grade of a sarcoma helps predict how rapidly it will grow and spread. It is useful in predicting a patient's outlook and helps determine treatment options.
Immunohistochemistry: Sometimes these special tests are needed to accurately determine whether a sarcoma is present and, if so, what type. Part of the biopsy sample is treated with special man-made antibodies that recognize cell proteins typical of certain kinds of sarcomas. The cells are treated with chemicals that cause the cells containing the specific proteins to change color. The color change is then seen under a microscope.
Cytogenetics: For this test, cells' chromosomes are examined with a microscope to look for changes. For example, in certain types of sarcomas part of one chromosome may be abnormally attached to part of a different chromosome (called a translocation). To see the chromosomes clearly, the cancer cells usually must be grown in laboratory flasks for at least a week.
Fluorescent in situ hybridization (FISH) can sometimes be used to detect translocations and other chromosome changes without first growing the cells in the lab. Tests of chromosome changes are not required to diagnose most sarcomas, but they are sometimes very useful. And as new changes are discovered, these tests may become more important and more common.
Reverse transcription polymerase chain reaction (RT-PCR): This test is another way to find translocations in some sarcomas (such as the Ewing family of tumors, alveolar rhabdomyosarcoma, and synovial sarcoma) to confirm the type of tumor. Instead of using a microscope to look for the chromosome changes as in cytogenetic testing or FISH, RT-PCR uses chemical analysis of the RNA (a substance related to DNA) from genes affected by the translocation. RT-PCR testing is often able to find translocations that aren't detected by cytogenetics.
Imaging tests
These may be done before a biopsy in certain cases, but often are done once a diagnosis of sarcoma is made.
Chest x-ray : This test may be done to determine if the sarcoma has spread to your lungs.
Computed tomography scans : The computed tomography (CT) scan is an x-ray procedure that produces detailed, cross-sectional images of your body. Instead of taking one picture like a conventional x-ray, a CT scanner takes many pictures as it rotates around you. A computer then combines these pictures into an image of a slice of your body. The machine will create multiple images of the part of your body that is being studied. A CT scan is often done if the doctor suspects a soft tissue sarcoma in the chest, abdomen, or the retroperitoneum. This test is also used to see if the sarcoma has spread into the liver or other organs.
Before any pictures are taken, you may be asked to drink 1 to 2 pints of a liquid called oral contrast. This helps outline the intestine so that certain areas are not mistaken for tumors. You may also receive an IV (intravenous) line through which a different kind of contrast dye (IV contrast) is injected. This helps better outline structures in your body.
The IV contrast dye can also cause some flushing (redness and warm feeling). Some people are allergic and get hives or, rarely, more serious reactions like trouble breathing and low blood pressure. Be sure to tell the doctor if you have ever had a reaction to any contrast material used for x-rays.
CT scans take longer than regular x-rays. You will need to lie still on a table, and the part of your body being examined is placed within the scanner, a doughnut-shaped machine that completely surrounds the table. Most scans only take a few minutes.
CT scans might be done to precisely guide a biopsy needle into a tumor that is inside the body — the chest or abdomen, for example. For this procedure, called a CT-guided needle biopsy, the patient remains on the CT scanning table while a radiologist advances a biopsy needle toward the location of the mass. CT scans are repeated until the doctors are sure the needle is within the mass.
Magnetic resonance imaging scans : Magnetic resonance imaging (MRI) scans use radio waves and strong magnets instead of x-rays to take pictures of the body. The energy from the radio waves is absorbed and then released in a pattern formed by the type of tissue and by certain diseases. A computer translates the pattern of radio waves given off by the tissues into a very detailed image of parts of the body. A contrast material might be injected, just as with CT scans, but is used less often.
MRI scans are often part of the work-up of any tumor that could be a sarcoma. They are often better than CT scans in evaluating sarcomas in the arms or legs. They provide a good picture of the extent of the tumor. They can tell your health care team many things about the tumor, including location, size, and sometimes even the type of tissue it comes from (like fat or muscle). This makes MRI scans useful in planning a biopsy.
MRIs are also very helpful in examining the brain and spinal cord : MRI scans are a little more uncomfortable than CT scans. First, they take longer — often up to an hour. Also, you have to lie inside a long tube, which is confining and can be upsetting. Special "open" MRI machines sometimes are an option for people who have claustrophobia (fear of enclosed spaces). MRI machines also make a thumping noise that you may find disturbing. Some places will provide headphones with music to block this noise out.
Ultrasound : Ultrasound uses sound waves and their echoes to produce pictures of parts of the body. A small instrument called a transducer emits sound waves and picks up the echoes as they bounce off the organs. The sound wave echoes are converted by a computer into an image that is displayed on a computer screen.
This is a very easy procedure. It uses no radiation, which is why it is often used to look at developing fetuses. For most ultrasounds, you simply lie on a table while a technician moves the transducer over the part of your body being examined. Usually, the skin is first lubricated with gel. This test may be done before a biopsy to see if the lump is a cyst, meaning it contains fluid and is likely benign, or if it is solid and more likely a tumor. This test is often not needed if an MRI was done.
Positron emission tomography scan: In this test, radioactive glucose (sugar) is injected into the patient's vein to look for cancer cells. Because cancers use glucose (sugar) at a higher rate than normal tissues, the radioactivity will tend to concentrate in the cancer. A scanner can spot the radioactive deposits. A positron emission tomography (PET) scan is useful when your doctor thinks the cancer has spread but doesn't know where. A PET scan can be used instead of several different x-rays because it scans your whole body. Often the PET scan is used with a CT scan. This helps decide if abnormalities seen on the CT scan are cancer or something else. PET is not often used for sarcoma, but it can be helpful in certain cases.
How are soft tissue sarcomas staged?
The process of finding out how far the cancer has spread is called staging. In sarcoma staging, doctors also evaluate the appearance of the tumor under the microscope and judge how fast the cancer seems to be growing. The stage of a sarcoma is the most significant factor in determining each patient's prognosis (the course of the disease and the chances of survival) and in selecting treatment options.
The information needed to stage sarcomas includes biopsies, imaging tests of the main tumor (usually with CT or MRI scans), and imaging tests of other parts of the body where the cancer may have spread.
When examining the biopsy sample, the pathologist (doctor who specializes in diagnosing diseases by looking at the tissue under a microscope) takes into account the number of cells that are actively dividing and how closely the cancer resembles normal tissue. He or she determines the cell type and grade and estimates how rapidly it will grow and spread.
A staging system is a standard way for the cancer care team to summarize the extent of a cancer's spread. The system often used to stage sarcomas is the TNM system of American Joint Committee on Cancer.
T stands for the size of the tumor.
N stands for spread to lymph nodes (small bean-shaped collections of immune system cells found throughout the body that help fight infections and cancers).
M is for metastasis (spread to distant organs).
In soft tissue sarcomas, an additional factor, called grade (G), is also part of tumor stage. The grade is based on how the sarcoma cells look under the microscope.
Grade (G)
The grade is a sign of how likely it is the cancer will spread. Previously, the grade of a sarcoma was only based on how normal the cells looked under the microscope (called differentiation). This was not very helpful, and under a new system (known as the French or FNCLCC system), grade is based on 3 factors:
Differentiation — given a score of 1 to 3, with 1 being assigned when the cancer cells look similar to normal cells and 3 being used when the cancer cells look very abnormal
Mitotic count — how many cancer cells are seen dividing under the microscope; given a score from 1 to 3 (a lower score means fewer cells were seen dividing)
Tumor necrosis — how much of the tumor is made up of dying tissue; given a score from zero to 2 (a lower score means there was less dying tissue present).
The scores for each factor are added up to determine the grade for the cancer. Higher-grade cancers tend to grow and spread faster than lower-grade cancers.
GX: the grade cannot be assessed (because of incomplete information).
Grade 1 (G1): Total score of 2 or 3
Grade 2 (G2): Total score of 4 or 5
Grade 3 (G3): Total score of 6 or higher
Tumor (T)
T1: The sarcoma is 5 cm (2 inches) or less across
T1a: The tumor is superficial − near the surface of the body.
T1b: The tumor is deep in the limb or abdomen.
T2: The sarcoma is greater than 5 cm across.
T2a: The tumor is superficial − near the surface of the body.
T2b: The tumor is deep in the limb or abdomen.
Lymph nodes (N)
N0: The sarcoma has not spread to nearby lymph nodes.
N1: The sarcoma has spread to nearby lymph nodes.
Metastasis (M)
M0: No distant metastases (spread) of sarcoma are found.
M1: The sarcoma has spread to distant organs or tissues (such as the lungs).
Stage grouping for soft tissue sarcomas
To assign a stage, information about the tumor, its grade, lymph nodes, and metastasis is combined by a process called stage grouping. The stage is described by Roman numerals from I to IV with the letters A or B. The stage is useful in selecting treatment, but other factors, like where the sarcoma is located, also impact treatment planning and outlook.
Stage IA
T1, N0, M0, G1 or GX: The tumor is not larger than 5 cm (2 inches) across (T1). It has not spread to lymph nodes (N0) or more distant sites (M0). The cancer is grade 1 (or the grade cannot be assessed).
Stage IB
T2, N0, M0, G1 or GX: The tumor is larger than 5 cm (2 inches) across (T2). It has not spread to lymph nodes (N0) or more distant sites (M0). The cancer is grade 1 (or the grade cannot be assessed).
Stage IIA
T1, N0, M0, G2 or G3: The tumor is not larger than 5 cm (2 inches) across (T1). It has not spread to lymph nodes (N0) or more distant sites (M0). The cancer is grade 2 or 3.
Stage IIB
T2, N0, M0, G2: The tumor is larger than 5 cm (2 inches) across (T2). It has not spread to lymph nodes (N0) or more distant sites (M0). The cancer is grade 2.
Stage III:
Either
T2, N0, M0, G3: It is larger than 5 cm (2 inches) across (T2). It has not spread to lymph nodes (N0) or more distant sites (M0). The cancer is grade 3.
OR
Any T, N1, M0, any G: The cancer can be any size (any T) and any grade. It has spread to nearby lymph nodes (N1). It has not spread to distant sites (M0).
Stage IV
Any G, Any T, Any N, M1: The tumor has spread to lymph nodes near the tumor (N1) and/or to distant sites (M1). It can be any size (any T) and grade (any G).
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