SarcomaLast Updated: October 01, 2009 This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/09 Overview
Soft tissue sarcoma is cancer that develops in the tissues that support and connect the body. It can occur in fat, muscle, nerves, tendons, joints, blood vessels, or lymph vessels. Cancer begins when cells grow out of control, and instead of developing into normal cells, they form a tumor or mass (lump). When sarcoma is small, it can go unnoticed or is ignored, since it does not usually cause problems at this stage. As sarcoma grows, it can interfere with the body's normal activities; it can also spread to other places in the body. This section covers sarcoma of the soft tissue. For information on sarcoma that starts in a bone, read the Cancer.Net Guide to Bone Cancer. Sarcoma can appear in any part of the body. About 60% occur in the arms or legs, 30% occur in the trunk or abdomen, and 10% occur in the head or neck. Sarcoma is rare, accounting for about 1% of all cancers. Because there are several different types of soft tissue sarcoma, it is probably best considered as a family of related diseases, rather than a single, specific disease. The specific types of sarcoma are frequently named according to the normal tissue cells they most closely resemble (Table 1), rather than referring to an anatomical site (part of the body) as many other cancer types are named. Table 1: Types of Sarcoma
Grade is the term a pathologist (a doctor who specializes in interpreting laboratory tests and diagnosing disease) uses to describe how aggressive the sarcoma is likely to be. A low-grade tumor usually stays in one place. A high-grade tumor is more likely to spread to other places, a process known as metastasis. Experts have identified many types and subtypes of sarcoma. Pathologists are now trying to find new ways to quickly determine a tumor's subtype, as this helps determine treatment. Examination of a tumor's abnormal genetic code may help determine its characteristics and predict which treatments will be most effective. For at least one type of sarcoma, gastrointestinal stromal tumor (GIST), major advances have been made in targeted therapy (drug treatments targeted to specific genetic abnormalities in the sarcoma cell). Statistics In 2009, approximately 10,660 people (5,780 males and 4,880 females) will be diagnosed with soft tissue sarcoma in the United States. An estimated 3,820 adults and children (1,960 males and 1,860 females) are expected to die of the disease this year. The overall five-year relative survival rate (percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases), combining all stages and types of sarcoma, is approximately 50%. If the sarcoma is diagnosed at an early stage and hasn’t spread from the original location of the tumor, the five-year survival rate is 83%. The five-year survival rate for cancer that has spread to the lymph nodes is 54%, and the five-year survival rate is 16% if cancer has spread to other parts of the body. However, if the sarcoma is located in an arm or leg, the five-year survival rates are slightly higher for each stage. Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of cases of this type of cancer, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with sarcoma. Because the survival statistics are measured in five-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer. Statistics adapted from the American Cancer Society's publication, Cancer Facts & Figures 2009, and from data from the National Cancer Institute and Memorial Sloan-Kettering Cancer Center. Find out more about basic cancer terms used in this section. Medical Illustrations
Risk Factors
A risk factor is anything that increases a person’s chance of developing cancer. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and communicating them to your doctor may help you make more informed lifestyle and health care choices. Most sarcomas do not have known causes. The following factors can raise a person’s risk of developing sarcoma:Previous radiation therapy. People who have been treated with radiation therapy for a previous cancer have a slightly increased risk of later developing sarcoma. Genetics. People with certain inherited diseases, such as von Recklinghausen’s disease (neurofibromatosis), Gardner syndrome, Werner syndrome, tuberous sclerosis, nevoid basal cell carcinoma syndrome, Li-Fraumeni syndrome, or retinoblastoma are at greater risk for sarcoma. Chemicals. Workplace exposure to vinyl chloride monomer (used in making some types of plastics) or to dioxin may increase the risk of sarcoma. However, most sarcoma is not known to be associated with specific environmental hazards. Symptoms
People with sarcoma may experience the following symptoms. Sometimes, people with sarcoma do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom, please talk with your doctor. Soft tissue sarcoma rarely causes symptoms in the early stages. The first sign of a sarcoma in the limbs or trunk may be a painless lump or swelling, which should be reported to a doctor. Most lumps are not sarcoma. The most common soft tissue lumps are lipomas, which are made of fat cells and are not cancer. These lumps have often been present for many years and do not change in size. Lumps that begin changing in size, however, need to be brought to the attention of a doctor. People with sarcoma that starts in the abdomen may not have any symptoms or may have pain or a sense of fullness. Because soft tissue sarcoma develops in flexible, elastic tissues, the tumor can often easily push normal tissue out of its way as it grows. Therefore, a sarcoma may grow quite large before it causes symptoms. Eventually, it may cause pain or soreness as the growing tumor begins to press against nerves and muscles. Diagnosis
Doctors use many tests to diagnose cancer and determine if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of cancer, including sarcoma, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test:
There are no standard screening tests for sarcoma. A doctor should examine any unusual or new lumps or bumps that are growing to make sure it is not cancer. Sarcoma is rare. This makes it important to consult a doctor who has experience with this type of cancer as soon as there is a possibility that it might be sarcoma. A diagnosis of sarcoma is made by a combination of clinical examination and imaging tests. It is confirmed by the results of a biopsy, a procedure in which a small amount of tissue is removed for examination under a microscope. In addition to a physical examination, the following tests may be used to diagnose sarcoma: Imaging tests A benign (noncancerous) and malignant (cancerous) tumor may look different on imaging tests. In general, a benign tumor has round, smooth, well-defined borders. A malignant tumor has irregular, poorly defined margins due to its aggressive growth. X-ray. An x-ray is a picture of the inside of the body. For instance, a chest x-ray can help doctors determine if the cancer has spread to the lungs. Typically, if an x-ray suggests cancer, the doctor will order other imaging tests. X-ray is particularly useful for bone sarcomas. Ultrasound. An ultrasound uses sound waves to create a picture of the internal organs. Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail. Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture. MRI scans are useful to check for tumors in soft tissues nearby. Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive substance is injected into a patient’s body and absorbed by the organs or tissues being studied. This substance gives off energy that is detected by a scanner, which produces the images. Integrated PET-CT scan. This scanning method collects images both from CT and PET scans at the same time, and then combines the images. This technique has the advantage of looking at both the structure and metabolism of the tumor and normal tissues. This information can be helpful in treatment planning and evaluating treatment benefit. Imaging tests may suggest the diagnosis of sarcoma, but a biopsy will always be performed to confirm the diagnosis and determine the subtype. It is vitally important for a patient to be seen by a sarcoma specialist before any surgery or biopsy is performed. Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). For a needle biopsy (usually a core needle biopsy, less often a thin needle biopsy), a doctor removes a small sample of tissue from the tumor with a needle-like instrument. This may be performed with the help of ultrasound, CT scan, or MRI to precisely locate the site of the tumor. In an incisional biopsy, the surgeon cuts into the tumor and removes a sample of tissue. In an excisional biopsy, the surgeon removes the entire tumor. Because these tumors are uncommon, it is important that the biopsy is reviewed by an expert pathologist to appropriately diagnose a sarcoma. Learn more about what to expect when having common tests, procedures, and scans. Find out more about common terms used during a diagnosis of cancer. Staging
Staging is a way of describing a cancer, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of cancer. There are a number of methods that are used to determine the stage of sarcoma. These systems differ somewhat, but all rely on overall stage to predict prognosis. One tool that doctors use to describe the stage is the TNM system. This system uses three criteria to judge the stage of the cancer: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to the rest of the body. The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero) and stages I through IV (one through four). The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments. TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:
When staging sarcoma, two other factors are added to the TNM system: depth (D) and grade (G). This is different than staging for many other types of cancer. Tumor. Using the TNM system, the "T" plus a number (0 to 2) and letter (a or b) is used to describe the size and location of the tumor. Some stages are also divided into smaller groups that help describe the tumor in more detail. Specific tumor stage information is listed below. Depth (D) helps describe the tumor (T). It is described as either superficial (the tumor is above the body's superficial tissue) or deep (the tumor is either beneath the body's superficial tissue or invades the deep tissue). TX: The primary tumor cannot be evaluated. T0: There is no evidence of a primary tumor. T1:The size of the tumor is 5 centimeters (cm) or smaller. T1a: The tumor is superficial. T1b: The tumor is deep. T2: The size of the tumor is larger than 5 cm. T2a: The tumor is superficial. T2b: The tumor is deep. Node. The "N" in the TNM staging system stands for lymph nodes, the tiny, bean-shaped organs located all over the body that normally help fight infections and cancer as part of the body's immune system. Each type of tumor drains into lymph nodes nearby called regional lymph nodes. As noted above, lymph node involvement is unusual for soft tissue sarcoma. NX: The regional lymph nodes cannot be evaluated. N0: The cancer has not spread to the regional lymph nodes. N1: The cancer has spread to the regional lymph nodes. Metastasis. The "M" in the TNM system indicates whether the cancer has spread to other parts of the body. MX: Metastasis cannot be evaluated. M0: The cancer has not metastasized. M1: There is metastasis to another part of the body. Histologic grade (G). Histologic grade describes how different the cancer cells are from normal tissue cells when examined under a microscope. A tumor's grade is described using the letter “G” and a number. The following is a commonly used grading system, but there are others that may alternatively be used. For instance, some doctors grade sarcomas as either “low grade” or “high grade”. GX: The tumor grade cannot be identified. G1: Describes cells that look more like normal tissue cells (well differentiated). G2: The cells are somewhat different (moderately differentiated). G3: The cells look very different from normal cells (poorly differentiated). G4: The cells barely resemble normal cells (undifferentiated). Cancer stage grouping Doctors assign the stage of the cancer by combining the T, N, and M classifications. For sarcoma, stages I and II are described in smaller groups according to the tumor's depth (D). Stage I: The tumor is low grade (G1), small, and either superficial or deep (T1a, T1b, T2a, T2b). It has not spread to lymph nodes or to other parts of the body (N0, M0). Stage II: The tumor is high grade (G2, G3, or G4), small or superficial (T1a or T2a). It has not spread to lymph nodes or to other parts of the body (N0, M0). Stage III: The tumor is high grade (G3 or G4), large, and deep (T2b). It has not spread to lymph nodes or to other parts of the body (N0, M0). Stage IV: The tumor has spread to lymph nodes (N1) or other parts of the body (M1). The tumor can be any size (T1 or T2) and any grade (G1, G2, G3, or G4). This staging system applies to all types of soft tissue sarcoma except Kaposi's sarcoma, dermatofibrosarcoma, and desmoid type of fibrosarcoma grade 1. Also excluded from this staging system is sarcoma that begins in the dura mater (the membrane lining the brain), including the brain, and in solid organs and hollow viscera, such as the bowel or bladder. Recurrent: Recurrent cancer is cancer that comes back after treatment. Used with permission of the American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Manual, Sixth Edition (2002) published by Springer-Verlag New York, www.cancerstaging.net. Treatment
The treatment of sarcoma depends on the size and location of the tumor, its grade, its subtype, whether the cancer has spread, and the person’s overall health. In many cases, a team of doctors will work with the patient to determine the best treatment plan. This section outlines treatments that are the standard of care (the best treatments available) for this specific type of cancer. Patients are also encouraged to consider clinical trials when making treatment plan decisions. A clinical trial is a research study to test a new treatment to prove it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, visit the Clinical Trials section. Surgery is the most common treatment for sarcoma. If the tumor cannot be removed by surgery, it may be permanently controlled with radiation therapy. For a tumor that can be surgically removed, radiation therapy and/or chemotherapy may be given before or after surgery to reduce the risk of recurrence (cancer coming back after treatment). Chemotherapy and radiation therapy may also be used to reduce the size of the sarcoma or relieve pain and other symptoms. Descriptions of the most common treatment options for sarcoma are listed below. Surgery Surgery is typically the main treatment for soft tissue sarcoma. A surgical oncologist is a doctor who specializes in treating cancer using surgery. The surgeon's goal is to remove the tumor and at least 1 cm to 3 cm (about 1 inch) of healthy tissue around it, to leave behind a clean margin. Small sarcomas can usually be cured by surgery alone, but those larger than 5 cm are usually treated with a combination of surgery and radiation therapy. Radiation therapy or chemotherapy may be used before surgery (to shrink the tumor and make removal easier), or during and after surgery (to kill any remaining cancer cells). Learn more about cancer surgery. Chemotherapy Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. Some people may receive chemotherapy in their doctor’s office; others may go to the hospital. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a specific time. Most chemotherapy drugs are given by injection into a vein (called intravenous or IV injection). Some types of chemotherapy that might be used alone or in combination include doxorubicin (Adriamycin, Rubex), ifosfamide (Ifex), gemcitabine (Gemzar), docetaxel (Taxotere), dacarbazine (Matulane), paclitaxel (Taxol), as well as some targeted therapies (see below) called imatinib (Gleevec) (for GIST and a rare sarcoma called dermatofibrosarcoma protuberans), and sunitinib (Sutent; for GIST). Trabectedin (Yondelis) was recently approved in European countries for patients with sarcoma that continued to grow after initial chemotherapy. The specific drugs used to treat sarcoma depend on what subtype of sarcoma it is. Different drugs are used to treat different types and subtypes of sarcoma. Chemotherapy for sarcoma can usually be given as an outpatient treatment. Most side effects of the drugs go away within a short time after treatment. Chemotherapy is often useful in cases in which a cancer has already metastasized. A fast-growing sarcoma can be treated with chemotherapy before surgery. This often reduces the size of the main tumor and may destroy tiny areas of metastasis if some of the cancer cells have already drifted into other areas. For large high-grade sarcoma, where surgery may not be possible or is problematic, the doctor may recommend giving chemotherapy for three to four cycles before surgery to shrink the primary tumor, so it is more easily removable. Some chemotherapy before surgery may also improve survival since it may kill cells that have broken away from the original tumor. Chemotherapy may be given alone or in combination with surgery and/or radiation therapy. Chemotherapy given before surgery is called preoperative chemotherapy, neoadjuvant chemotherapy, or induction chemotherapy. Chemotherapy given after surgery is called adjuvant chemotherapy or postoperative chemotherapy. After the patient has recovered from surgery, the oncologist may give more chemotherapy to kill any remaining tumor cells. Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases. Radiation therapy Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. Radiation therapy may be done before surgery to shrink the tumor, so it is more easily removed. Or, it may be done after surgery to remove any cancer cells left behind. Radiation treatment may make it possible to do less surgery, often preserving the arm or leg when that is where the sarcoma is located. Radiation therapy can also damage normal cells, but because it is focused around the tumor, side effects occur mainly in those areas. Most radiation therapy side effects go away soon after treatment ends. Newer radiation techniques, including intensity modulated radiation therapy and proton beam radiation (see Current Research), may help control sarcoma, as well as reduce the frequency of shorter-term and longer-term side effects. Intensity modulated radiation therapy (IMRT). IMRT is a way to better direct radiation therapy at the tumor and reduce the risk of harming the bone or other nearby tissues. Targeted therapy Targeted therapy is a treatment that targets faulty genes or proteins that contribute to cancer growth and development. Therefore, it is much more specific for the treatment of the cancer, usually with fewer and less severe side effects. In 2002, the U.S. Food and Drug Administration (FDA) approved imatinib for the treatment of gastrointestinal stromal tumor (GIST) in advanced stages. This drug is now the standard treatment for GIST. In 2006, a second targeted therapy, sunitinib, was approved for the treatment of GIST, for patients whose GIST is not treatable with imatinib. More recently, imatinib has been approved for use in GIST patients after initial surgery, to try to prevent a recurrence. In addition, imatinib is approved for the treatment of patients with advanced stage of a rare type of sarcoma called dermatofibrosarcoma protuberans. New targeted drugs are being tested in clinical trials. Read more about targeted treatments. Advanced and recurrent sarcoma An advanced sarcoma is one that has spread to other sites at the time of diagnosis or recurred after initial treatment. The recurrence may be in the tissue where the sarcoma first appeared (local recurrence) or in another place (regional or distant recurrence). About one-third of patients treated for soft tissue sarcoma of the arm or leg have a recurrence of the tumor, most likely in the lungs. More than half of those treated for sarcoma of the abdomen or trunk will have some type of recurrence, which can be local, regional, and/or distant. Treatment of the recurrence will depend on the location and type of recurrence and on the method of previous treatment. An isolated local recurrence is usually treated with additional surgery, if possible, plus radiation therapy. Treatment of sarcoma that has spread to other organs or lymph nodes may include surgery alone, surgery plus radiation therapy, surgery plus chemotherapy, chemotherapy alone and also, in the rare cases when the tumor is not growing, a “watch and wait” policy, which is also called active surveillance. Chemotherapy combinations are often used for advanced soft tissue sarcoma. In rare instances, generally in patients with a very large tumor involving the major nerves and blood vessels of the arm or leg, amputation is necessary to control the tumor. This can also be necessary if the tumor grows back in the arm or leg after prior surgery, radiation therapy, and/or chemotherapy. People who have been treated for sarcoma should have regular follow-up examinations to watch for recurrence, based on a schedule set up by their oncologist (see After Treatment). Find out more about common terms used during cancer treatment.Clinical Trials Resources
Doctors and scientists are always looking for better ways to treat patients with sarcoma. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. The clinical trial may be evaluating a new drug, a new combination of existing treatments, a new approach to radiation therapy or surgery, or a new method of treatment or prevention. Patients who participate in clinical trials are among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment. Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that finding new drugs and other therapies is the only way to make progress in treating sarcoma. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with sarcoma. Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill”. The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials. To join a clinical trial, patients must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials. For specific topics being studied for sarcoma, learn more in the Current Research section. Side Effects
Cancer and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of cancer treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur. Fear of treatment side effects is common after a diagnosis of cancer, but it may be helpful to know that preventing and controlling side effects is a major focus of your health care team. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of cancer, its location, the individual treatment plan (including the length and dosage of treatment), and the person’s overall health. Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health care team if they do happen. Also, be sure to communicate with the doctor about side effects you experience during and after treatment. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Learn more about the importance of addressing such needs, including concerns about managing the cost of your medical care. Learn more about late effects or long-term side effects by reading the After Treatment section or talking with your doctor. After Treatment
After treatment for sarcoma ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. Regularly scheduled follow-up visits with the doctors involved in the sarcoma treatment is not only important to detect any possible tumor recurrence, but also help to manage and hopefully prevent some side effects related to treatment. A commonly used follow-up schedule includes visits every three to four months for the first three years after treatment, and then every six months until five years after the completion of treatment, and then annually thereafter. Periodic chest x-rays or CT scans will be done to detect possible later spread of cancer to the lungs during these follow-up visits. Imaging of the site of the original tumor with MRI, ultrasound, CT scan, and/or PET scan is also sometimes performed. People who have been treated for soft tissue sarcoma should be particularly careful to report any new symptoms, such as a cough, new onset of pain, or a new lump. It may be a sign of a cancer recurrence, a side effect of treatment, or a problem unrelated to cancer. If a recurrence happens, it is likely to be within the first two years, but some sarcomas recur much later. Local recurrences (recurrences at the site of the original tumor) can often be successfully treated. Metastases (recurrences at sites away from the original site) are treatable, but rarely curable. For people who received radiation therapy, the region of the body that received radiation therapy can be at risk for limb swelling (lymphedema), fracture of the thigh or leg bones, reduction in mobility of joints, and fibrosis (hardness) of the soft tissues. In rare instances, another sarcoma that is different from the original tumor may develop as a result of radiation therapy. Limb swelling can be managed with compression stockings and other special therapies that can be prescribed by doctors. Bone fractures may be prevented by avoiding certain high-impact exercises, which patients should discuss with their doctors. Joint mobility can be improved with a rehabilitation program. Fibrosis may respond to several months of treatment with a combination of vitamin E and pentoxifylline, another oral medication. Skin that was subject to radiation therapy should be protected from sun exposure with clothing or sunscreen regularly to reduce the chance of a skin cancer developing in that area. A rehabilitation program after surgery or radiation therapy can be important to help patients treated for sarcoma in the arm or leg regain or maintain maximum limb function. Range-of-motion exercises, strengthening exercises, and a program to reduce limb swelling if present may be prescribed. A formal consultation with a rehabilitation medicine specialist may be extremely important to help optimize the rehabilitation of the patient after treatment. The majority of patients with a sarcoma in an arm or leg can be successfully treated and do maintain good limb function. However, even when the treatment included amputation, access to prosthetic services (artificial limbs) and mental health support can help manage the adjustment to life following amputation. Learn more about rehabilitation . People recovering from sarcoma are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about healthy living after cancer. Find out more about common terms used after cancer treatment is complete. Current Research
Research for sarcoma is ongoing. The following advances may still be under investigation in clinical trials and may not be approved or available at this time. Always discuss all diagnostic and treatment options with your doctor. Proton or particle beam radiation therapy. Proton or particle beam radiation therapy is a form of radiation therapy that uses particles (protons or other charged particles) that travel only the desired distance in tissue, allowing doctors to target the radiation much more precisely at the tumor and reduce unwanted radiation affecting normal tissues. This procedure is currently only available in a few specialized cancer centers in the United States. Learn more about proton therapy. Brachytherapy as outpatient treatment. Doctors are exploring a way to give brachytherapy (internal radiation therapy) as an outpatient procedure. Traditionally, patients stayed in the hospital while the radioactive seeds were in place. This newer procedure would allow the patients to go home during treatment. Isolated limb perfusion (ILP). Isolated limb perfusion is used to treat patients with soft tissue sarcoma that is limited to the arm or leg. Blood flow to the affected arm or leg is temporarily separated from the rest of the body, and high doses of chemotherapy are given directly to the limb. The chemotherapy most commonly used with isolated limb perfusion is melphalan (Alkeran), sometimes in combination with a type of drug called a tumor necrosis factor; neither drug is standard chemotherapy for sarcoma. This procedure is currently only available in a few specialized cancer centers in the United States. New drugs. New chemotherapy is being developed and tested that may be effective in treating some subtypes of soft tissue sarcoma. An example is trabectedin, which was approved in Europe for the treatment of soft tissue sarcoma. However, in the United States, FDA only allows patients access to it under special circumstances. Learn more about drug development and approval. Targeted therapy. As the biological processes that lead to a cell becoming cancerous are better understood, drugs designed to specifically target these processes are being developed. These drugs have the potential to be both less toxic and more effective than current therapies. As explained in the Treatment section, imatinib mesylate and sunitinib malate, a class of drugs called tyrosine kinase inhibitors, are the first examples of such drugs approved for treatment of specific types of sarcoma, most commonly GIST; a number of other tyrosine kinase inhibitors are being studied in clinical trials. Another targeted therapy, ridaforolimus, is currently being studied in a phase III clinical trial for people with sarcoma that has spread to other parts of the body. Ridaforolimus is a mTOR inhibitor, meaning that it blocks the protein mTOR that participates in cell growth activity. A number of clinical trials are being done with another class of targeted therapies, called insulin-like growth factor receptor (IGFR) inhibitors. The IGFR is an important growth protein for sarcomas. Inhibiting its activity may be an important new way to improve sarcoma therapy. Early results look promising, especially for patients with Ewing sarcoma, but the clinical trials are still ongoing. Targeted oncogene treatments. Drugs are being researched that may inhibit the function of one or more of the proteins found in tumor cells that drive cell survival and division. Genetics. Researchers are learning that some sarcomas have unique genetic “fingerprints.” Understanding these fingerprints may help doctors determine better treatment and possibly better predict a patient’s prognosis. Angiogenesis inhibitors. Drugs that prevent angiogenesis (the formation of new blood vessels) starve the tumor of oxygen and nutrients. Improved drug delivery. Some chemotherapy drugs are incorporated into fat molecules called liposomes to improve the absorption and distribution of the drug in the patient’s body. Questions to Ask the Doctor
Regular communication with your doctor is important in making informed decisions about your health care. Consider asking the following questions of your doctor: General questions:
Questions to ask before surgery:
Questions to ask before radiation therapy:
Questions to ask before chemotherapy:
After treatment:
Patient Information Resources
In addition to Cancer.Net, there are other sources of information about this type of cancer available online. Cancer.Net maintains a list of national, not-for-profit organizations that may be helpful in finding additional information, services, and support. As always, be sure to talk with your doctor about questions you may have about information you find about this disease. View organizations that offer information on this specific type of cancer. |
