SarcomaLast Updated: July 18, 2011 This section has been reviewed and approved by the Cancer.Net Editorial Board, 10/10 Overview
Cancer begins when cells begin to change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). About sarcoma Both children and adults can develop a sarcoma, and it can start in any part of the body, such as the bone or soft tissue. About 60% begin in the arms or legs, 30% start in the trunk or abdomen, and 10% are in the head or neck. Sarcoma is rare in adults, accounting for about 1% of all adult cancers. However, sarcoma in general represents about 15% of all cancers in children. This section covers sarcoma of the soft tissues. Learn more about sarcoma that starts in a bone or a type of soft-tissue sarcoma called gastrointestinal stromal tumor (GIST) that affects the gastrointestinal tract. About soft-tissue sarcoma Soft-tissue sarcomas (STS) are a group of cancers that begin in the tissues that support and connect the body. STS can occur almost anywhere in the body. The sarcoma cells resemble the cells that hold the body together, including fat cells, muscle, nerves, tendons, joints, blood vessels, or lymph vessels. When an STS is small, it can go unnoticed, since it does not usually cause problems early on. As an STS grows, it can cause problems with the body's normal activities. Because there are several different types of STS, it is more of a family of related diseases, rather than a single, specific disease. The specific types of sarcoma are often named according to the normal tissue cells they most closely resemble (see below). This is different from most other types of cancer, which are usually named by the part of the body where the cancer began.
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, meaning how likely it will grow and spread to another part of the body. A low-grade tumor is usually less likely to spread quickly, and it often stays in the place where it started. A high-grade tumor is more likely to spread to other places, a process called metastasis. Learn more about grade in the Staging section. Experts have found many types and subtypes of sarcomas. Pathologists are now trying to find new ways to quickly determine a tumor's subtype, as this helps determine treatment. Looking at a tumor's abnormal genetics may help determine its characteristics and predict which treatments will be most effective. For at least two types of sarcoma, GIST and dermatofibrosarcoma protuberans (DFSP), major advances have been made in a type of treatment called targeted therapy (see Treatment). Find out more about basic cancer terms used in this section. Looking for More of an Overview? If you would like additional introductory information, explore these related items on Cancer.Net:
Or, choose “Next” (below, right) to continue reading this detailed section. Statistics
This year, approximately 10,980 people (6,050 males and 4,930 females) will be diagnosed with soft-tissue sarcoma in the United States. An estimated 3,920 adults and children (2,060 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%. However, when the sarcoma is located in an arm or leg, the five-year survival rates are slightly higher for each stage.How long a person lives with sarcoma depends on many factors, including the size of the tumor, where it is located, the type, and whether it is superficial or deep (see Staging). If the sarcoma is diagnosed at an early stage and hasn’t spread from where it started, treatment is very effective and many people can be cured. On the other hand, if the sarcoma has spread to other parts of the body, treatment can usually control the tumor, but it is not often curable. Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of people with 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. Learn more about understanding statistics. Statistics adapted from the American Cancer Society's publication, Cancer Facts & Figures 2011. Medical Illustrations
Risk Factors
A risk factor is anything that increases a person’s chance of developing cancer. 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 have a higher risk of 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 or signs. 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 or sign, please talk with your doctor. STS rarely causes symptoms in the early stages. The first sign of a sarcoma in the arms, legs, or trunk may be a painless lump or swelling. 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 there for many years and do not change in size. It’s important to talk to your doctor about any lumps that begin changing in size. People with sarcoma that starts in the abdomen may not have any symptoms or may have pain or a sense of fullness. Because STS can develop in flexible, elastic tissues or deep spaces in the body, 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 find out 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 (a procedure in which a small amount of tissue is removed for examination under a microscope) 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 talk with 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. In addition to a physical examination, the following tests may be used to diagnose sarcoma: Imaging tests A benign and malignant tumor may look different on imaging tests, such as an x-ray. In general, a benign tumor has round, smooth, well-defined borders. A malignant tumor has irregular, poorly defined edges because of 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. This substance is absorbed mainly by organs and tissues that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body. 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 can be used to look at both the structure and how much energy is used by the tumor and normal tissues. This information can be helpful in treatment planning and evaluating how well treatment is working. Imaging tests may suggest the diagnosis of sarcoma, but a biopsy will always be performed to confirm the diagnosis and find out the subtype. It is very important for a patient to see a sarcoma specialist before any surgery or biopsy is done. 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). There are different types of biopsies. 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 find 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 sample of tissue removed 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 systems used to find out the stage of sarcomas. These systems are somewhat different from each other, but all use an overall stage to help predict a person’s prognosis. One tool that doctors use to describe the stage of common cancers is known as 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, another factor, grade (G), is added to the TNM system. 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. The tumor (T) is further described by its depth. The tumor depth 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). Specific tumor stage information is listed below. 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. M0: The cancer has not metastasized. M1: There is metastasis to another part of the body. Grade (G). The grade for sarcoma describes how aggressive a tumor is and how likely it is to grow and spread. A tumor's grade is described using the letter “G” and a number. There are four grades for sarcoma: GX (the grade cannot be evaluated), G1, G2, and G3. The grade is determined by calculating a score based on the following factors:
The lower the combined score for these three factors, the lower the grade, meaning that the tumor is less aggressive and a patient’s prognosis is better. This is the recommended grading system, but there are others that may also be used. For instance, some doctors grade sarcomas as either “low grade” or “high grade,” particularly if the tumor has been treated before surgery. 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. Stage IA: The tumor is 5 cm or less in size, and either superficial or deep. It has not spread to lymph nodes or to other parts of the body (T1a or T1b, N0, M0, GX or G1). Stage IB: The tumor is more than 5 cm in size, and either superficial or deep. It has not spread to lymph nodes or to other parts of the body (T2a or T2b, N0, M0, GX or G1). Stage IIA: The tumor is 5 cm or less in size, and either superficial or deep. It has not spread to lymph nodes or to other parts of the body. The grade is higher than the grade for stage IA (T1a or T1b, N0, M0, G2 or G3). Stage IIB: The tumor is more than 5 cm in size, and either superficial or deep. It has not spread to lymph nodes or to other parts of the body. The grade is higher than the grade for stage IB (T2a or T2b, N0, M0, G2). Stage III: This stage can be described by either of the following:
Stage IV: The tumor is any size, either superficial or deep, any grade, and may or may not have spread to the regional lymph nodes. It has spread to other parts of the body (any T, any N, M1, and any G). This staging system applies to all types of soft tissue sarcoma except Kaposi sarcoma, GIST, and infantile fibrosarcoma. Also excluded from this staging system are sarcomas that begin in the central nervous system (including the brain or the lining around the brain and spinal cord), and sarcomas that begin in an organ or a hollow organ, such as the bowel or urinary tract. 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, Seventh Edition (2010) published by Springer-Verlag New York, www.cancerstaging.net. Treatment
The treatment of a soft tissue sarcoma depends on the specific subtype of sarcoma, in addition to other factors such as the size and location of the tumor, its grade, whether the cancer has spread, and the person’s overall health and personal preferences. In many cases, a team of doctors will work with the patient and family to determine the best treatment plan for that individual. 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 evaluate whether it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, see the Clinical Trials section. Surgery is the most common first treatment for sarcomas that are smaller and in specific locations. If the tumor cannot be removed by surgery, it may be possible to permanently control its growth 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 Before surgery, it’s important to have a biopsy to confirm the diagnosis (see Diagnosis). After a biopsy, surgery is typically the first and main treatment for an STS that is smaller and located in only one area. A surgical oncologist is a doctor who specializes in treating cancer using surgery. Sometimes, when an STS is on a limb (arm or leg) or spine, orthopedic oncologists may perform surgery to remove the tumor. The surgeon's goal is to remove the tumor and enough normal tissue around it to leave behind what is called a “clean” margin (meaning there are no tumor cells left in the area where the tumor was removed). Small sarcomas can usually be cured by surgery alone. Those larger than 5 cm are often 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. 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. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a specific time. 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 if 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 and cause fewer shorter-term and longer-term side effects. Brachytherapy as outpatient treatment. Doctors are often now able to give brachytherapy as an outpatient procedure. Traditionally, patients stayed in the hospital while the radioactive seeds were in place. This newer procedure uses specialized equipment that can painlessly insert the radiation seeds and remove them after about 15 minutes one or two times a day. This may allow patients to go home during treatment, or if the patient needs to be in the hospital while they are still recovering from the surgery, the patient will not need to be in an isolated, shielded room because the patient will be free of radioactivity for most of the day. This means many patients can return home if medically able or, if still hospitalized, can enjoy visits by family and friends without any concern about radiation exposure. Learn more about radiation therapy. Chemotherapy Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, which can reach sarcoma cells throughout the body wherever they may be located. 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 or outpatient clinic; others may go to the hospital. A chemotherapy regimen 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). Different drugs are used to treat different types and subtypes of sarcoma. Chemotherapy for sarcoma can usually be given as an outpatient treatment. Some types of chemotherapy that might be used alone or in combination for STS include:
The specific drugs used to treat sarcoma depends the subtype of sarcoma. Some chemotherapy drugs are only used for certain subtypes of sarcoma. For angiosarcoma:
For rhabodomyosarcoma and extraskeletal (meaning it begins outside of the bones) Ewing sarcoma:
Chemotherapy is often used when a sarcoma has already spread. In addition, certain types of sarcoma might be treated with chemotherapy before surgery to more easily remove the tumor with surgery. Chemotherapy may be given alone or in combination with surgery and/or radiation therapy. Chemotherapy given before surgery may be called by different names, including preoperative chemotherapy, neoadjuvant chemotherapy, or induction chemotherapy. If a patient has not received chemotherapy before surgery, chemotherapy may be given to destroy any microscopic tumor cells that remain after a patient has recovered from surgery. Chemotherapy given after surgery is called adjuvant chemotherapy or postoperative chemotherapy. Side effects vary depending on the drug and the dose. Talk with your doctor about the potential side effects, how long they may last, and how they can be relieved. 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. Targeted therapy Targeted therapy is a treatment that targets specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. 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 (Gleevec) for the treatment of GIST in advanced stages. This drug is now the standard first-line treatment for GIST worldwide. In 2006, a second targeted therapy, sunitinib (Sutent), was approved for the treatment of GIST when imatinib doesn’t work. More recently, imatinib has been approved for use for patients with GIST after initial surgery, to try to prevent recurrence in patients who might have a high risk of recurrence. In addition, imatinib is approved for the treatment of patients with advanced stage dermatofibrosarcoma protuberans (DFSP). Trabectedin was approved in 2007 outside of the United States for patients with sarcoma when conventional chemotherapy failed to control the disease. Many other new targeted therapies are being tested in clinical trials; learn more about current research. Read more about targeted treatments. Advanced sarcoma An advanced sarcoma is one that has spread to other sites at the time of diagnosis, recurred after initial treatment, or is not possible to remove with surgery alone. Treatment of sarcoma that has spread may include surgery alone, surgery plus radiation therapy, surgery plus chemotherapy, or chemotherapy alone. Rarely, when the tumor is not growing, a “watch and wait” (also called active surveillance) approach may be used, which means that the tumor is monitored and active treatment begins only if the tumor begins to grow. Chemotherapy combinations are often used for advanced soft tissue sarcoma. Rarely, for patients with a very large tumor involving the major nerves and blood vessels of the arm or leg, amputation (surgical removal of the limb) 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. It’s important to remember that the operation that results in the most useful and strongest limb may be different from the one that gives the most normal appearance. If amputation is needed, rehabilitation, including physical therapy, can help maximize the patient’s physical functioning. Rehabilitation can also help a person cope with the social and emotional effects of losing a limb. Recurrent sarcoma A recurrence may start in the tissue where the sarcoma first appeared (local recurrence) or in another place (regional or distant recurrence). 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 only often curable for patients who have a small number of tumors that have spread to the lung that can be completely removed surgically or destroyed with radiofrequency ablation (use of a needle inserted into the tumor to destroy the cancer with an electrical current) or focused, high-dose radiation therapy. If patients treated for STS of the arm or leg have a recurrence of the tumor, it will mostly likely recur in the lungs. Patients treated for sarcoma of the abdomen or trunk have a risk of some type of recurrence, which can be local, regional, and/or distant. Treatment of a recurrence depends on the location and type of recurrence and on the treatment the patient received for the original tumor. An isolated local recurrence is usually treated with additional surgery, if possible, plus radiation therapy. People who have been treated for sarcoma should have regular follow-up examinations to look for a possible 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 clinical trials. For specific topics being studied for sarcoma, learn more in the Current Research section. Side Effects
Sarcoma 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 your 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, you may experience 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. ASCO offers cancer treatment summary forms to help keep track of the cancer treatment you received and develop a survivorship care plan once treatment is completed. Regularly scheduled follow-up visits with the doctors involved in your treatment are important to find any possible tumor recurrence and to help 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 treatment, and then annually thereafter. Periodic chest x-rays or CT scans will be done to look for any possible spread of cancer to the lungs during these follow-up visits. Imaging tests of where the tumor began with MRI, ultrasound, CT scan, and/or PET scan is also sometimes performed. People who have been treated for STS should be particularly careful to talk with their doctor about any new symptoms, such as a cough, 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. 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, poor mobility of joints, and fibrosis (hardness) of the soft tissues. Rarely, 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 talk about with their doctors. Joint mobility can be improved with a rehabilitation program. Fibrosis may lessened with several months of treatment with a combination of vitamin E and pentoxifylline, another oral medication. Skin that received 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 limb function. Range-of-motion exercises, strengthening exercises, and a program to reduce limb swelling, if needed, may be recommended. Visiting a rehabilitation medicine specialist may be extremely important to help the patient receive the most appropriate rehabilitation 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, particularly when the treatment included amputation, prosthetic services (artificial limbs) and mental health support can help manage the adjustment to life following treatment. 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. New Forms of Radiation Therapy Proton or particle beam radiation therapy. Proton therapy (also called proton beam therapy) is a type of external-beam radiation therapy that uses protons rather than x-rays. At high energy, protons can destroy cancer cells. Protons travel only the desired distance in tissue, allowing doctors to target the radiation much more precisely at the tumor and lessen the amount of radiation to nearby healthy tissue. This procedure is currently only available in a few specialized cancer centers in the United States. Learn more about proton therapy. New Ways of Delivering Drugs Isolated limb perfusion (ILP). Isolated limb perfusion is used to treat patients with STS that is located only in an arm or leg. Blood flow to the 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. 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. New Drugs New chemotherapy is being developed and tested that may be effective in treating some subtypes of STS. An example is trabectedin, which was approved in Europe. However, in the United States, FDA only allows patients access to it under special circumstances. Researchers are working to find less toxic versions of standard chemotherapy used to treat sarcoma. An example is two new versions of ifosfamide, called palifosfamide and glufosfamide, being studied in ongoing clinical trials. Learn more about drug development and approval. Targeted therapy. Drugs are being designed to specifically target the processes that lead to cells becoming cancerous. These drugs are called targeted therapy and have the potential to be both less toxic and more effective than current therapies. As explained in the Treatment section, imatinib and sunitinib, types of drugs called tyrosine kinase inhibitors, are the first of such drugs approved to treat specific types of sarcoma, most commonly GIST. Other tyrosine kinase inhibitors are being studied in clinical trials. In particular, pazopanib (Votrient) and sorafenib (Nexavar) have been studied in several clinical trials. Research suggests that cediranib (Recentin) might be a very effective treatment for alveolar soft part sarcoma, and studies on this drug are ongoing. 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 an mTOR inhibitor, meaning that it blocks the protein mTOR that helps cells grow. Several clinical trials are being done with another type of targeted therapy, called insulin-like growth factor receptor (IGFR) inhibitors. The IGFR is an important growth protein for sarcomas. Blocking it from helping a tumor grow may be an important new way to improve sarcoma treatment. Early results look promising, especially for patients with Ewing sarcoma, but the clinical trials are still ongoing. Some research suggests that the combination of an mTOR inhibitor and an IGFR inhibitor may be more effective and clinical trials of this combination are planned. Other trials are looking at targeted therapies called angiogenesis inhibitors. These are drugs that prevent the formation of new blood vessels and starve the tumor of oxygen and nutrients needed for it to grow. Targeted oncogene treatments. Drugs are being researched that may block one or more of the proteins found in tumor cells that help the tumor grow and spread. 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. To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now. 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 or targeted therapy:
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. |
