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Depending on the site and stage of a sarcoma, surgery may be able to remove the cancer and some of the nearby tissue. The goal of surgery is to remove the entire tumor along with at least 1 to 2 cm (less than an inch) of the normal tissue surrounding the tumor. This is to make sure that no cancer cells are left behind. When the removed tissue is looked at under a microscope, the doctor will check to see if cancer is growing in the edges (margins) of the specimen. If cancer cells are present at the edges, the tissue removed is said to have positive margins. This means that cancer cells may have been left behind.
When cancer cells are left after surgery, the patient may need more treatment − such as radiation or another surgery. If cancer isn’t growing into the edges of the tissue removed, it is said to have negative or clear margins. The sarcoma has much less chance of coming back after surgery if it is removed with clear margins. When the tumor is in the abdomen, removing the tumor with enough normal tissue to get clear margins may be difficult because the tumor could be next to vital organs that can’t be taken out.
In the past, many of the sarcomas in the arms and legs were treated by amputating (removing) the limb. Now, amputation rarely is needed. Instead, most patients can be treated with surgery to remove the tumor without amputation (called limb-sparing surgery). This is usually followed by radiation therapy. These patients have the same overall survival rates as those who have amputations.
Sometimes, an amputation can’t be avoided. It might be the only way to remove all of the cancer. Other times, critical nerves, muscles, bone, and blood vessels would have to be removed along with the cancer. If removing this tissue would mean leaving a limb that can’t function well or result in chronic pain, amputation may be the best option.
If the sarcoma has spread to distant sites (such as the lungs or other organs), all of the cancer will be removed if possible. That includes the original tumor plus the areas of spread. If it isn't possible to remove all of the sarcoma, then surgery may not be done at all.
Sometimes chemotherapy (chemo), radiation, or both is given before surgery. This, called neoadjuvant treatment, can shrink the tumor and allow it to be removed completely. Chemo or radiation can also be given before surgery to treat high-grade sarcomas when there is a high risk of the cancer spreading.
Once a sarcoma has spread, surgery cannot cure it most of the time. But if it has only spread to the lung, the metastatic tumor can sometimes be removed. This can cure many patients, or at least lead to long-term survival.
Radiation therapy for soft tissue sarcomas
Radiation therapy uses high-energy rays (such as x-rays) or particles to kill cancer cells.
Most of the time radiation is given after surgery as an added measure. This, called adjuvant treatment, is done to kill any cancer cells that may be left behind after surgery.
Radiation may also be used before surgery to shrink the tumor and make the operation easier. This is called neoadjuvant treatment.
Radiation can be the main treatment for sarcoma in people whose general health is too poor to undergo surgery.
Radiation therapy can also be used to help symptoms of sarcoma when it has spread. This is called palliative treatment.
Types of radiation therapy
External beam radiation therapy: For this treatment, radiation delivered from outside the body is focused on the cancer. This is the type of radiation therapy most often used to treat sarcomas. Treatments are often given daily, 5 days a week, usually for several weeks. Newer forms of external beam radiation may be used that can lessen the impact of the radiation on healthy tissue, such as:
Intensity modulated radiation therapy (IMRT)
Proton beam radiation
Brachytherapy: Brachytherapy (sometimes called internal radiation therapy) is a treatment that places small pellets (or seeds) of radioactive material in or near the cancer. For soft tissue sarcoma, these pellets are put into catheters (very thin tubes) that have been placed during surgery. In high-dose rate (HDR) brachytherapy, the pellets give off a lot of radiation in a short time, and so stay in place for only minutes at a time. In low-dose rate (LDR) brachytherapy, the pellets may stay in place for days at a time, and then removed.
Brachytherapy may be the only form of radiation therapy used or it can be combined with external beam radiation.
Side effects of radiation treatment
Side effects of radiation therapy depend on the area treated and the dose given. Common side effects include mild skin problems and fatigue. These often go away after a short time. If given before surgery, radiation may cause problems with wound healing. Radiation to the abdomen may cause nausea, vomiting, and diarrhea, while radiation to the chest may cause pain with swallowing and lung damage leading to problems breathing. Radiation of large areas of an arm or leg can cause swelling, pain, and weakness. Sometimes the bone that was treated becomes weak, and can fracture years after the treatment. Side effects of radiation therapy to the brain for metastatic sarcoma include hair loss, headaches, and problems thinking.
Chemotherapy for soft tissue sarcomas
Chemotherapy (chemo) is the use of drugs given into a vein or taken by mouth to treat cancer. These drugs enter the bloodstream and reach all areas of the body, making this treatment useful for cancer that has spread (metastasized) to other organs. Depending on the type and stage of sarcoma, chemotherapy may be given as the main treatment or as an adjuvant (addition) to surgery. Chemotherapy for soft tissue sarcoma generally uses a combination of several anti-cancer drugs.
The most commonly used drugs are ifosfamide (Ifex®) and doxorubicin (Adriamycin®). When ifosfamide is used, the drug mesna is also given. Mesna is not a chemo drug. It protects the bladder from the toxic effects of ifosfamide.
Other chemo drugs may be used as well, including cisplatin, dacarbazine (DTIC), docetaxel (Taxotere®), gemcitabine (Gemzar®), methotrexate, oxaliplatin, paclitaxel (Taxol®), vincristine, and vinorelbine (Navelbine®). When several drugs are used together, the combination is given a shortened name such as: MAID (mesna, doxorubicin [Adriamycin], ifosfamide, and dacarbazine).
Chemotherapy drugs kill cancer cells but also damage some normal cells. Side effects depend on the type of drugs, the amount taken, and the length of treatment. Common chemo side effects include: Nausea and vomiting,Loss of appetite,Loss of hair,Mouth sores,Fatigue,Low blood counts
Because chemotherapy can damage the blood-producing cells of the bone marrow, patients may have low blood cell counts. This can result in:
Increased chance of infection (from too few white blood cells)
Problems with bleeding or bruising (from too few blood platelets)
Fatigue and weakness (from too few red blood cells)
Most side effects disappear once treatment is stopped. Hair will grow back after treatment ends, but it might look different. There are remedies for many of the temporary side effects of chemotherapy. For example, anti-emetic drugs can be given to prevent or reduce nausea and vomiting.
Some chemo side effects can last a long time or even be permanent. For example, doxorubicin can weaken the heart if too much is given. If you are to be treated with this drug, your doctor might check your heart function with special studies before starting this drug. The doctor will also watch the dose of doxorubicin closely during therapy.
Some chemo drugs cause nerve damage (called neuropathy), leading to numbness, tingling, or even pain in the hands and feet. Chemotherapy may also permanently damage ovaries or testicles, which can lead to infertility (not being able to have children).
If you will be getting chemo, ask your health care team about the drugs used and their possible side effects.
Hyperthermia and limb perfusion
This procedure is a different way to give chemo. The circulation of the limb (arm or leg) with the tumor in it is separated from that of the rest of the body. Chemo is given just to that limb and the blood is warmed up a bit to help the chemo work better. This has been studied for many years, but it is still not a standard part of the treatment for soft tissue sarcoma. Still, it may be able to help people live longer than standard chemo. It should only be done at centers with a lot of experience in giving chemo this way.
Targeted therapy is a newer type of cancer treatment that uses drugs or other substances to identify and attack cancer cells while doing little damage to normal cells. These therapies attack the cancer cells' inner workings − the programming that makes them different from normal, healthy cells. Each type of targeted therapy works differently, but all alter the way a cancer cell grows, divides, repairs itself, or interacts with other cells.
At this time, pazopanib (Votrient®), is the only targeted therapy drug approved to treat soft tissue sarcoma. It blocks several cellular enzymes called tyrosine kinases that are important for cell growth and survival. In a study of patients with advanced soft tissue sarcomas that had been treated with chemotherapy, pazopanib stopped the cancers from growing for an average of about 3 months longer than the patients given a sugar pill. So far, though, this drug hasn’t been shown to help patients live longer. This drug is taken in pill form, once a day.
Common side effects include high blood pressure, nausea, diarrhea, headaches, low blood cell counts, and liver problems. In some patients this drug causes lab test results of liver function to become abnormal, but it also rarely leads to severe liver damage that can be life threatening. Problems with bleeding, clotting, and wound healing can occur, as well. This drug also rarely causes a problem with the heart rhythm or even a heart attack. If you are taking pazopanib, your doctor will monitor your heart with EKGs as well as check your blood tests to check for liver or other problems.
Treatment of soft tissue sarcomas, by stage
The only way to cure a soft tissue sarcoma is to remove it with surgery, so surgery is part of the treatment of all soft tissue sarcomas whenever possible. It is important that your surgeon and other doctors are experienced in the treatment of sarcomas. These are difficult tumors to treat and require both experience and expertise. Studies have shown that patients with sarcomas have better outcomes when they are treated at specialized cancer centers that have experience in sarcoma treatment.
Desmoid tumors:Desmoid tumors are often not considered true cancers because they do not spread to distant sites. The most common treatment for these tumors is surgery. If the entire tumor is removed and the margins are clear, no other treatment is needed. These tumors can also be treated with radiation (instead of surgery).
For tumors that are large or have come back after treatment, drug therapy may be helpful. The drug sulindac, normally used to treat arthritis, can stop tumor growth or even cause the tumor to shrink. It can take months for the drug to work, but its effect can last for years. Drugs that block estrogen (tamoxifen and toremifene) have also been helpful in some patients. Some desmoid tumors have responded to treatment with chemotherapy (chemo) using the drug doxorubicin (alone or with other drugs). The combination of methotrexate and vinblastine has also been helpful. Interferon, an immune-boosting drug, has also been used with some success.
Stage I soft tissue sarcoma:Stage I soft tissue sarcomas are low-grade tumors of any size. Small (less than 5 cm or about 2 inches across) tumors of the arms or legs may be treated with surgery alone. Radiation therapy may be given after surgery if the tissue removed showed positive or close margins. Positive margins mean that sarcoma cells were growing into the edges of the tissue that was removed. Close margins means that the cancer was found close to the edges of the tissue removed. Either of these can mean that some cancer was left behind and that the cancer may grow back.
For larger tumors, radiation therapy is sometimes given after surgery to lower the chance that the cancer will come back.
If the tumor is not in the arms or legs, (for example the head, neck, or abdomen), removing the entire tumor with enough normal tissue around it can be more difficult. For these tumors, radiation with or without chemo may be given before surgery. This may be able to shrink the tumor enough to remove it entirely with surgery. If radiation is not used before surgery, it may be given after surgery to lower the chance that the tumor will come back.
Stage II and III soft tissue sarcoma:Some stage III tumors have already spread to nearby lymph nodes. Most stage II and III sarcomas are high-grade tumors. They tend to grow and spread quickly. Even when these sarcomas have not yet spread to lymph nodes, the risk of spread (to lymph nodes or distant sites) is very high. These tumors also tend to grow back in the same area after they are removed (this is called local recurrence).
For all stage II and III sarcomas, surgically removing the tumor is still the main treatment. Lymph nodes will be removed as well if they contain cancer. If the tumor is large or in a place that would make surgery difficult, the patient may be treated with chemo, radiation, or both before surgery. For large tumors in the arms or legs, giving chemo by isolated limb perfusion is also an option. The goal of treatment is to shrink the tumor, making it easier to remove. These treatments also lower the chance of the tumor coming back in or near the same place it started. Smaller tumors may be treated with surgery first, then radiation to lower the risk of the tumor coming back. Sometimes chemo is given as well. When chemo is given, the drug most often used is doxorubicin. This drug may be combined with ifosfamide and other drugs.
In rare cases, amputation is needed to remove the entire tumor. As with stage I sarcomas, radiation therapy with or without chemo can be used alone when the tumor's location or size or the patient's health in general makes surgery impossible. There is evidence that chemo after surgery may benefit some people with stage II and III sarcomas.
Stage IV soft tissue sarcoma:A sarcoma is considered stage IV when it has spread to distant sites (M1). Stage IV sarcomas are rarely curable. But some patients may be cured if the main tumor and all of the metastases (areas of cancer spread) can be removed by surgery. This has the best success rate when it has spread only to the lungs. This is still an area where doctors disagree about which patients will benefit. Those patients’ main tumors should be treated as in stages II or III, and metastases should be completely removed, if possible.
For patients whose primary tumor and all metastases cannot be completely removed by surgery, radiation therapy and/or chemotherapy are often given to relieve symptoms. The chemo drugs doxorubicin and ifosfamide are often the first choice — either alone or together with other drugs. Gemcitabine and docetaxel may be given if the first combination stops working (or doesn't work). Patients with angiosarcomas may benefit from treatment with paclitaxel or docetaxel with vinorelbine.
Recurrent sarcoma:Cancer is called recurrent when it come backs after treatment. Recurrence can be local (in or near the same place it started) or distant (spread to other organs or tissues such as the lungs or brain). If the sarcoma comes back in the same area where it started, it may be treated with surgery. Radiation therapy is another option, especially if radiation wasn’t part of the treatment of the original tumor. If external beam radiation was used before, brachytherapy may still be an option.
What happens after treatment for soft tissue sarcomas?
For some people with soft tissue sarcoma, treatment may remove or destroy the cancer. Completing treatment can be both stressful and exciting. You may be relieved to finish treatment, but find it hard not to worry about cancer coming back. (When cancer comes back after treatment, it is called recurrence.) This is a very common concern in people who have had cancer.
It may take a while before your fears lessen. But it may help to know that many cancer survivors have learned to live with this uncertainty and are leading full lives.
For other people, the cancer may never go away completely. These people may get regular treatments with chemotherapy, radiation therapy, or other therapies to try to help keep the cancer in check. Learning to live with cancer that does not go away can be difficult and very stressful. It has its own type of uncertainty.
Follow-up care
When treatment ends, your doctors will still want to watch you closely. It is very important to go to all of your follow-up appointments. During these visits, your doctors will ask questions about any problems you have and might do exams and lab tests or x-rays and scans to look for signs of cancer or treatment side effects. Almost any cancer treatment can have side effects. Some may last for a few weeks to months, but others can last the rest of your life. This is the time for you to talk to your cancer care team about any changes or problems you notice and any questions or concerns you have.
It is important to keep health insurance. Tests and doctor visits cost a lot, and even though no one wants to think about their cancer coming back, this could happen.
If treatment for soft tissue sarcoma stops working
If cancer keeps growing or comes back after one kind of treatment, it is possible that another treatment plan might still cure the cancer, or at least shrink it enough to help you live longer and feel better. But when a person has tried many different treatments and has not gotten any better, the cancer tends to become resistant to all treatment. If this happens, it's important to weigh the possible limited benefits of a new treatment against the possible downsides. Everyone has their own way of looking at this.
This is likely to be the hardest part of your battle with cancer — when you have been through many medical treatments and nothing's working anymore. Your doctor may offer you new options, but at some point you may need to consider that treatment is not likely to improve your health or change your outcome or survival.
If you want to continue to get treatment for as long as you can, you need to think about the odds of treatment having any benefit and how this compares to the possible risks and side effects. In many cases, your doctor can estimate how likely it is the cancer will respond to treatment you are considering. For instance, the doctor may say that more chemo or radiation might have about a 1% chance of working. Some people are still tempted to try this. But it is important to think about and understand your reasons for choosing this plan.
No matter what you decide to do, you need to feel as good as you can. Make sure you are asking for and getting treatment for any symptoms you might have, such as nausea or pain. This type of treatment is called palliative care.
Palliative care helps relieve symptoms, but is not expected to cure the disease. It can be given along with cancer treatment, or can even be cancer treatment. The difference is its purpose— the main purpose of palliative care is to improve the quality of your life, or help you feel as good as you can for as long as you can. Sometimes this means using drugs to help with symptoms like pain or nausea. Sometimes, though, the treatments used to control your symptoms are the same as those used to treat cancer. For instance, radiation might be used to help relieve bone pain caused by cancer that has spread to the bones. Or chemo might be used to help shrink a tumor and keep it from blocking the bowels. But this is not the same as treatment to try to cure the cancer.
At some point, you may benefit from hospice care. This is special care that treats the person rather than the disease; it focuses on quality rather than length of life. Most of the time, it is given at home. Your cancer may be causing problems that need to be managed, and hospice focuses on your comfort. You should know that while getting hospice care often means the end of treatments such as chemo and radiation, it doesn't mean you can't have treatment for the problems caused by your cancer or other health conditions. In hospice the focus of your care is on living life as fully as possible and feeling as well as you can at this difficult time. You can learn more about hospice in our document called Hospice Care.
Staying hopeful is important, too. Your hope for a cure may not be as bright, but there is still hope for good times with family and friends — times that are filled with happiness and meaning. Pausing at this time in your cancer treatment gives you a chance to refocus on the most important things in your life. Now is the time to do some things you've always wanted to do and to stop doing the things you no longer want to do. Though the cancer may be beyond your control, there are still choices you can make.
What`s new in soft tissue sarcoma research and treatment?
Research is ongoing in the area of soft tissue sarcomas. Scientists are learning more about causes and ways to prevent sarcomas, and doctors are working to improve treatments.
Basic research:Scientists have made progress in understanding how certain changes in the DNA of soft tissue cells cause sarcomas to develop. This information is already being applied to new tests to diagnose and classify sarcomas. This is important because accurate classification helps doctors select the most appropriate treatment. It is hoped that this information will soon lead to new strategies for treating these cancers, based on specific differences between normal and malignant soft tissue cells.
Classification:Classification of most cancers, including sarcomas, is based mostly on the way they look under a microscope. Recent research has shown that several different kinds of soft tissue sarcomas can look very similar under the microscope. By using new lab methods, researchers discovered that most cancers that used to be called malignant fibrous histiocytoma (MFH) are actually high-grade forms of liposarcoma, rhabdomyosarcoma, leiomyosarcoma, other sarcomas, and even carcinomas or lymphomas. About 10% to15% of cancers called MFH before, still cannot be given a precise classification, and these are now called pleomorphic undifferentiated sarcomas or undifferentiated pleomorphic sarcomas (although the current classification system of the World health Organization permits use of MFH as an alternate name).
Chemotherapy:Active research in chemotherapy for soft tissue sarcomas includes studies of new drugs and new ways to give drugs now available.
A new drug called trabectedin (Yondelis®) has been shown to help some patients with soft tissue sarcomas. It is approved for use in Europe, but it is still being tested in the United States. In this country, it is currently only available as part of a clinical trial.
Targeted therapy:Even more active than research into chemotherapy is research into so-called targeted drugs. These are drugs that specifically block molecules in the cancer cells that cause the cancers to grow.
Other targeted drugs may also be helpful against sarcomas. For example, one type of sarcoma, gastrointestinal stromal tumor (discussed in a separate document) can be treated with a drug called imatinib (Gleevec®). This drug is also helpful in treating desmoid tumors. Another targeted drug, sunitinib (Sutent®), seems to slow the growth of many sarcomas. Sirolimus (Rapamune®) has shown some promise in treating patients with PEComa, especially pulmonary lymphangioleiomyomatosis.
Anti-angiogenesis drugs:Drugs that block new blood vessel formation may help kill sarcomas by preventing their nourishment by the blood vessels. One such drug, bevacizumab (Avastin®) has shown a small benefit in sarcoma patients, when given with doxorubicin. Other anti-angiogenesis drugs are currently being studied as treatment for sarcomas.
Radiation therapy:Studies of radiation therapy methods, for example, the roles of external beam radiation and brachytherapy (internal radiation) are also in progress. Use of intraoperative (during surgery) radiation therapy for abdominal and retroperitoneal sarcomas is being tested.
