People with a strong family history of sarcomas or other cancers occurring at a young age may wish to discuss the benefits and disadvantages of genetic testing with their doctor. The genetic testing results should always be explained by a genetic counselor or a specially trained doctor who can interpret the results and advise highrisk patients about early cancer detection.
Families with a history of certain inherited conditions caused by mutated tumor suppressor genes have an increased risk of developing soft tissue sarcomas. The mutated genes can be detected by genetic testing, so family members should discuss this option with their doctors. They should let their doctor know about any lumps or growths right away.
No screening tests and exams are recommended for people who have no family history of sarcoma or other sarcoma risk factors. For these people, the best approach to early detection is to notify their health care professional of any unexplained lumps or growths or other symptoms that may be caused by a soft tissue sarcoma.
The only way to cure a soft tissue sarcoma is to remove it with surgery, so surgery is part of the treatment for 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 act differently from most softtissue sarcomas in that although they can grow into nearby tissues and often come back after surgery, they rarely spread to distant sites.
Some desmoid tumors can be watched without treatment for a time. Treatment will be given if the tumor is growing or is causing pain or other symptoms.
If treatment is needed and the entire tumor can be removed, the first treatment is often 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 (Adriamycin), which may be used alone or with other drugs. The combination of methotrexate and vinblastine has also been helpful. Interferon, an immuneboosting drug, has also been used with some success. Another option is the targeted drug imatinib (Gleevec).
Stage I soft tissue sarcoma
Stage I soft tissue sarcomas are lowgrade 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. The goal of surgery is to remove the tumor with some of the normal tissue around it. If cancer cells are found in or near the edges of the tissue removed (called positive or close margins), it can mean that some cancer was left behind. Often the best option for positive or close margins is more surgery. Another option is treating with radiation therapy after surgery. This lowers the chance of the cancer coming back.
If the tumor is not in a limb, (for example it is in 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 lessen the chance that the tumor will come back.
Stages II and III soft tissue sarcoma
Some stage III tumors have already spread to nearby lymph nodes. Most stage II and III sarcomas are highgrade 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 (Adriamycin). This drug may be combined with ifosfamide (Ifex) 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 areas of cancer spread (metastases) can be removed by surgery. The best success rate is 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 (Taxol) or docetaxel (Taxotere) with vinorelbine (Navelbine).
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.
If the sarcoma returns at a distant site, chemo may be given. If the sarcoma has spread only to the lungs, it may be possible to remove all the areas of spread with surgery. Radiation is used to treat sarcomas that spread to the brain, as well as any recurrences that cause symptoms such as pain.