Treatment of Soft Tissue Sarcomas, by Stage

The best chance 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's important that your surgeon and other doctors are experienced in the treatment of sarcomas. These tumors are hard to treat and require both experience and expertise. Studies have shown that people with sarcomas tend to have better outcomes when they're treated at specialized cancer centers that have experience in sarcoma treatment.

Treatment is generally similar for most types of soft tissue sarcoma, especially for earlier stage sarcomas. But in recent years, as doctors have learned more about the differences between the types, newer targeted therapy and immunotherapy drugs have become important treatment options for some types of advanced soft tissue sarcomas.

Stage I soft tissue sarcomas

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. 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's in the head, neck, or abdomen), it can be harder to take out the entire tumor and enough normal tissue around it. For these tumors, radiation with or without chemotherapy (chemo) may be given before surgery. This may shrink the tumor enough to remove it entirely with surgery. If radiation is not used before surgery, it may be given after surgery to help lessen the chance that the tumor will come back.

Stage II and III soft tissue sarcomas

Most stage II and III sarcomas are high-grade tumors. They tend to grow and spread quickly. Some stage III tumors have already spread to nearby lymph nodes. Even when these sarcomas have not yet spread to lymph nodes, the risk of spread (to lymph nodes or other parts of the body) is very high. These tumors also tend to grow back in the same area after they're removed. (This is called local recurrence.)

For all stage II and III sarcomas, removing the tumor with surgery is the main treatment. Lymph nodes will also be removed if they might contain cancer. Radiation may be given after surgery.

If the tumor is large or in a place that would make surgery difficult, but not in lymph nodes, 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. Chemo, radiation, or both might also be given after surgery. These treatments 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 help lower the risk of the tumor coming back.

In rare cases, amputation of part or all of a limb might be needed to remove the entire tumor, although this is now done much less often than it was in the past.

Radiation therapy with or without chemo can be used alone when the tumor's location or size or the patient's overall health makes surgery impossible.

Stage IV soft tissue sarcomas

A sarcoma is considered stage IV when it has spread to distant parts of the body. Stage IV sarcomas are rarely curable. But some patients may be cured if the main (primary) 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. In this case, the main tumors are treated as in stages II or III, and metastases are removed completely, if possible. This is still an area where doctors disagree about what the best treatment is and which patients are most likely to benefit.

For people whose primary tumor and all metastases cannot be removed completely by surgery, radiation therapy and/or chemotherapy are often used. The chemo drugs doxorubicin and ifosfamide are often the first choice — either together or along with other drugs. Gemcitabine and docetaxel may be given if the first combination doesn't work or stops working. People with angiosarcomas may benefit from treatment with paclitaxel or docetaxel with vinorelbine.

For some types of soft tissue sarcomas, treatment with newer targeted drugs or immunotherapy might also be an option.

Recurrent sarcomas

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 may be given after surgery, 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 in a distant part of the body, chemo, targeted therapy, or immunotherapy drugs may be options. If the sarcoma has spread only to the lungs, it may be possible to remove all the areas of spread with surgery. Radiation is often used to treat sarcomas that spread to the brain, as well as any recurrences that cause symptoms such as pain.