Sarcoma

Treatment Options

Both soft tissue sarcoma and bone sarcoma share similar treatment options, but certain options may be more common, depending on the type of sarcoma. Your doctor will develop your treatment plan based on many factors, including the type, stage and location of your sarcoma, as well as your age and overall health.

Surgery is the main treatment for soft tissue and bone sarcomas. Radiation therapy, chemotherapy or targeted therapy also may be included. Typically, a combination of these treatments is the best approach. In addition, clinical trials for both types of sarcoma offer the newest treatment options to consider, especially if your type of sarcoma is uncommon or rare. In some cases of bone cancer, cryosurgery may be an option. Cryosurgery (also called cryotherapy) is the use of extreme cold produced by liquid nitrogen (or argon gas) to treat tumors inside the body.

Advancements in research have led to more available treatments and better outcomes, with many people living longer with no or stable disease. Be sure to talk to your doctor about all of your treatment options, including any clinical trials that may be appropriate for you.

Surgery

The most common surgical procedure for both soft tissue and bone sarcomas is known as wide local excision. In this procedure, the surgeon’s goal is to remove the entire tumor along with a portion of the normal tissue surrounding it (margin). The width of the margin is determined by the size and thickness of the tumor; the margin is wider for bigger or thicker tumors. When the sarcoma is in the abdomen, head, neck or trunk, the surgeon will try to remove as little normal tissue as possible. A pathologist (a doctor who specializes in diagnosing disease, including cancer) will examine the margin under a microscope to see whether cancer cells are present. If no cancer cells are present (referred to as negative, clear or clean margin), no further treatment is necessary. If cancer cells are found (referred to as positive margin), another surgery, radiation therapy and/or chemotherapy may be used to get rid of any remaining cancer cells.

If sarcoma is in the arm or leg, techniques may be used, when possible, to avoid amputation of the limb. In many cases, amputation may be avoided because of advances in surgical techniques and other types of treatment options. Today, surgeons prefer a limb-sparing procedure, which preserves the use and appearance of the limb. With a limb-sparing procedure, the surgeon may use bone or skin grafts from elsewhere in the body to replace lost tissue or bone in the limb.

However, sometimes amputation may offer the best treatment option, especially for people whose cancer is located where it would be difficult to remove through surgery, for people who cannot have reconstruction or for people with a surgical area that cannot be fully covered with soft tissue.

If the surgeon thinks that the sarcoma has spread to nearby lymph nodes, the lymph nodes will be removed and examined to see if cancer cells are present. Removal of lymph nodes is called lymphadenectomy.

Radiation therapy

Radiation therapy is the use of high-energy X-rays or other particles to kill cancer cells or keep them from growing. The most common type used for sarcomas is external-beam radiation therapy. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. Other types of radiation therapy also may be used as part of treatment (see Table 1). For osteosarcomas, radiation therapy is seldom used because it requires high doses to kill the tumor. When it is used, it’s for bone tumors that cannot be removed with surgery.

Newer techniques of radiation therapy allow the radiation to be directed more precisely, which means higher doses of radiation can be given while avoiding damage to healthy tissue, which reduces side effects. Studies have shown that these newer techniques also lead to lower rates of recurrence. Radiation therapy may be integrated into the overall treatment plan in several ways:

  • After surgery, radiation therapy is given to kill any cancer cells that may remain near the location of the tumor. Treatment given after surgery is known as adjuvant treatment.
  • Before surgery, radiation therapy is given to shrink the tumor and make it easier to remove. Treatment given before surgery is known as neoadjuvant treatment.
  • Instead of surgery, radiation therapy may be used as primary treatment for a person whose health is too poor to tolerate surgery or who has a tumor that is unresectable (unable to be surgically removed) or to help relieve symptoms in a person whose sarcoma has spread (metastasized) to other parts of the body.

If your doctor includes radiation therapy in your treatment plan, a radiation oncologist will carefully plan and oversee your treatment.

Table 1. Types of Radiation Therapy Used for Soft Tissue Sarcoma


Type of Radiation Therapy Description
Brachytherapy Radiation is given via radioactive seeds implanted inside the body near the tumor.
External-beam radiation therapy (EBRT) Radiation is delivered via a radiation machine; is similar to a conventional X-ray except the radiation beams are strong enough to kill cancer cells.
Intensity-modulated radiation therapy (IMRT) Computer images are used to match the radiation delivered to the size and shape of the tumor.
Intraoperative radiation therapy (IORT) A single, powerful dose of radiation is delivered during the surgical procedure, after the sarcoma is removed.
Proton-beam therapy Radiation is delivered through protons (particles that are easier to control than X-rays), which allows precise delivery of radiation to the tumor.
Stereotactic body radiation therapy (SBRT) A specialized form of radiation that involves multiple radiation beam angles allows a powerful dose of radiation to be delivered to a specific area.

 

Chemotherapy

Chemotherapy drugs, also called cytotoxic drugs, are used to stop the growth of cancer, either by killing cancer cells or by preventing them from dividing and growing. Chemotherapy is considered a systemic treatment because the drugs travel throughout the body in the bloodstream. Chemotherapy may be given orally (a pill) or intravenously through a small tube inserted into a vein.

Chemotherapy may be given before or after the primary treatment, which could be surgery or radiation therapy. Both chemotherapy and radiation therapy may be given; this combination is known as chemoradiation therapy. Chemotherapy also may be used as a primary treatment if surgery is not possible. Because chemotherapy drugs travel throughout the entire body, they’re a useful option when the sarcoma has already metastasized to several sites.

For sarcoma, chemotherapy is primarily used to treat Ewing sarcoma, embryonal or alveolar rhabdomyosarcoma in children or young adults, and sarcomas that have spread. When used for bone sarcomas, chemotherapy is usually given before and after surgery.

When chemotherapy drugs are used to treat sarcomas, they may be used alone or in combination with other drugs. These drugs have been used for many years to treat a variety of cancers, and they have been shown to be safe. If chemotherapy is part of your treatment plan, the specific drug(s) chosen for you will be based on the subtype of your particular sarcoma.

The most commonly used chemotherapy drug for soft tissue sarcomas is doxorubicin (Adriamycin). Other chemotherapy drugs may be used as well, including cisplatin, dacarbazine (DTIC), dactinomycin (Cosmegen), eribulin mesylate (Halaven), methotrexate (Otrexup, Rasuvo, Rheumatrex, Trexall), paclitaxel (Taxol), trabectedin (Yondelis), and vincristine (Oncovin, Vincasar Pfs). A combination treatment is also available and is referred to as MAID (mesna [Mesnex], doxorubicin, ifosfamide [Ifex] and dacarbazine).

Sometimes, chemotherapy is not an option because it has not been found to be effective for certain types of sarcomas. For example, studies have shown that gastrointestinal stromal tumors (GISTs) are resistant to chemotherapy, so other treatment must be used.

Targeted therapy

Targeted therapy may be a treatment option for you because some sarcomas are resistant to chemotherapy. Targeted therapy drugs block the signals that proteins and other molecules send along signaling pathways, which are systems in the body that direct basic cell functions like growth, division and death.

Effective targeted therapy depends on two factors: identifying targets that play an important role in the growth and survival of cancer cells and developing agents that can attack those targets. For example, imatinib (Gleevec) targets GIST cells with a mutation (abnormality) in the C-KIT gene; this mutation is found in more than 85 percent of GISTs. Molecular testing is done on a GIST. If the C-KIT mutation is not present, imatinib will not be effective. But if the C-KIT mutation is present, imatinib helps to prevent recurrence after surgical removal of GIST. It is given as a pill and is usually given for three years after surgery for maximum effect. Imatinib also may be used as primary treatment for GISTs that are unresectable or that have metastasized. If imatinib stops working, other targeted drugs are available, including sunitinib (Sutent) and regorafenib (Stivarga).

The most recent targeted therapy drug to be approved is olaratumab (Lartruvo), which is approved for use in combination with doxorubicin (Adriamycin) for adults with soft tissue sarcoma in whom radiation therapy or surgery has not been successful and who have a sarcoma for which an anthracycline-containing chemotherapy is appropriate but has not yet been used.

Pazopanib (Votrient) is another targeted therapy drug approved for some advanced soft tissue sarcomas that have not responded to chemotherapy. Denosumab (Xgeva) is a monoclonal antibody that binds to a protein called RANK ligand. RANK ligand normally tells cells called osteoclasts to break down bone, but when denosumab binds to it, that action is blocked. This drug is used with giant cell tumors of bone that have either come back after surgery or cannot be removed with surgery. Denosumab can help shrink tumors for a while.

Many clinical trials are being conducted to find additional targets in soft tissue and bone sarcoma and to develop targeted therapy drugs directed at them.

Palliative care

Palliative care is treatment that is designed to relieve pain and other side effects of cancer and its treatment; to improve the quality of life; and to support the emotional, spiritual and social needs of people with a serious illness.

Palliative care is often confused with hospice care. Both aim to improve quality of life, but palliative care can benefit anyone with a serious or life-threatening illness and is available at any time during treatment, whereas hospice care is reserved for care at the end of life. Palliative care focuses on quality of life and relief of the symptoms, pain and stress that serious illness can bring. It is provided by a team of specialists who work with your usual health care providers to provide an additional layer of support at any age and any stage in a serious illness and can be provided alongside curative treatment. This care is provided by a multidisciplinary team that consists of doctors, nurses, social workers and others to help relieve suffering and treat symptoms so that you can live as well as you can despite the disease.

Palliative care can help control common symptoms of cancer treatment. For people with sarcoma, palliative care may involve the use of drugs to relieve pain or nausea, radiation therapy to help relieve bone pain, or chemotherapy to help shrink a tumor that’s causing pressure or a blockage.

Because palliative care is available from the time you’re diagnosed throughout the duration of your treatment — as long as you’re experiencing disease-related symptoms — any time is a good time to talk to your doctor about it.

Follow-up care

Despite treatment, there is a risk of recurrence with sarcomas. Recurrence usually occurs within two to five years after initial treatment. Your doctor will establish a follow-up schedule so that if sarcoma does recur, it can be detected — and treated — as soon as possible. Follow-up consists of a history and physical examination, along with imaging studies, such as computed tomography (CT), magnetic resonance imaging (MRI) and ultrasound. In general, follow-up visits are every three to six months for two to three years, then every six months for two years, then annually.

Additional Resources

 

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