Sarcoma

Treatment Options

Some treatment options are similar for both soft tissue sarcoma and bone sarcoma, but sometimes the best treatment option depends on the type of sarcoma. Your doctor will create your treatment plan based on many factors, including the type, stage and location of your sarcoma, as well as your age and overall health.

Typically, a combination of these treatments is the best approach. In addition, clinical trials may be of particular benefit if your type of sarcoma is uncommon or rare.

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.

Surgery

Surgery is the main treatment for soft tissue and bone sarcomas. The most common surgical procedure for both types 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 (called the "margin"). The surgeon will try to remove as little normal tissue as possible consistent with achieving a negative margin. 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 help control possible remaining cancer cells.

If sarcoma is in the arm or leg, techniques may be used, when possible, to avoid amputation of the limb. Advances in surgical techniques and other types of treatment options have led to a decrease in the number of amputations done for sarcoma. 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 when the cancer is located where it would be difficult to surgically remove or for people who cannot have reconstruction or who have a surgical area that cannot be fully covered with soft tissue. Depending on the type of surgery you have, reconstruction may be necessary as part of the primary procedure. This may include the use of prostheses, grafts and metal rods or screws.

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.

In some cases of bone cancer, cryosurgery (or cryotherapy) may be an option. Cryosurgery differs greatly from traditional surgery. Instead of removing a sarcoma through an incision, the doctor inserts a hollow instrument through the skin into the tumor and extreme cold, produced by liquid nitrogen (or argon gas), is used to kill the sarcoma cells.

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 (see Figure 1). Other types of radiation therapy may also be used as part of treatment (see Table 1). For osteosarcomas, radiation therapy is usually used only 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:

  • 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.
  • After surgery, radiation therapy is given to kill any cancer cells that may remain near the site of the tumor. Treatment given after surgery is known as adjuvant 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 metastasized (spread) to other parts of the body.

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, which allows a powerful dose of radiation to be delivered to a specific area.

 

Chemotherapy

Chemotherapy drugs are used to stop the growth of cancer, by either killing cancer cells or preventing them from dividing and growing. Chemotherapy is considered a systemic treatment because the drugs travel in the bloodstream throughout the body and may be given orally (a pill) or intravenously through a small tube inserted into a vein (see Figure 2).

Chemotherapy may be given before (neoadjuvant) or after (adjuvant) the primary treatment, which is typically surgery or radiation therapy. Chemoradiation, a combination of chemotherapy and radiation therapy, may also be used. If surgery is not possible, chemotherapy may be used as a primary treatment. Because chemotherapy drugs travel throughout the entire body, they’re a useful option when the sarcoma has spread 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.

Several chemotherapy drugs can be used to treat sarcomas, and they may be used alone or in combination with other drugs. Your doctor will consider your particular type of sarcoma before recommending chemotherapy in your treatment plan.

 

Common Chemotherapy Medications
dacarbazine (DTIC)
dactinomycin (Cosmegen)
doxorubicin (Adriamycin)
eribulin mesylate (Halaven)
ifosfamide (Cyfos, Ifex)
methotrexate (Otrexup, Rasuvo, Rheumatrex, Trexall)
trabectedin (Yondelis)
vinblastine (Velban)
vincristine
Combination treatment: MAID (mesna, doxorubicin, ifosfamide and dacarbazine)

 

Targeted Therapy

Targeted therapy may be a treatment option for people who have a sarcoma that is 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. Also considered a systemic treatment because the drugs travel in the bloodstream throughout the body, targeted therapy may be given orally (a pill) or intravenously through a small tube inserted into a vein (see Figure 2 above).

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, one type of targeted therapy targets gastrointestinal stromal tumor (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 to see if the C-KIT mutation is present in the GIST. If the mutation is not present, the drug will not be effective. But if it is present, the drug helps to prevent recurrence after the GIST has been surgically removed. This strategy may also be used as primary treatment for GISTs that are unresectable or that have metastasized. If the initial drug used stops working, other targeted drugs are available.

Another type of targeted therapy 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 the drug 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 and may help shrink tumors for a while.

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

 

Common Targeted Therapy Medications
denosumab (Xgeva)
imatinib (Gleevec)
olaratumab (Lartruvo)
pazopanib (Votrient)
regorafenib (Stivarga)
sunitinib (Sutent)

Immunotherapy

Immunotherapy uses the body’s own immune system to slow the growth of and kill cancer cells. To do so, the immune system uses substances made either by the body or in a laboratory to find and destroy cancer cells, much like it fights off foreign bacteria. One type of immunotherapy drug is known as a checkpoint inhibitor. This type of drug blocks specific proteins on the surface of immune T-cells. Blocking these proteins releases a natural brake on the immune system, allowing it to attack the cancer. Checkpoint inhibitors have been found to be effective in many other types of cancer, such as melanoma and lung cancer. Ongoing clinical trials are evaluating the use of checkpoint inhibitors in sarcoma.

Palliative Care

Although sarcoma treatment focuses on curative intent, palliative care may be needed. Palliative care includes pain management and helps with other side effects of cancer and its treatment. Its focus is to improve 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 relieving pain, other symptoms and the emotional distress that serious illness can bring. This care is provided by a multidisciplinary team that consists of doctors, nurses, social workers and others, all of whom work together to enhance your quality of life.

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 is 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

After you are finished with treatment, follow-up care is recommended. Your doctor will establish a schedule that may consist of a history and physical examination, blood tests, bone scan and imaging studies, such as computed tomography (CT), magnetic resonance imaging (MRI) and ultrasound. In general, follow-up visits are every four months for two to three years, then every six months for three years, then annually.

Additional Resources

 

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