Oncologist-approved cancer information from the American Society of Clinical Oncology
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Sarcoma - Alveolar Soft Part and Cardiac

This section has been reviewed and approved by the Cancer.Net Editorial Board, 9/2013
Treatment Options

ON THIS PAGE: You will learn about the different ways doctors use to treat people with these types of sarcoma. To see other pages in this guide, use the colored boxes on the right side of your screen, or click “Next” at the bottom.

This section outlines treatments that are the standard of care (the best proven treatments available) for ASPS and cardiac sarcoma. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new treatment to evaluate whether it is safe, effective, and possibly better than the standard treatment. Your doctor can help you review all treatment options. For more information, see the Clinical Trials and Latest Research sections.

Treatment overview

In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team.

Descriptions of the most common treatment options for ASPS and cardiac sarcoma are listed below. Treatment options and recommendations depend on several factors, including the type and stage of the sarcoma, possible side effects, and the patient’s preferences and overall health. Take time to learn about your treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with your doctor and what you can expect while receiving the treatment. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and surrounding tissue (called the margin) during an operation. A surgical oncologist is a doctor who specializes in treating cancer using surgery.

Surgery is the most common treatment for ASPS. If the tumor is in an arm or leg, limb-sparing surgical techniques can often be used. However, because the surgeon may need to take a wide margin of tissue to make sure no cancer remains, occasionally there may be a need to remove part of a limb or all of a limb (called amputation). If amputation is needed, rehabilitation that includes physical therapy can help maximize a patient’s physical functioning. It can also help a person cope with the social and emotional effects of losing a limb.

For 80% of people with cardiac sarcoma, by the time the tumor causes symptoms, it has already spread and cannot be completely removed with surgery. In these situations, radiation therapy or chemotherapy may be used (see below).

Learn more about cancer surgery.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist.

Radiation therapy may be used before surgery to shrink the size of the tumor or after surgery to destroy any remaining cancer cells. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation therapy given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time.

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished.

Learn more about radiation therapy.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. A chemotherapy regimen usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.

Unfortunately, chemotherapy is not very effective for ASPS or cardiac sarcoma and often causes serious side effects. Therefore, newer drug treatment options, such as cediranib for ASPS (see Targeted Therapy below) or other drugs currently being studied in clinical trials, may be recommended instead.

Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases.

Targeted therapy

Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells while limiting damage to normal cells.

Recent studies have discovered specific genetic mutations (changes) found in ASPS cells that are not found in healthy cells (see the Risk Factors section). These changes cause ASPS genes to be “turned on,” causing cells to grow uncontrollably, spread into other tissues, and eventually metastasize to other parts of the body. Drugs are now being developed that “turn off” or target these genes.

Cediranib (Recentin, AZD2171) is a drug currently being studied in clinical trials that specifically targets three genes that are known to promote the growth of ASPS through angiogenesis (the process of making new blood vessels). Because a tumor needs the nutrients delivered by blood vessels to grow and spread, the goal of anti-angiogenesis therapies is to “starve” the tumor.

In a recently published study, 46 patients who had ASPS that could not be surgically removed were given cediranib every day. Approximately one-third of the patients had significant decrease in the size of the tumor that lasted months or, in some cases, years without the tumor re-growing. This study also suggested that for people whose tumor did not shrink, cediranib still may have kept the tumor from growing for a longer period of time than would have been expected.

The most common side effects of cediranib are diarrhea and high blood pressure. Talk with your doctor about possible side effects for a specific medication and how they can be managed.

Many other research studies are now taking place to find out more about cediranib and other targeted therapies for ASPS (see the Latest Research section for more information).

Learn more about targeted treatments.

Heart transplantation

Heart transplantation involves using a heart from a donor to replace the heart of a person with cardiac sarcoma. For a transplant to be successful, the patient will have to take immunosuppressive medication (medications that help a patient’s body accept the new heart). As a result of taking this medication, the patient could develop a new cancer or the sarcoma might come back; therefore, patients and their doctors should carefully consider and talk about this treatment option.

Getting care for symptoms and side effects

Sarcoma and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the tumor, an important part of cancer care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care can help a person at any stage of illness. People often receive treatment for the tumor and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, and other therapies. You may also receive palliative treatments similar to those meant to eliminate the sarcoma, such as chemotherapy, surgery, and radiation therapy. Talk with your doctor about the goals of each treatment in the treatment plan.

Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and supportive care options. And during and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem so it is addressed as quickly as possible. Learn more about palliative care.

Recurrent ASPS or cardiac sarcoma

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called “no evidence of disease” or NED.

A remission can be temporary or permanent. This uncertainty leads many survivors to feel worried or anxious that the cancer will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the sarcoma returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if the sarcoma does return. Learn more about coping with the fear of recurrence.

If the sarcoma does return after the original treatment, it is called recurrent sarcoma. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence, including whether the sarcoma’s stage has changed. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies described above (such as surgery, chemotherapy, and radiation therapy), but they may be used in a different combination or given at a different pace. Your doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent sarcoma.

People with recurrent sarcoma often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with cancer recurrence.

Metastatic ASPS or cardiac sarcoma

If sarcoma has spread to another location in the body, it is called metastatic sarcoma. Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of sarcoma, because there can be different opinions about the best treatment plan. Learn more about seeking a second opinion before starting treatment, so you are comfortable with the treatment plan chosen. This discussion may include clinical trials.

Your health care team may recommend a treatment plan that might include a combination of chemotherapy, radiation therapy, and possibly surgery. Supportive care will also be important to help relieve symptoms and side effects.

For most patients, a diagnosis of metastatic sarcoma is very stressful and, at times, difficult to bear. Patients and their families are encouraged to talk about the way they are feeling with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group.

If treatment fails

Recovery from sarcoma is not always possible. If treatment is not successful, the disease may be called advanced or terminal cancer.

This diagnosis is stressful, and this is difficult to discuss for many people. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain is extremely important.

Patients who have advanced cancer and who are expected to live less than six months may want to consider a type of palliative care called hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to think about where you would be most comfortable: at home, in the hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative for many families. Learn more about advanced cancer care planning.

After the death of a loved one, many people need support to help them cope with the loss. Learn more about grief and bereavement.

Choose “Next” (below, right) to continue reading to learn more about clinical trials, which are research studies. Or, use the colored boxes located on the right side of your screen to visit any section.

© 2005-2014 American Society of Clinical Oncology (ASCO). All rights reserved worldwide.

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