This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of tumor. 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 standard treatment. Your doctor can help you review all treatment options. 

Treatment overview

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 an endocrine tumor are listed below. For more information, please see the Treatment section for the specific tumor type that has been diagnosed.

Treatment options and recommendations depend on several factors, including the type and stage of the tumor, if it is cancerous; possible side effects; and the patient's preferences and overall health. Your doctor may also recommend genetic testing of your tumor to help plan treatment. 

Surgery

The purpose of surgery is typically to remove the entire tumor, along with some of the healthy tissue around it, called the margin. A surgical oncologist is a doctor who specializes in treating cancer using surgery. If the tumor cannot be removed entirely, “debulking” surgery may be performed. Debulking surgery is a procedure in which the goal is to remove as much of the tumor as possible.

Side effects of surgery include weakness, fatigue, and pain for the first few days following the procedure.

Chemotherapy

Chemotherapy is the use of drugs to kill tumor cells, usually by stopping the cells' ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach tumor cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating tumors with medication. A chemotherapy regimen (schedule) 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.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.

The medications used to treat tumors 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. 

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill tumor cells. A doctor who specializes in giving radiation therapy to treat a tumor is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation 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 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.

Hormone therapy

The goal of hormone therapy is often to lower the levels of hormones in the body. Hormone therapy may be given to help stop the tumor from growing or to relieve symptoms caused by the tumor. In addition, for thyroid cancer, hormone therapy will be given if the thyroid gland has been removed, to replace the hormone that is needed by the body to function properly.

Immunotherapy

Immunotherapy (also called biologic therapy) is designed to boost the body's natural defenses to fight the tumor. It uses materials made either by the body or in a laboratory to bolster, target, or restore immune system function. Examples of immunotherapy include cancer vaccines, monoclonal antibodies, and interferons.

Alpha interferon is a form of biologic therapy given as an injection under the skin. This is sometimes used to help relieve symptoms caused by the tumor, but it can have severe side effects including fatigue, depression, and flu-like symptoms. 

Targeted therapy

Targeted therapy is a treatment that targets the tumor'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 tumor cells while limiting damage to normal cells, usually leading to fewer side effects than other cancer medications.

Recent studies show that not all tumors have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors in your tumor. As a result, doctors can better match each patient with the most effective treatment whenever possible. In addition, many research studies are taking place now to find out more about specific molecular targets and new treatments directed at them.

Depending on the type of endocrine tumor, targeted therapy may be a possible treatment option. For instance, targeted therapies, such as sunitinib (Sutent) and everolimus (Afinitor), have been approved for treating advanced islet cell tumors. Early results of clinical trials (research studies) with targeted therapy drugs for other types of endocrine tumors are promising, but more research is needed to prove they are effective.

Recurrent endocrine tumor

Once your treatment is complete and there is a remission (absence of symptoms; also called “no evidence of disease” or NED), talk with your doctor about the possibility of the tumor returning. Many survivors feel worried or anxious that the tumor will come back. 

If the tumor does return after the original treatment, it is called a recurrent tumor. 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. 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 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 cancer.

People with a recurrent tumor 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. 

Metastatic endocrine tumor

If a cancerous tumor has spread to another location in the body, it is called metastatic cancer.

Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of cancer, because there can be different opinions about the best treatment plan. 

Your health care team may recommend a treatment plan that includes a combination of surgery, chemotherapy, radiation therapy, hormone therapy, immunotherapy, or targeted therapy.

In addition to treatment to slow, stop, or eliminate the cancer (also called disease-directed treatment), an important part of cancer care is relieving a person's symptoms and side effects. It includes supporting the patient with his or her physical, emotional, and social needs, an approach called palliative or supportive care. People often receive disease-directed therapy and treatment to ease symptoms at the same time.

If disease-directed treatment is not successful, this may also be called advanced cancer. This diagnosis is stressful, and it may be difficult to discuss. 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.

Side Effects

A tumor and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects occur.

Fear of treatment side effects is common after a diagnosis of a tumor, but it may be helpful to know that preventing and controlling side effects is a major focus of your health care team. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of tumor, its location, the individual treatment plan (including the length and dosage of treatment), and your overall health. Common side effects for each treatment option are described in detail within the Treatment section.

Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health care team. Also, be sure to communicate with the doctor about side effects you experience during and after treatment. Care of a patient's symptoms and side effects is an important part of a person's overall treatment plan; this is called palliative or supportive care. It helps people with an endocrine tumor at any stage of illness be as comfortable as possible. 

Be sure to talk with your doctor about the level of caregiving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with an endocrine tumor. 

In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. For many patients, a diagnosis of an endocrine tumor is stressful and can bring difficult emotions. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies. 

A side effect that occurs more than five years after treatment is called a late effect. Treatment of late effects is an important part of survivorship care.

After Treatment

After treatment for an endocrine tumor ends, talk with your doctor about developing a follow-up care plan. Although there are no specific guidelines on follow-up care for an endocrine tumor, this plan may include regular physical examinations, blood tests, and/or a CT scan about three months after surgery. Follow-up care thereafter should include a physical examination and blood tests approximately every six to 12 months, with additional imaging studies (such as x-rays) to be performed on an as-needed basis. It is important to talk with your doctor about any new symptoms you experience.

People recovering from the successful treatment of an endocrine tumor are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help you rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level.

Latest Research

Doctors are working to learn more about endocrine tumors, ways to prevent them, how to best treat them, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the diagnostic and treatment options best for you. Also, be sure to read the Current Research section of the specific endocrine tumor type that has been diagnosed.

Combination chemotherapy and surgery. The combination of the drugs oxaliplatin (Eloxatin) and irinotecan (Camptosar) along with surgery is being studied to treat endocrine tumors.

Targeted therapy. Targeted therapy is being researched as a treatment option for several types of endocrine tumors, including neuroendocrine tumors and thyroid cancer. The focus of targeted therapy is to stop the growth and spread of a tumor in several different ways. Some of the drugs being studied for endocrine tumors include:

Vatalanib may block some of the enzymes needed for cell growth and the blood flow to the tumor. Vatalanib is given with the drug octreotide (Sandostatin), which helps control symptoms, such as diarrhea, caused by some tumors.

Several drugs are being studied for advanced thyroid cancer that does not respond to surgery and/or I-131 treatment. These include axitinib, sorafenib (Nexavar), pazopanib (Votrient), and motesanib diphosphate. 

Pazopanib and motesanib diphospate are also being studied for advanced islet cell tumors. In addition, sunitinib and bevacizumab (Avastin) in combination with chemotherapy, octreotide, and everolimus (Afinitor) are being studied for patients with advanced islet cell tumors. 

Bevacizumab is being researched for neuroendocrine tumors. 

Genetic and molecular testing. The genetic testing and the refinement of RET oncogenes  is an ongoing area of active research that will improve selection of treatment and give more precise prognosis. Researchers are also looking at using the molecular biology (the study of the structure and function of cells at the molecular level) of the tumor to help diagnose endocrine tumors and predict how well treatment will work.

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current endocrine tumor treatments in order to improve patients' comfort and quality of life.