Neuroendocrine Tumor of the Gastrointestinal Tract: Types of Treatment

Approved by the Cancer.Net Editorial Board, 08/2018

ON THIS PAGE: You will learn about the different types of treatments doctors use for people with a GI tract NET. Use the menu to see other pages.

This section explains the types of treatments that are the standard of care for a GI tract NET. “Standard of care” means the best treatments known. When making treatment plan decisions, you are also encouraged to consider clinical trials as an option. A clinical trial is a research study that tests a new approach to treatment. Doctors want to learn whether the new treatment is safe, effective, and possibly better than the standard treatment. Clinical trials can test a new drug, a new combination of standard treatments, or new doses of standard drugs or other treatments. Clinical trials are an option to consider for treatment and care for all stages of cancer. Your doctor can help you consider all your treatment options. To learn more about clinical trials, see the About 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 and is especially important for people with a NET. Cancer care teams include a variety of other health care professionals, such as physician assistants, oncology nurses, social workers, pharmacists, counselors, dietitians, and others.

Descriptions of the common types of treatments used for a GI tract NET are listed below, followed by an outline of treatment options by stage. Your care plan may also include treatment for symptoms and side effects, an important part of cancer care.

Treatment options and recommendations depend on several factors, including the stage and grade of cancer, possible side effects, and the patient’s preferences and overall health. Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Talk with your doctor about the goals of each treatment and what you can expect while receiving the treatment. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. A surgical oncologist is a doctor who specializes in cancer surgery. Completely removing the entire tumor is the standard treatment, when possible. Most localized GI tract NETs are successfully treated with surgery alone. The surgeon will usually remove some tissue surrounding the tumor, called a margin, in an effort to leave no traces of cancer in the body.

When completely removing the tumor is not possible, debulking surgery is sometimes recommended. Debulking surgery removes as much of the tumor as possible and may provide some relief from symptoms, but it generally does not cure a NET.

People who have developed carcinoid syndrome are at risk of experiencing a carcinoid crisis during surgery (see Symptoms and Signs). To avoid major complications from a carcinoid crisis, the anesthesiology team must be fully aware of this risk before surgery, so they can have treatment on hand to control the symptoms.

Before surgery, talk with your health care team about the possible side effects from the specific surgery you will have. Learn more about the basics of cancer surgery.

Surgical options for a GI tract NET include:

  • Local excision. During this operation, the surgeon removes the tumor and some surrounding healthy tissue. Most localized tumors can be surgically removed through a skin incision, but a rectal neuroendocrine tumor may be removed through the anus. Other GI tract NETs can sometimes be removed using an endoscope (see Diagnosis).

  • Partial gastrectomy. This surgery removes part of the stomach.

  • Esophagectomy. This procedure is the removal of all or part of the esophagus. The esophagus is the tube that connects the throat to the stomach.

  • Small bowel resection. The small bowel is another term for the small intestine. This surgery removes parts of the small intestine.

  • Appendectomy. Appendectomy is surgery to remove the appendix.

  • Segmental colon resection or hemicolectomy. This surgery removes one-third to one-half of the colon, including nearby blood vessels and lymph nodes.

  • Low anterior resection. During this surgery, a portion of the upper part of the rectum is removed.

  • Abdominoperineal resection. This surgery is used for a large tumor located in the lower part of the rectum. During the operation, the surgeon removes the anus, rectum, and part of the colon. After surgery, a colostomy may be created to carry waste out of the body. A colostomy is an opening from the colon to the outside of the body.

  • Liver resection. This operation removes cancer that has spread to the liver. The goal of this surgery is not to eliminate the cancer, but it often helps relieve or reduce the symptoms of carcinoid syndrome.

Therapies using medication

Systemic therapy is the use of medication to destroy cancer cells. Common ways to give systemic therapies include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally). This type of medication can reach cancer cells throughout the body. Systemic therapies are generally prescribed by a medical oncologist, a doctor who specializes in treating cancer with medication.

The types of systemic therapies used for a GI tract NET include:

  • Somatostatin analogs

  • Chemotherapy

  • Targeted therapy

  • Immunotherapy

  • Peptide receptor radionuclide therapy (PRRT)

Each of these types of therapies are discussed below in more detail. A person may receive only 1 type of systemic therapy at a time or a combination of systemic therapies given at the same time. They can also be given as part of a treatment plan that includes surgery and/or radiation therapy.

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.

Somatostatin analogs

Somatostatin is a hormone in the body that controls the release of several other hormones, such as insulin and glucagon. Somatostatin analogs are drugs that are similar to somatostatin and are used to control the symptoms created by the hormones released by a GI tract NET. They may also slow the growth of a NET, although they do not generally shrink the tumors.

There are 2 somatostatin analogs used to treat NETs, octreotide and lanreotide.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by ending the cancer cells’ ability to grow and divide. Chemotherapy for a GI tract NET is most often used when the tumor has spread to other organs or is causing severe symptoms.

A chemotherapy regimen, or schedule, usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or a combination of different drugs given at the same time. Common drugs for a GI tract NET include:

  • 5-fluorouracil (5-FU)

  • Capecitabine (Xeloda)

  • Carboplatin

  • Cisplatin

  • Dacarbazine (DTIC)

  • Etoposide

  • Oxaliplatin (Eloxatin)

  • Streptozocin (Zanosar)

  • Temozolomide (Temodar)

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

Learn more about the basics of chemotherapy.

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 healthy cells.

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. This helps doctors better match each patient with the most effective treatment whenever possible. In addition, research studies continue to find out more about specific molecular targets and new treatments directed at them. Learn more about the basics of targeted treatments.

A U.S. Food and Drug Administration (FDA)-approved targeted therapy for the treatment of advanced GI tract NETs is everolimus (Afinitor). This treatment can help slow down the growth of these tumors in some patients, but it does not usually shrink tumors.

Other targeted therapies for GI tract NETs are being researched in clinical trials. They include drugs that interfere with new blood vessel formation or with specific survival pathways of cancer cells. Talk with your doctor about possible side effects for a specific medication and how they can be managed.

Immunotherapy

Immunotherapy, also called biologic therapy, is designed to boost the body's natural defenses to fight the cancer. It uses materials made either by the body or in a laboratory to improve, target, or restore immune system function. Interferon alfa-2b (Intron A) is a type of immunotherapy that has been used to treat NETs. Interferon helps the body’s immune system work better and can lessen diarrhea and flushing. It may also shrink tumors.

Different types of immunotherapy can cause different side effects. Interferon alfa-2b may cause flu-like symptoms, fatigue, and low blood counts. Talk with your doctor about possible side effects for the immunotherapy recommended for you. Learn more about the basics of immunotherapy.

Peptide receptor radionuclide therapy (PRRT)

In January 2018, the FDA approved a treatment called 177Lu-dotatate (Lutathera) for advanced GI tract NETs. This is a radioactive drug that works by binding to a cell’s somatostatin receptor, which may be present on certain tumors. After binding to the receptor, the drug enters the cell, allowing radiation to damage the tumor cells. Common side effects include low levels of white blood cells, high enzyme levels in some organs, high blood sugar levels, low level of potassium, and nausea and vomiting. Talk with your doctor about ways these can be avoided or managed. The broader term to describe this treatment is peptide receptor radionuclide therapy (PRRT).

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist.

The most common type of radiation treatment for a GI tract NET is called external-beam radiation therapy, which is radiation given from a machine outside the body. It is most often used as part of supportive care to relieve symptoms, such as pain, caused by cancer that has spread to the bone and other areas of the body (see “Getting care for symptoms and side effects” below).

Patients receiving radiation therapy may experience fatigue during treatment, and the treated area may become red and dry. Radiation therapy to the chest or neck may cause a dry, sore throat or a dry cough. Some patients have shortness of breath during radiation therapy. Most side effects go away after the treatment is finished. Learn more about the basics of radiation therapy.

Liver-directed treatment

If cancer has spread to the liver, the following treatments may be used:

  • Radiofrequency ablation (RFA).  RFA destroys a tumor by heating it with an electric current. It is usually used for small liver metastases and does not work well on larger tumors.

  • Hepatic artery embolization. This procedure blocks the tumor’s blood supply by sealing off the blood vessels leading to the tumor. If embolization is done by itself, it is called bland embolization. When combined with chemotherapy, it is called chemoembolization. It is not yet clear if 1 treatment is more effective than the other. These treatments are usually used for people with metastatic disease that mostly affects the liver, in particular those who have symptoms caused by the size of the tumor or by hormones produced by the tumor.

  • Radioembolization. This form of radiation therapy involves the use of tiny beads made of glass or resin that contain low levels of a radioactive material called yttrium-90. The beads are put into the blood vessel that sends blood to the tumor in the liver. The beads then become stuck in the liver and deliver the radiation directly to the tumor. This treatment avoids exposing other areas of the body to radiation. There are 2 FDA-approved methods of radioembolization: SIR-Spheres and TheraSphere.

Care for symptoms and side effects

Cancer and its treatment often cause side effects. In addition to treatments intended to slow, stop, or eliminate the cancer, 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 is any treatment that focuses on reducing symptoms, improving quality of life, and supporting patients and their families. Any person, regardless of age or type and stage of cancer, may receive palliative care. It works best when palliative care is started as early as needed in the cancer treatment process. People often receive treatment for the cancer at the same time that they receive treatment to ease side effects. In fact, people who receive both at the same time 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, emotional support, and other therapies. You may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, or radiation therapy. Talk with your doctor about the goals of each treatment in your treatment plan.

For some people, such as those with lung disease, heart disease, or other specific medical conditions, surgery cannot successfully treat the cancer. In these cases, palliative surgery to relieve symptoms may be helpful. Palliative surgery is often used together with radiation therapy.

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

Treatment of a GI tract NET by stage

Stages I, II, and III

  • Stomach. A localized NET of the stomach can often be completely removed through an endoscope. A tumor larger than 2 cm is removed with a margin of surrounding stomach tissue through an incision, or cut, in the abdomen.

  • Small intestine. Surgery to remove the tumor and surrounding tissue is the most common treatment for a NET smaller than 1 cm located in the small intestine. Surgery for a larger tumor involves removing more surrounding tissue and some surrounding blood vessels and lymph nodes.

  • Large intestine/colon. The most common treatment for a tumor smaller than 2 cm is the surgical removal of the tumor and surrounding tissue, often done through a colonoscope (see Diagnosis). If the tumor is larger than 2 cm, surgery most often involves an incision through the skin.

  • Appendix. An appendectomy is usually the only treatment needed for a NET smaller than 1.5 cm. For a tumor larger than 2 cm, the removal of about one-third of the colon next to the appendix and nearby blood vessels and lymph nodes is often needed.

  • Rectum. A rectal NET smaller than 1 cm is treated with RFA (see “Surgery” above). A tumor larger than 2 cm is more likely to grow and spread quickly. Larger tumors are removed using the same procedure that is used for rectal cancer. This involves removing some healthy colorectal tissue and some of the nearby lymph nodes.

Stage IV

If cancer spreads to another part in the body from where it started, doctors call it metastatic cancer. If this happens, it is a good idea to talk with doctors who have experience in treating it. Doctors can have different opinions about the best standard treatment plan. Clinical trials might also be an option. Learn more about getting a second opinion before starting treatment, so you are comfortable with your chosen treatment plan.

Your treatment plan may include a combination of the types of treatment described above. Surgery is used to relieve symptoms rather than eliminate the cancer at this stage. If distant metastases are not causing symptoms, surgery may not be needed. If the person develops carcinoid syndrome, surgery to remove as much cancer as possible is often recommended. Chemotherapy and radiation therapy may also be offered to help relieve symptoms.

Participation in clinical trials is encouraged. Palliative care will also be important to help relieve symptoms and side effects.

For most people, a diagnosis of metastatic cancer is very stressful and, at times, difficult to bear. You and your family are encouraged to talk about how you feel 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.

Remission and the chance of recurrence

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

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

If the cancer does return after the original treatment, it is called recurrent cancer. 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 new cycle of testing will begin again to learn as much as possible about the recurrence. After this testing is done, you and your doctor will talk about the treatment options. Often the treatment plan will include the treatments 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 suggest clinical trials that are studying new ways to treat this type of recurrent tumor. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

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

If treatment does not work

Recovery from a GI tract NET is not always possible. If the cancer cannot be cured or controlled, the disease may be called advanced or terminal.

This diagnosis is stressful, and for many people, advanced cancer is difficult to discuss. However, it is important to have open and honest conversations with your 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.

People who have advanced cancer and who are expected to live less than 6 months may want to consider 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 talk with the health care team about hospice care options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option 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 loss.

The next section in this guide is About Clinical Trials. It offers more information about research studies that are focused on finding better ways to care for people with a GI tract NET. Use the menu to choose a different section to read in this guide.