How Are Neuroendocrine Tumors Treated?

Neuroendocrine cells are specialized cells with traits similar to both nerve cells (neuro) and hormone-releasing cells (endocrine). Neuroendocrine cells are found throughout the body, so neuroendocrine tumors may be found anywhere. However, the most common locations where these tumors appear are in the lungs, appendix, small intestine, rectum, and pancreas.^1,2

Neuroendocrine tumors are rare. Some grow quickly and others slowly. If one of these tumors releases extra hormones, it is called a functional neuroendocrine tumor. If it does not release hormones, or not enough to cause symptoms, it is called a nonfunctional neuroendocrine tumor.

Treatment can vary widely because treatment is shaped by several things, such as:³

• Where the tumor first began
• Whether the tumor is producing hormones
• Stage
• Grade
• How fast the cancer cells are dividing
• Somatostatin receptor status

Treatment choices also vary based on possible side effects, your current health, personal goals, and preferences for treatment.

Treatment options

There are many treatment options available for neuroendocrine tumors, including:^1,3

• Active surveillance
• Surgery
• Medicines
• Somatostatin analogs
• Chemotherapy
• Targeted therapy
• Immunotherapy
• Peptide receptor radionuclide therapy (PRRT)
• Radiation
• Liver treatments
• Palliative and hospice care

Active surveillance

Sometimes your doctor may recommend a "watch and wait" approach. This is called active surveillance. This may be an option if your cancer is slow-growing and unlikely to cause problems for months or years. During this time, you receive regular tests and exams to monitor the cancer. If it changes or begins to grow, active treatment may begin.

Surgery

Surgery is an option that depends on where the tumor is located and how much it has spread. Ideally, your doctor will try to remove the whole tumor and some surrounding healthy tissue.

If removing the whole tumor is not possible, your doctor will remove as much of it as they can. This is called debulking surgery. This type of surgery may relieve some of your symptoms, but it generally does not provide a cure.

Sometimes surgery is not possible. In these cases, the cancer is said to be inoperable.³

Medicines for neuroendocrine tumors

There are many drugs that may be used to treat neuroendocrine tumors. These may be given alone or in combination with surgery or radiation. These drugs may be given through an IV (intravenous tube) needle placed in a vein, in a pill and swallowed, or injected directly into muscle. Drugs that may be used include:³

Somatostatin analogs

Somatostatin is a hormone that the body naturally makes. It controls several other hormones, including insulin. Somatostatin analogs are drugs that act similarly to the natural hormone. These drugs may be used to control the symptoms of hormone-like substances released by the cancer. They may also help slow cancer growth. There are 2 somatostatin analogs used to treat neuroendocrine tumors:³

• Octreotide (Sandostatin®)
• Lanreotide (Somatuline® Depot)

Chemotherapy

Chemotherapy drugs are ones used to kill any fast-growing cells, including cancer cells. Chemotherapy may be given 1 drug at a time, or in combination with other treatments. Chemotherapy is usually given on a set schedule over a specific length of time.^3,4

Targeted therapy

Traditional chemotherapy kills both cancer cells and normal cells. Targeted therapies are drugs that target specific genes, proteins, or tissue environments to stop or slow a cancer's growth. Targeted therapy is also called precision medicine.^3,4

Targeted therapies approved to treat neuroendocrine tumors include:²

• Everolimus (Afinitor®)
• Sunitinib (Sutent®)

Immunotherapy

Immunotherapy is a type of biologic therapy. These drugs help boost the body's immune system to fight the cancer.³

Peptide receptor radionuclide therapy (PRRT)

Peptide receptor radionuclide therapy (PRRT) is a way to deliver radiation to a neuroendocrine tumor. Instead of a beam of energy like traditional radiation, PRRT is delivered by IV. It then moves through the body until it attaches to the cancer cells. There is one PRRT currently available: 177Lu-dotatate (Lutathera®).^3,4

Radiation

Radiation therapy uses a high-energy beam of light to destroy cancer cells. It is given on a set schedule over a period of time. External beam radiation therapy is the type most often used for neuroendocrine tumors.^3,4

Liver treatments

When neuroendocrine tumors spread past their original site, they often move to the liver. Treatments used to treat neuroendocrine cancer of the liver include chemotherapy and radioactive beads. Doctors may also use high-energy radio waves to heat and destroy cancer cells.^3,4

Palliative care

Palliative care is often confused with hospice care. However, these 2 branches of medicine have different purposes.

Palliative care is medical care that helps relieve pain and other symptoms of a serious illness like neuroendocrine tumors. Its goal is to improve quality of life while someone is still receiving treatment. Other symptoms your palliative care team may help with include:⁵

• Nausea and vomiting
• Depression and anxiety
• Constipation
• Trouble breathing or sleeping

Your doctor may recommend palliative care at any time in your treatment. It may or may not include end-of-life planning.⁵

Hospice care

Hospice care is medical care given when a person has no possible cure to their disease or chooses not to undergo more treatment. It too focuses on relieving symptoms and making the person comfortable, but all attempts to treat cancer have stopped.⁵