This page was reviewed under our medical and editorial policy by
Maurie Markman, MD, President, Medicine & Science
This page was updated on June 6, 2022.
After receiving a neuroendocrine tumor diagnosis, the care team will work with the patient to develop a comprehensive treatment plan based on his or her unique diagnosis and needs. Most neuroendocrine tumor (NET) patients are diagnosed at an advanced stage, so an supportive approach is key to the patient's well-being.
Treatment options vary, depending on factors such as the NET type and stage. The cancer care team also considers the patient's overall health and preferences.
When discussing treatment possibilities, they may also offer information on side effects for each option and how these may affect the patient's quality of life.
The tumor itself may greatly impact the patient's treatment options as well, including factors such as:
Choosing a treatment option should be a shared decision between the patient and his or her care team based on the patient's goals and preferences.
Common treatment options for neuroendocrine tumors include those listed below.
Active surveillance may be a treatment option for low-grade NETs. Because such NETs tend to grow slowly, chances are low that they’ll spread or cause symptoms without the care team first noticing a change. This approach may prevent unnecessary treatment.
The care team may recommend regular testing, including physical examinations, blood tests and/or imaging tests such as magnetic resonance imaging (MRI) or computed tomography (CT) scans to monitor any changes in the tumor(s). If they do notice any changes that suggest the tumor may grow or spread, your care team can provide further treatment options.
Surgically excising the primary tumor is often the first approach recommended for patients with localized NETs. The goal of surgery is to fully remove a neuroendocrine tumor or reduce the tumor burden. Surgery may also be an option for those with advanced disease, to help relieve neuroendocrine tumor symptoms. Some surgical procedures for NETs include debulking or cytoreductive surgery, minimally invasive laparoscopic resections and liver transplantation, depending on where the tumor is located.
Enucleation: This surgery removes only the tumor. It may be an option for small and localized tumors.
Debulking surgery: If the entire tumor cannot be removed, debulking it—or removing some of it—may be an option. Debulking surgery may help relieve some symptoms caused by an NET.
Cytoreductive surgery: This type of surgery for gastrointestinal NETs removes as many cancerous cells as possible in order to reduce the number of hormones the tumor can produce. It may also reduce some symptoms caused by the tumor.
Laparoscopic surgery: This type of surgery uses one or more small incisions to remove cancerous cells in the abdomen. The benefit of laparoscopic surgery is a faster recovery, since it’s a less invasive technique than standard surgery.
Liver transplant: For NETs that have spread to the liver, a liver transplant may be a treatment option.
Palliative surgery: If a tumor cannot be fully removed, but reducing its size could decrease symptoms, palliative surgery may be an option. This type of surgery focuses on improving comfort and quality of life. It may be paired with radiation therapy.
Chemotherapy for NETs may be used alone or alongside other treatments. It’s typically used when the tumor begins causing symptoms or has spread. A number of chemotherapy drugs may help prevent cancer cells from multiplying and growing.
Chemoembolization, in which chemotherapy drugs are administered directly into the tumor, may be an option if the disease has metastasized (spread) to the liver. During the course of chemotherapy treatments, patients may experience side effects, including:
Targeted therapy drugs work by targeting specific genes, proteins or other factors of the tumor. The benefit of targeted therapy is that it may stop or slow the growth of cancer cells while sparing damage to healthy cells. The care team may need to run additional tests to find out more about the patient's tumor in order to identify the right targeted therapy for him or her.
Targeted therapies for NETs may include:
Hormone therapy for NETs typically won’t shrink a tumor, but it may slow its growth. It’s primarily used to control the substances released from an NET, which are similar to hormones. These substances often cause symptoms, so hormone therapy may reduce some of these symptoms. Hormone therapy for NETs centers around somatostatin, a hormone that plays a part in controlling other hormones such as insulin. Somatostatin analogs are drugs that act like the hormone somatostatin to reduce the symptoms caused by an NET.
The two somatostatin analogs are:
Both drugs are typically given as an injection and may cause side effects such as:
The care team may be able to suggest ways to manage these side effects.
Radiation therapy is generally used when a neuroendocrine tumor has spread or is in a location that makes surgery difficult. CyberKnife® may be a non-invasive option for some patients and enables radiation oncologists to deliver high, targeted doses of radiation to NETs. For metastatic disease to the liver, TheraSphere® and SIR-Spheres® offer innovative options that deliver radiation directly to tumors in the liver.
For NETs that have spread to the liver, embolization procedures may help reduce or cut off the supply of blood to the tumor. Though these interventional radiology treatments aren’t surgical, they may involve staying overnight in the hospital. Embolization procedures may have an effect on liver enzymes for a few days or weeks after the treatment, and they may cause fever or pain around the liver as well.
The care team may provide a wide range of treatment options for gastrointestinal NETs. The gastroenterology team uses various technologies to look at the GI tract in different and minimally invasive ways. They may recommend innovative techniques and ablative treatments to help remove obstructions in the GI tract and relieve pain or breathing problems.
PRRT is a molecular targeted therapy that may be used to treat certain NETs. Molecular targeted therapies use drugs or other substances to identify and attack cancer cells while reducing harm to healthy tissue.
An emerging NET treatment involves the use of theranostics. This technology pairs diagnostic biomarkers with therapeutic agents to identify and target cancer cells for destruction.
The Food and Drug Administration has approved a drug called LutatheraⓇ (Lu 177 dotatate) to treat some types of neuroendocrine tumors. This radioactive drug attaches to cancer cells, enabling radiation to damage the cells.
The cancer care team can also help the patient explore options for taking part in a clinical trial, which provides treatment as part of a research study. Clinical trials help determine whether a new treatment—including new drugs, different doses or a novel combination of treatments—may be better than the current standard approach for that type and stage of cancer.
Next topic: What are the symptoms of neuroendocrine tumors?