Neuroendocrine Tumors

The neuroendocrine system is composed of cells that are a cross between nerve cells and hormone-producing endocrine cells. Scattered throughout the body—including the lungs and the gastrointestinal tract—neuroendocrine cells regulate the flow of air and blood in the lungs and control the speed of digestion. Neuroendocrine tumors (NETs) form from neuroendocrine cells and can be either benign or cancerous.

Neuroendocrine tumors are usually classified as either pancreatic neuroendocrine tumors, also called pancreatic islet cell tumors, which develop in the pancreas, or neuroendocrine carcinoma (carcinoid tumors) that start in other parts of the body. Carcinoid tumors are most often found in the digestive system since it is large and has the most neuroendocrine cells in the body. Gastrointestinal carcinoid tumors form in the lining of the gastrointestinal tract, typically in the appendix, small intestine or rectum. Gastrointestinal carcinoid tumors are uncommon and tend to grow slowly.

Though neuroendocrine tumors are rare, they are on the rise, with the incidence of these types of tumors increasing over the past few decades. Despite this rarity, surgeons at Brigham and Women’s Hospital (BWH) are experienced in diagnosing and treating patients with neuroendocrine tumors. Our surgeons work with a team of medical experts in the Program of Neuroendocrine and Carcinoid Tumors at Dana-Farber Brigham Cancer Center, combining their specialized knowledge to offer multidisciplinary cancer care for patients.

Our board certified surgeons perform a large volume of minimally invasive approaches, such as laparoscopic and robotic surgery, to surgically remove neuroendocrine tumors.

Neuroendocrine Tumor Surgeons

• Thomas Edward Clancy, MD
• Matthew A. Nehs, MD

Types of Neuroendocrine Tumors

Our surgeons are experts in diagnosing and treating patients with the following types of neuroendocrine tumors:

Pancreatic neuroendocrine tumors, also called pancreatic islet cell tumors and pancreatic carcinoid, are rare types of hormone-producing tumors that form in tissues of the pancreas. Learn more about pancreatic neuroendocrine tumors.

Neuroendocrine carcinoma Also referred to as carcinoid tumors, these tumors arise throughout the body and are classified by their site of origin:

• Foregut tumors include bronchial and gastric carcinoid tumors
• Midgut tumors include carcinoids tumors of the small intestine and appendix
• Hindgut tumors include colon and rectal carcinoid tumors

Other types of neuroendocrine tumors include:

• Pheochromocytoma, a rare, typically benign tumor that begins in the chromaffin cells of the adrenal gland
• Merkel cell cancer, also called neuroendocrine carcinoma of the skin or trabecular cancer, is a rare, fast-growing cancer that starts beneath the skin and in the hair follicles

Risk Factors for Neuroendocrine Tumors

In rare cases, neuroendocrine tumors may be inherited but, for the majority of neuroendocrine tumors, no clear cause has been identified. Researchers in the Program of Neuroendocrine and Carcinoid Tumors at the Dana-Farber Brigham Cancer Center are currently actively investigating potential environmental or genetic causes for neuroendocrine tumors.

Symptoms of Neuroendocrine Tumors

Carcinoid tumors are often found during tests or treatments for other conditions and sometimes a patient has no symptoms. Alert your doctor if you experience any of the following symptoms:

• Abdominal pain
• Constipation
• Diarrhea
• Change in stool color
• Blood in the stool
• Pain in the rectum
• Nausea
• Vomiting
• Jaundice
• Heartburn
• Weight loss
• Feeling very tired
• Feeling bloated

Learn more about symptoms of pancreatic islet cell tumor, a type of neuroendocrine tumor.

Diagnosis of Neuroendocrine Tumors

A range of test and procedures may be performed to diagnose neuroendocrine tumors:

• Physical exam and history
• Blood chemistry studies to check for abnormal levels of hormones and other substances
• Tumor marker test to measure the amounts of substances, such as Chromogranin A
• Twenty-four-hour urine test to measure the amounts of substances, such as 5-HIAA or serotonin (hormone)
• MIBG scan A small amount of radioactive material called MIBG (metaiodobenzylguanidine) is injected into a vein and travels through the bloodstream. Carcinoid tumors take up the radioactive material and are detected by a device that measures radiation.
• CT scan (CAT scan)
• MRI (magnetic resonance imaging)
• PET scan (positron emission tomography scan)
• Endoscopic ultrasound
• Upper endoscopy
• Colonoscopy
• Biopsy

Learn about diagnostic tests for pancreatic islet cell tumors, a type of neuroendocrine tumor.

Stages of Neuroendocrine Tumors

After a neuroendocrine tumor has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.

There is no standard staging system for a neuroendocrine tumor. The stages used to describe a pancreatic neuroendocrine tumor are the same as the stages for pancreatic cancer.

Learn more about neuroendocrine tumor stages.

Source: National Cancer Institute.

Treatment for Neuroendocrine Tumors

Surgical Treatment

Our surgeons offer extensive expertise in the most advanced minimally invasive surgical procedures for neuroendocrine tumors. When neuroendocrine tumors are detected at an early stage, they can usually be removed surgically. In most cases, post-surgical treatment with chemotherapy or radiation therapy is not necessary, and patients can be monitored with blood tests and scans.

One of the following surgical procedures may be used:

• Endoscopic resection Surgery to remove a small tumor that is on the inside lining of the GI tract. An endoscope is inserted through the mouth and passed through the esophagus to the stomach and sometimes, the duodenum. An endoscope is a thin, tube-like instrument with a light, a lens for viewing, and a tool for removing tumor tissue.
• Local excision Surgery to remove the tumor and a small amount of normal tissue around it.
• Resection Surgery to remove part or all of the organ that contains cancer. Nearby lymph nodes may also be removed.
• Cryosurgery A treatment that uses an instrument to freeze and destroy carcinoid tumor tissue. This type of treatment is also called cryotherapy. The surgeon may use ultrasound to guide the instrument.
• Radiofrequency ablation The use of a special probe with tiny electrodes that release high-energy radio waves (similar to microwaves) that kill cancer cells. The probe may be inserted through the skin or through an incision (cut) in the abdomen.
• Liver transplant Surgery to remove the whole liver and replace it with a healthy donated liver.
• Hepatic artery embolization A procedure to embolize (block) the hepatic artery, which is the main blood vessel that brings blood into the liver. Blocking the flow of blood to the liver helps kill cancer cells growing there.

Learn about surgery for pancreatic islet cell tumors, a type of neuroendocrine tumor.

Non-Surgical Treatment

• Medication Synthetic somatostatin analogs that mimic the action of somatostatin can be used to treat neuroendocrine tumors. These drugs are effective in decreasing hormone production and slowing tumor growth.
• Chemotherapy has been used for many years as a treatment for neuroendocrine tumors, and can in some cases be highly effective—particularly for pancreatic neuroendocrine tumors.
• Interferon is a biological agent administered using a subcutaneous injection. Treatment with interferon has been reported to slow tumor growth in some cases.
• Targeted therapies are molecules that specifically target growth pathways in the tumor cells.
• Radiation Therapy
• Hormone Therapy

What You Should Expect

You will receive a thorough diagnostic examination to evaluate if you have a neuroendocrine tumor and determine what course of treatment is needed. Careful monitoring and the involvement of an experienced surgeon are important to successful outcomes for patients.

If you are having surgery or a procedure, you will likely be scheduled for a visit to the Weiner Center for Preoperative Evaluation for pre-operative information and tests.

The day of surgery, you will be cared for in the operating room by surgeons, anesthesiologists and nurses who specialize in neuroendocrine tumor surgery. After surgery, you will recover in the post-surgical care unit where you will receive comprehensive care by an experienced surgical and nursing staff.

Learn more about your hospital stay and returning home.

Multidisciplinary Care

The Program of Neuroendocrine and Carcinoid Tumors at the Gastrointestinal Cancer Treatment Center at Dana-Farber Brigham Cancer Center provides advanced and innovative multidisciplinary care for patients with neuroendocrine tumors. Our treatment team includes surgeons, medical and radiation oncologists, pathologists, radiologists, anesthesiologists, gastroenterologists and nursing and research staff. In addition, patients have full access to BWH’s world-renowned academic medical community with its diverse specialists and state-of-the-art facilities.

Cancer Surgery Appointments and Locations

Contact one of our cancer surgeons in the list at the top of the page to make an appointment.

Cancer Surgery Locations