While neuroendocrine tumors are uncommon, they're not uncommon to the experts at Stanford Health Care. Our physician-scientists are leaders in NET research and treatment.
What are neuroendocrine tumors?
Neuroendocrine tumors are masses of cells that form in the neuroendocrine system. Neuroendocrine cells appear throughout the body and carry traits of nerve cells and endocrine (hormone-producing) cells.
NETs can happen anywhere in the body and often have symptoms similar to those of other, less serious conditions. It's important to discuss any troublesome symptoms with your doctor.
How does neuroendocrine cancer develop?
Neuroendocrine cancer happens when neuroendocrine cells change abnormally (mutate), multiply, and form tumors and spread. These tumors affect the organs where the abnormal cells are located.
Neuroendocrine tumors develop most often in the gastrointestinal (GI) tract, lungs, and pancreas. Sometimes NETs spread (metastasize) to other parts of the body.
While we don't know what causes NETs, most people with NETs have certain inherited (passed down in families) genetic conditions.
Connect to Care
Let us help find personalized care options for you and your family.
Neuroendocrine Tumor Symptoms
Neuroendocrine tumors often produce no symptoms in their early stages. NET symptoms, when they appear can include general cancer symptoms such as fatigue, a loss of appetite or weight loss. They also can produce symptoms according to where they are in the body, as well as the type of hormone they produce.
Depending on the tumor’s location, you may see one or more of the following NET symptoms: Pain that won't go away
- Bleeding or discharge
- Bowel or bladder changes
- Lump or thickening in the affected area
- Nausea or vomiting
- Persistent cough or hoarseness
- Jaundice
Depending on the hormone the tumor releases, you may also experience NET symptoms such as:
- Anxiety and confusion
- Diarrhea
- Flushing, causing the neck and face to become red and feel warm
- Hyperglycemia or hypoglycemia (high or low blood sugar)
- Skin rash
- Ulcer disease
Carcinoid syndrome happens when a neuroendocrine tumor produces too much serotonin. The extra hormones go to the bloodstream and cause symptoms such as:
- Flushed face
- Sweating
- Diarrhea
- Blood pressure fluctuations
- Breathing problems such as wheezing and shortness of breath
- Heart murmur or fast heartbeat
- Weakness
Neuroendocrine Tumor Risk Factors
Risk factors increase the chance of developing cancer and other conditions. You may have a higher risk of developing neuroendocrine tumor or cancer if you have certain inherited syndromes, including:
- Multiple endocrine neoplasia type 1 (MEN1)
- Von Hippel-Lindau syndrome
- Neurofibromatosis type 1
- Multiple endocrine neoplasia type 2 (MEN2)
- Familial paraganglioma syndrome (SDHx)
- Tuberous sclerosis complex
Types of Neuroendocrine Tumors
Neuroendocrine tumors get their name from their location, most commonly appearing in the GI tract, lungs, and pancreas. NETs in the intestine are sometimes called carcinoid tumors. NET types include:
Gastrointestinal NETs can form anywhere in the digestive system, from the esophagus to the anus. They most frequently appear in the small intestine and rectum. GI NETs include:
- Gastroenteropancreatic neuroendocrine tumors (GEP-NETs or islet cell tumors): These tumors can develop anywhere along the GI tract.
- Appendiceal neuroendocrine tumor: This tumor appears in the appendix.
- Small intestine neuroendocrine tumor: Another name for this tumor is small bowel neuroendocrine tumor or carcinoid.
- Gastric carcinoid tumor: This NET forms in the lining of the GI tract.
- Colon carcinoid tumor: This NET appears in the colon.
- Rectal carcinoid tumor: The type of tumor is in the rectum.
Lung NETs are types of lung cancer and include:
- Small cell lung cancer (SCLC): This type of neuroendocrine cancer in the lungs grows and spreads quickly.
- Large cell neuroendocrine carcinoma: This tumor is similar to SCLC but has larger cancerous cells.
- Typical carcinoid tumors: These tumors usually grow slowly and do not spread beyond the lungs. Most lung carcinoids are typical carcinoid tumors.
- Atypical carcinoid tumors: These tumors are less common than typical carcinoids. They grow faster than typical carcinoids and tend to spread to other organs.
Pancreatic neuroendocrine tumor types include:
- Gastrinoma: This tumor produces above-average levels of gastrin, a hormone that stimulates the stomach to secrete acids and enzymes. Gastrinoma can cause peptic ulcers.
- Glucagonoma: This pancreatic tumor secretes glucagon, a hormone that raises the blood's glucose levels and leads to a rash.
- Insulinoma: This rare pancreatic tumor secretes insulin, the hormone that lowers glucose levels in the blood.
- VIPoma: This type of neuroendocrine pancreatic tumor produces vasoactive intestinal polypeptide (VIP) and occasionally other hormones. VIP secretion can lead to severe intermittent diarrhea that causes further problems, including dramatic potassium loss.
- Somatostatinoma: This type of tumor affects cells that produce somatostatin, a hormone that regulates other hormones in the body. These cells can grow in the pancreas, intestine, or stomach.
NETs that form elsewhere in the body include:
- Merkel cell carcinoma in the skin
- Pheochromocytoma in the adrenal glands above the kidneys
- Paraganglioma, most often in the lower head and neck
- Medullary thyroid cancer in the thyroid (a gland in the neck)
- Pituitary adenomas in the pituitary gland in the skull base
Neuroendocrine Tumor Stages
Doctors use a process called staging to determine if cancer has spread and, if so, how far. Staging helps doctors determine a prognosis (probable outcome based on others' experiences) and develop a treatment plan. Stage 0 describes the early signs of disease, and Stage 4 indicates an advanced case.
Your care team uses a T, N, M system to stage neuroendocrine cancer, considering these factors:
- Tumor: Your doctor looks at the tumor's size and location.
- Nodes: Your doctor determines if cancer has spread to nearby lymph nodes.
- Metastasis: Your doctor checks to see if cancer has spread (metastasized) to other body parts.
During the staging process, doctors also consider the grade of the tumor. Grading describes how fast tumor cells grow and divide.
Cancer staging can be complicated, and each type of neuroendocrine cancer has its own staging system. Your doctor will explain the staging process to you and help you understand the extent of the cancer you have.
Diagnostic Tests for Neuroendocrine Tumors
Your doctor starts your diagnosis by completing a comprehensive physical examination and asking questions about your:
- Symptoms
- Medical history
- Family history of cancer and other conditions
Depending on the type of NET your doctor suspects, you may undergo specialized diagnostic tests, such as:
Imaging tests provide detailed pictures of tissues and bones inside your body. Your doctor may order several imaging studies, such as:
- Computed tomography scan (CT scan): A specialized X-ray beam moves around your body while capturing pictures of bones, muscles, tissues, and organs.
- Magnetic resonance imaging scan (MRI): A large magnet, radio waves, and computer work together to take clear, detailed pictures.
- Positron emission tomography (PET) scan: This type of imaging measures your body's cell activity. You receive a small amount of a tracer (radioactive substance) that travels through your body. The tracer collects in areas of increased cell activity (typically indicating cancer) and appears brighter on the scan.
- PET/CT scan: PET and CT technology combine to show areas of increased cell activity and highly detailed images of nearby organs and tissues.
- PET/MRI scan: This PET and MRI combination shows cell differences and detailed images of the brain, internal organs and soft tissues. It uses less radiation than a CT scan.
- Ultrasound: This test uses sound waves to create images of the body's blood vessels, tissues, and organs.
- X-ray: This test uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
Your doctor may take small samples of your urine or blood to check for levels of proteins, hormones, or other substances in your body. Lab tests can also help identify if you have genetic conditions that increase your risk of certain NETs.
With an endoscopy, your doctor inserts an endoscope (a thin, lighted tube) through the nose, mouth, or rectum to examine the digestive tract. They may also take tissue samples for a biopsy.
If imaging and lab tests indicate you might have neuroendocrine cancer, your doctor may request a biopsy. Your doctor removes a small tissue sample, and a pathologist analyzes the cells under a microscope.
Connect to Care
Why Choose Stanford?
View Treatment Options
Neuroendocrine Tumors
Neuroendocrine cancer is rare and starts in neuroendocrine cells. Learn about neuroendocrine tumors and our approach to care.
neuroendocrine tumors
carcinoid tumor (MSV: 31
100)
pancreatic neuroendocrine tumor (7
800)
neuroendocrine tumor symptoms (4
100)
neuroendocrine tumor treatment (2
600)
neuroendocrine cancer (MSV: 29
600)
pheochromocytoma (183
000)
paraganglioma (MSV: 27
100)
medullary thyroid cancer (12
700)