Neuroendocrine cancer is a complex disease made up of widely varying tumors that can arise in many parts of the body. At the OHSU Knight Cancer Institute, we're experts on this complicated condition. We treat more neuroendocrine cancers than any hospital in the Northwest, offering:
- Team-based care with a range of specialists working together
- The latest diagnostic techniques
- A full array of treatments for every stage of illness
- Access to clinical trials to try a promising new therapy
- A wide range of support services to help you manage any side effects of cancer or cancer treatment
Understanding neuroendocrine cancer
Neuroendocrine tumors, called NETs for short, start in cells that have aspects of nerve cells (neuro) and hormone-producing cells (endocrine). These cells release hormones into the bloodstream in response to signals from the nervous system. Some neuroendocrine tumors are malignant (cancer), and some are benign (not cancer).
Neuroendocrine tumors are relatively rare. Most often, they develop in the digestive tract or in the lungs, but they can also form in places such as the pancreas and skin. They can be difficult to diagnose because they may have no or few symptoms. They also develop in many forms, making them more challenging to treat.
What is neuroendocrine cancer?
Neuroendocrine cells release hormones to help the body with air and blood flow, and with how quickly food moves through the gastrointestinal, or digestive, tract. A tumor forms when these cells begin to rapidly divide and grow out of control. Most of these tumors are slow-growing.
Who gets neuroendocrine cancer?
- There are many types of NETs, and all can affect nearly anyone.
- Roughly five in 100,000 people are diagnosed with a NET each year in the U.S.
- Studies have found that incidence rates have significantly increased over the past few decades, though that's probably because doctors are able to diagnose NETs more often.
What causes neuroendocrine cancer?
In most cases, doctors aren't certain of any specific cause. Smoking may increase risk, and a small portion of NETs are linked to inherited genetic conditions such as:
- Multiple endocrine neoplasia Type 1, or MEN1
- Neurofibromatosis Type 1
- Von-Hippel Lindau syndrome OHSU offers genetic counseling, testing and risk-assessment services if you have or might have a family history of cancer.
Statistics on survival rates for neuroendocrine cancers are unreliable because the disease can take so many forms and develop in so many parts of the body. It's also common for the cancer to be found after it has spread, making it more difficult to treat.
A 2012 study that included a co-author from OHSU, however, looked at nearly 20,000 patients diagnosed with a gastrointestinal NET from 1973 to 2008. It found that 67% to 96% of patients survived at least five years after diagnosis, depending on which part of the digestive tract was affected.¹
Symptoms of neuroendocrine cancer
NETS are often misdiagnosed at first because some patients have no symptoms while others have symptoms similar to those of other ailments, such as irritable bowel syndrome. Some NETs produce an abnormally high amount of hormones, causing symptoms such as:
- Asthma-like wheezing
- Abdominal pain
- Skin changes
- Weight loss
- Unusual bleeding
Diagnosing neuroendocrine cancer
Your care team may use the following tests to detect a NET.
Blood/urine test: Your doctor may order blood or urine tests to check hormone levels. Elevated levels may indicate the presence of a NET, although further testing is likely. Fasting may be necessary in some cases.
Biopsy: A doctor called a pathologist examines tissue extracted from the tumor. Using a microscope and special stains, the doctor can compare it with normal tissue. How normal or abnormal the tumor's cells look can indicate how fast the NET may grow.
Endoscopy: A flexible scope with a camera is inserted into the digestive tract through the throat or rectum, with the patient under sedation. Tools can be used to take a sample of abnormal-looking tissue.
Endoscopic ultrasound: This uses the same process as endoscopy but includes an ultrasound probe to provide images. The gastroenterology doctors who perform this procedure at OHSU are experts, doing hundreds of endoscopic ultrasounds a year.
Wireless capsule endoscopy: You swallow a small capsule, which looks like a bigger-than-normal vitamin but is actually a color camera complete with a battery, flash and transmitter. It takes continuous pictures of your insides over about eight hours, wiring the images to a recorder worn on your waist.
MRI: Magnetic resonance imaging essentially takes a picture of structures inside your body using magnets and radio waves.
Octreotide scan: The tumor's cancer cells may have proteins, or receptors, that bind to other substances. These receptors may bind to a substance called octreotide. To detect cancer, octreotide is mixed with a radioactive agent and injected into your arm. A special scan shows highlighted areas if cancer is present.
Types of neuroendocrine cancer
Carcinoid: These slow-growing tumors are found in the lining of the gastrointestinal tract, which has more neuroendocrine cells than anywhere else in the body. According to the American Cancer Society, about 8,000 cases of this type are diagnosed each year in the U.S. The average age of people affected is the early 60s. Black people are more likely than white people to develop them, and they occur slightly more often in women.
Pancreatic neuroendocrine tumors: These make up less than 5% of pancreatic tumors. They typically have better outcomes than other pancreatic cancers. They form in hormone-producing endocrine cells and may be malignant or benign.
- Functioning NETs: About half of pancreatic NETs are functioning, meaning they make hormones that cause symptoms.
- Nonfunctioning NETs: These don't make enough hormone to cause symptoms. This can delay diagnosis.
Pheochromocytoma: This extremely rare tumor forms in the adrenal glands, which sit above the kidneys. While only about 10% of cases contain cancer cells that can spread, the tumor releases hormones that can cause high blood pressure, elevated heart rate and other heart-related issues.
Merkel cell cancer: This uncommon skin cancer can spread if untreated. It often develops in areas with high sun exposure but can start anywhere. Symptoms include firm bumps on the skin that are usually red, pink or purple. According to the American Cancer Society, about 1,500 cases are diagnosed each year in the U.S. More than 9 in 10 people affected are older than 50, and more than 9 in 10 cases are found in whites.
Medullary thyroid carcinoma: This cancer develops in the cells that produce calcitonin, which controls the amount of calcium in the body. It is hard to find and manage. There are two subtypes:
- Sporadic: This type, accounting for about 8 in 10 cases, does not run in families and affects most older people in just one thyroid lobe.
- Familial: This type is inherited and usually develops in childhood or early adulthood. According to the American Cancer Society, it accounts for 4% of thyroid cancers.
Staging neuroendocrine cancer
Normally, staging helps guide treatment decisions by assessing the size of the tumor and whether and how far cancer has spread.
Staging systems for NETs are constantly being refined. But for now, they do not reliably predict outcomes. That means knowing whether your neuroendocrine cancer is Stage I, II, III or IV doesn't mean much.
Instead, it's important to know that the Knight Cancer Institute offers treatment options for all types and stages of neuroendocrine cancer. Your care team will work with you to develop a treatment plan for your unique needs.
Neuroendocrine cancer treatments
At the Knight Cancer Institute, several types of doctors work as a team to treat neuroendocrine tumors. Your care team may include a surgical oncologist, endocrinologist, medical oncologist, radiation oncologist, gastroenterologist and others.
We also treat patients with every stage of disease. If surgery isn't an option, we offer treatments to give you the longest survival and best quality of life possible.
Surgically removing the tumor, sometimes with a minimally invasive laparoscopic surgery, is usually the best first option. If the entire tumor can't be removed, taking out as much of the tumor as possible can ease hormone-related symptoms and improve quality of life.
Somatostatin analog therapy
Somatostatin analogs are injected medications that slow a tumor's hormone production to relieve symptoms. OHSU was among the first centers to test this therapy, leading to approval by the U.S. Food and Drug Administration in 1999. OHSU continues to test new somatostatin analogs.
Targeted therapies work at the molecular level, attacking cancer cells while mostly leaving healthy cells alone, resulting in fewer side effects. Targeted therapies for neuroendocrine tumors include:
- Everolimus (brand name Afinitor), an FDA-approved pill that studies (including at OHSU) have shown can significantly lengthen survival for patients with certain neuroendocrine tumors of the pancreas, gastrointestinal tract or lung. The medication may be combined with somatostatin analog therapy.
- Sunitinib malate (brand name Sutent), is an FDA-approved capsule used to treat certain pancreatic neuroendocrine tumors. This medication lengthens survival by stopping cancer cells from dividing and by preventing tumors from developing blood vessels.
Medications, usually given in an IV drip, can fight cancer cells nearly anywhere in the body, often by preventing them from growing or dividing. It can be used before surgery to shrink a tumor, after surgery to kill cancer cells still circulating in the body, or with advanced cancer to control symptoms.
This therapy uses targeted beams of energy to kill cancer cells. For neuroendocrine cancers, it's often used to control cancer that has spread to another part of the body.
When neuroendocrine tumors spread, they most commonly spread to the liver. If the cancer can't be surgically removed from the liver, tumor embolization may be an option. This procedure uses substances placed in the body through a catheter (thin tube) to block or disrupt blood flow to the tumor to slow its growth.
- Look for a clinical trial for neuroendocrine cancers.
- Read about our cancer registries, including one for neuroendocrine tumors, which offer the opportunity to help research and receive updates.
- Gastrointestinal Carcinoid Tumors, American Cancer Society
- International Neuroendocrine Cancer Alliance
- NET Patient Foundation
- Merkel Cell Skin Cancer, American Cancer Society
¹ "Trends of Incidence and Survival of Gastrointestinal Neuroendocrine Tumors in the United States: A SEER Analysis," Journal of Cancer, July 2012, Vassiliki L. Tsikitis (of OHSU), Betsy C. Wertheim and Marlon A. Guerrero.
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