Pancreatic Neuroendocrine Tumors

The pancreas is an organ that’s behind the stomach and in front of the spine. It’s a gland that has two main parts:

• The exocrine pancreas. This makes enzymes that go into the intestines and help break down food.
• The endocrine pancreas. This makes hormones, such as insulin, that are released into the blood.

Most cancers that start in the pancreas are exocrine cancers. Endocrine tumors of the pancreas are covered here.

The hormone-making cells of the pancreas bunch together in small clusters all over the pancreas. These clusters are called islets of Langerhans. When a tumor forms in one of these islets, it’s called a pancreatic NET. These tumors can be either benign (non-cancerous) or malignant (cancerous). Both types can cause serious health problems.

There are five types of pancreatic NETs that are based on the hormones they make:

• Gastrinoma -Gastrin is a hormone that causes the stomach to make acid to help digest food. A tumor in gastrin-making cells is called a gastrinoma. About half of these tumors are cancer.
• Insulinoma -This tumor starts in the cells that make insulin. Insulin helps control the amount of sugar (glucose) in the blood. Most of these tumors are not cancer.
• Glucagonoma - Glucagon also plays a role in the amount of glucose in the blood. It increases the amount of glucose present. A glucagonoma starts in a group of glucagon-making cells. Most of them are cancer.
• VIPoma - These tumors start in cells that make vasoactive intestinal peptides. They make hormones that control the balance of water in your body. Most of them are cancer.
• Somatostatinoma - Somatostatin is a hormone that stops the body from making other hormones, such as insulin and gastrin. Most of these tumors are cancer.

These tumors may be called functional NETs because they make hormones. The most common types are gastrinomas and insulinomas.

Non-functional pancreatic NETs don’t make hormones. They’re often quite large when they’re found because they don’t cause symptoms. They’re more likely to be cancer than functional tumors.

These tumors are fairly rare. Healthcare providers aren’t exactly sure why people develop them.

A risk factor is anything that may increase your chances of having a disease. The exact cause of someone’s cancer may not be known. But risk factors can make it more likely for a person to have cancer. 

The only known risk factor for pancreatic NETs is not in your control. Having multiple endocrine neoplasia type 1 syndrome (MEN1 syndrome) puts you at greater risk for this cancer. This is a rare genetic disorder. It causes tumors within the pancreas that are often not cancer. But sometimes they can become cancer.

Talk with your healthcare provider if you have these risk factors for pancreatic NETs and what you can do about it.

The symptoms of a pancreatic NET depend on what type you have.

Nonfunctioning pancreatic NETs don’t cause symptoms until they’re big enough to put pressure on other organs, such as the bile ducts.

Other pancreatic NETs cause symptoms because of the overproduction of hormones. Here’s the breakdown of symptoms that you may have based on tumor type.

Nonfunctioning NET:

• Yellowing of the skin or whites of the eyes (jaundice).
• Diarrhea.
• Indigestion.
• Pain in the belly or back.
• A lump in the belly.
• Weight loss.

Gastrinoma:

Symptoms are caused because of too much stomach acid, and include:

• Acid reflux.
• Stomach ulcers.
• Pain in the belly or back.
• Diarrhea.

Insulinoma:

Symptoms are caused by low blood sugar levels, and include:

• Blurred eyesight.
• Weakness.
• Extreme tiredness (fatigue).
• Lightheadedness.
• Shakiness.
• Irritability.
• Hunger.
• Fast heartbeat.

Glucagonoma:

Symptoms are caused by high blood sugars, and include:

• Diarrhea.
• Weight loss.
• Mouth dryness and sores.
• Skin rash or dry skin.
• Headaches.
• Feeling very thirsty and hungry.
• Frequent urination.
• Feeling tired, weak, or dizzy.
• Blood clots.

VIPoma:

• Weight loss.
• Stomach cramps or pain.
• Watery diarrhea.
• Dehydration symptoms.
• Symptoms linked to low potassium levels, such as muscle weakness, aches, cramps, numbness, tingling, frequent urination, thirst, and irregular heartbeat (palpitations).

Somatostatinoma:

• Diarrhea.
• Gallstones.
• Fatty, bad-smelling stools that float.
• Yellowing of the skin or eyes.
• Unexplained weight loss.
• High blood sugar symptoms, such as weakness, hunger, confusion, headaches, frequent urination, and dry skin.

Many of these may be caused by other health problems. But it’s important to see your healthcare provider if you have these symptoms. Only a healthcare provider can tell if you have a tumor or cancer.

If you have any of these symptoms and your healthcare provider thinks you may have a pancreatic NET, he or she will first ask about your health history, symptoms, and family history. A physical exam will be done. 

You may also need one or more of these tests:

• Blood tests and other lab tests. These can show high hormone levels in the blood.
• Imaging tests. These are used to look for the tumor inside the pancreas. They include CT, MRI, ultrasound, and somatostatin receptor scintigraphy (SRS).

A biopsy is the only way to know if the tumor is cancer. Small pieces of tissue are taken out and checked for cancer cells. 

After a diagnosis of pancreatic NET, you’ll likely need more tests. These help your healthcare providers learn more about the exact type of cancer. They can help figure out how fast the cancer is likely to grow and the stage of cancer. The stage is how much and how far the cancer has spread (metastasized) in your body. It’s one of the most important things to know when deciding how to treat the cancer.  

Once your cancer is staged, your healthcare provider will talk with you about what the stage means for your treatment. Ask your healthcare provider to explain the stage of your cancer to you in a way you can understand.

Your treatment choices depend on the type of pancreatic NET you have, test results, if the tumor is cancer, and the stage of the cancer. The goal of treatment may be to cure you, control the tumor or cancer, or to help ease problems caused by the tumor or cancer. Talk with your healthcare team about your treatment choices, the goals of treatment, and what the risks and side effects may be.

Types of treatment for cancer are either local or systemic. Local treatments remove, destroy, or control cancer cells in one area. Surgery and radiation are local treatments. Systemic treatment is used to destroy or control cancer cells that may have traveled around your body. When taken by pill or injection, chemotherapy is a systemic treatment. You may have just one treatment or a combination of treatments.

Surgery is the most common treatment, if the tumor can be removed. The type of surgery depends on how big the tumor is and where it is in the pancreas. Nearby tissues, such as parts of the pancreas, stomach, small intestine, or liver may also need to be removed.

For tumors that can’t be removed by surgery, or that have spread to other parts of the body, other treatments may be used to shrink the tumor, destroy it, or control the symptoms it’s causing. These are:

• Active surveillance if the tumor is slow-growing and not cancer.
• Chemotherapy.
• Hormone therapy.
• Targeted therapy.
• Using heat to destroy the tumor (radiofrequency ablation).
• Freezing the cancer cells (cryosurgical ablation).

Your healthcare provider uses medicines to help manage your symptoms from the pancreatic NET. These may include:

• Medicines for stomach ulcers.
• Therapy or over-the-counter remedies to deal with diarrhea.
• Medicines or other strategies to keep your blood sugar in a healthy range.

Talk with your healthcare providers about your treatment options. Make a list of questions. Think about the benefits and possible side effects of each option. Talk about your concerns with your healthcare provider before making a decision.

Call your healthcare provider if you have: 

• New symptoms or symptoms that get worse.
• Signs of an infection, such as a fever.