Pancreatic neuroendocrine tumors (pNET) are a group of cancers that can occur in the hormone-producing cells of the pancreas. Pancreatic neuroendocrine tumors, also known as islet cell cancers, are very rare.
Pancreatic neuroendocrine tumors begin in small hormone-producing cells (islet cells) normally found in your pancreas.
Some pancreatic neuroendocrine tumor cells continue to secrete hormones (known as functional tumors), creating too much of the given hormone in your body. Examples of these types include gastrinoma and glucagonoma.
Many times these tumors do not secrete an excess amount of these hormones (known as nonfunctional tumors).
Pancreatic neuroendocrine tumors sometimes don't cause symptoms. When they do, signs and symptoms can include:
It's not clear what causes most pancreatic neuroendocrine tumors.
Pancreatic neuroendocrine tumors occur when hormone-producing cells in the pancreas (islet cells) develop changes (mutations) in their DNA — the material that provides instructions for every chemical process in your body. DNA mutations cause changes in these instructions. One result is that cells may begin to grow out of control and eventually form a tumor — a mass of cancerous cells. Sometimes the cancer cells break away and spread to other organs, such as the liver.
Some inherited syndromes increase the risk of this type of cancer, including:
Factors that are associated with an increased risk of pancreatic neuroendocrine tumors include:
Tests and procedures used to diagnose pancreatic neuroendocrine tumors include:
Imaging tests. Imaging tests help your doctor look for abnormalities in your pancreas. Tests may include ultrasound, CT and MRI.
Imaging might also be done with nuclear medicine tests, which involve injecting a radioactive tracer into your body. The tracer sticks to pancreatic neuroendocrine tumors so that they show clearly on the images, which may be created with positron emission tomography (PET) that's combined with CT or MRI.
Treatment for a pancreatic neuroendocrine tumor varies based on the types of cells involved in your cancer, the extent and characteristics of your cancer, your preferences, and your overall health.
Options may include:
Surgery. If the pancreatic neuroendocrine tumor is confined to the pancreas, treatment usually includes surgery. For cancers that occurs in the tail of the pancreas, surgery may involve removing the tail of the pancreas (distal pancreatectomy), leaving the head portion intact.
Cancers that affect the head of the pancreas may require a Whipple procedure (pancreaticoduodenectomy), which involves removing the cancer and part or most of your pancreas.
Peptide receptor radionuclide therapy (PRRT). PRRT combines a drug that targets cancer cells with a small amount of a radioactive substance that's injected into a vein. The medication sticks to the pancreatic neuroendocrine tumor cells hiding in the body. Over days to weeks, the medication delivers radiation directly to the cancer cells, causing them to die.
One PRRT, lutetium Lu 177 dotatate (Lutathera), is used to treat advanced cancers.
With time, you'll find what helps you cope with the uncertainty and distress of a cancer diagnosis. Until then, you may find that it helps to:
Find someone to talk with. Find a good listener who is willing to hear you talk about your hopes and fears. This may be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group also may be helpful.
Ask your doctor about support groups in your area. Other sources of information include the National Cancer Institute and the American Cancer Society.
Make an appointment with your doctor if you have any signs or symptoms that worry you. If your doctor suspects that you might have cancer, he or she may refer you to a specialist.
Here's some information to help you get ready for your appointment.
When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a specific test.
Gather the medical records that pertain to your condition and bring them to your appointment. If you're seeing a new doctor, ask your previous doctor to forward files and other information, such as glass slides that contain tissue samples, to your new doctor.
Make a list of:
Consider bringing a family member or friend to help you remember the information you're given.
For pancreatic neuroendocrine tumor, some basic questions to ask your doctor include:
Don't hesitate to ask other questions.
Your doctor is likely to ask you several questions, such as:
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