Pancreatic Neuroendocrine Tumors

A functional pancreatic neuroendocrine tumor is named based on the type of hormone it overproduces:

• Insulinoma – This affects the cells that release insulin, the hormone that keeps blood sugar from getting too high.
• Gastrinoma – This affects the cells that release gastrin, the hormone that controls stomach (gastric) acid.
• Glucagonoma – This affects the cells that release glucagon, the hormone that keeps blood sugar from getting too low.
• Somatostatinoma – This affects the cells that release somatostatin, which regulates a variety of body functions by preventing overproduction of other hormones.
• VIPoma – This affects the hormone vasoactive intestinal peptide hormone, which releases the muscles in the stomach and bowels.

Currently, for people of average risk, there are no widely recommended screening tests to detect pancreatic cancer before symptoms occur. If your doctor suspects a pancreatic neuroendocrine tumor, one or more of the following tests may be recommended.

Blood Tests
Labsmay be required to check the levels of several key substances in your blood. Blood tests may be used to look for tumor markers that signal a pancreatic NET, such as elevated level of CA 19-9 or carcinoembryonic antigen (CEA).

If you have jaundice, your doctor will also conduct a liver function test to look at your bilirubin levels.

Octreotide Scan
This non-invasive test uses radioactive dye to look for neuroendocrine tumor cells.

Imaging Tests
Your doctor may conduct one or more imaging tests such as ultrasound, magnetic resonance imaging (MRI), computerized tomography (CT) or positron emission tomography (PET) scans to identify the location of the neuroendocrine tumor and determine whether it has spread to other organs.

• Endoscopic ultrasound: An ultrasound is a type of imaging test that uses sound waves to visualize organs inside the pancreas and detect any abnormalities. An endoscopic ultrasound can detect small pancreatic tumors that could be missed with an MRI. An endoscopic ultrasound is a thin tube with a camera at the end that is passed through the mouth and into the stomach. The ultrasound probe at the end allows imaging of the pancreas, stomach and small intestine. If necessary, an ultrasound-guided biopsy (tissue sample) from the pancreas is taken.
• Magnetic resonance imaging (MRI) scan – An MRI scan uses magnets and radio waves to provide cross-sectional images of the pancreas and nearby organs.
• Computed tomography (CT) scan – A CT scan is a type of imaging test that uses X-rays to create detailed cross-sectional pictures inside the body. The scan creates a 3D view of your pancreas and nearby organs to help your doctor identify tumors.
• Positron emission tomography (PET) scan – For a PET scan, your doctor injects you with a slightly radioactive form of sugar that collects in cancer cells. Then your doctor uses a special camera to take pictures of areas of radioactivity. A PET scan is primarily used to look for cancer spread (metastasis) to help determine the stage of pancreatic cancer.

If you receive a pancreatic neuroendocrine tumor diagnosis, your oncology care team tailors your specific treatment plan to the type of cancer and its characteristics. We also consider your individual needs and lifestyle. Our goal is to provide effective treatments, while preserving healthy tissue and cells.

Depending on the type and stage of the pancreatic neuroendocrine tumor, your care team may use one or more of the following pancreatic cancer treatment options:

• Surgery, including the Whipple procedure (pancreaticoduodenectomy) – This procedure removes the cancer from the pancreas.
• Radiation therapy – This treatment uses high-energy X-rays or other types of radiation to kill cancer cells or keep them from growing in a targeted, specific area.
• Chemotherapy – This treatment uses drugs to stop the growth of cancer cells by killing them or halting cell division and growth.
• Targeted drug therapy – This treatment uses drugs that specifically recognize and target cancer cells to stop their growth without harming normal cells.

Surgery is typically the primary treatment option for pancreatic neuroendocrine tumors. Unlike the more aggressive and common form of pancreatic cancer (adenocarcinoma), pancreatic neuroendocrine tumors can often be removed surgically.

If the tumor is small, removal can sometimes be performed laparoscopically, using only a few small incisions and minimally invasive surgical techniques. If the tumor is larger or if it has spread (metastasized), surgery may still be used but the procedure is more complex.

Before or after surgery, you may also need chemotherapy, radiation or a combination of both (chemoradiation) to kill or stop the growth of the tumor cells.