Endocrine Pancreatic Cancer

Endocrine cells produce hormones. They make up less than five percent of the pancreas.

Neuroendocrine tumors, which can be benign or malignant, form in the islet cells and may be functional (hormone-producing) or nonfunctional.

Found in structures called the islets of Langerhans, these cells release hormones directly into the bloodstream.

If a tumor affects the endocrine function of the pancreas, blood sugar levels rise. This eventually causes diabetes.

Pancreatic endocrine tumors are less common than exocrine pancreatic cancer and have a better prognosis.

There is no specific cause of neuroendocrine tumors, but there are risk factors, including genetic history and preventable factors.

Both preventable and non-preventable factors can raise the risk of neuroendocrine tumors. These include:

• The hereditary condition multiple endocrine neoplasia type 1 (MEN1), which can cause tumors in the pituitary and parathyroid glands and pancreas. While usually not cancerous, these can cause other health problems such as kidney stones, fertility issues, and severe ulcers.
• Other genetic conditions such as neurofibromatosis, type 1, and Von Hippel-Lindau syndrome, can increase the risk for tumors.
• Smoking
• Heavy alcohol use
• Diabetes
• Chronic pancreatitis

There are two types of pancreatic neuroendocrine tumors:

• Functional - produce hormones and cause hormone-related symptoms
• Nonfunctional - do not cause hormone-related symptoms.

Tumors can create various hormones, which carry their own related symptoms. These include:

• Carcinoid tumors: Flushing, diarrhea, wheezing, fast heart rate, shortness of breath, weakness, heart murmurs
• Gastrinoma, or Zollinger-Ellison Syndrome: Acid reflux, burning abdominal pain, diarrhea, excess fat in the stools, weight loss
• Glucaganoma: High blood sugar, which sometimes leads to diabetes, skin swelling or irritation, mouth sores, anemia, diarrhea, weight loss
• Insulinoma: Low blood sugar, which can produce heart palpitations, sweating, confusion, weakness, and seizures
• Somatostatinoma: Diabetes, gallstones, loss of appetite, nausea, weight loss, diarrhea, jaundice
• VIPomas: Diarrhea, nausea, vomiting, muscle cramps, weakness, flushing, digestion problems

Because nonfunctional pancreatic neuroendocrine tumors do not produce hormones, their symptoms often do not show until the tumor grows large. Symptoms include:

• Jaundice
• Stomach pain
• Loss of appetite
• Weight loss

Treatment for endocrine pancreatic cancer depends on where the tumor is and whether it has spread. Options include:

• Surgery
• Radiation
• Chemotherapy
• Ablation or embolization treatments
• Targeted therapy
• Drug therapy
• Clinical trials