Pancreatic neuroendocrine tumors form in hormone-making cells (islet cells) of the pancreas. The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine.

Pancreatic neuroendocrine tumors (NETs) may be benign (not cancer) or malignant (cancer). When pancreatic NETs are malignant, they are called pancreatic endocrine cancer or islet cell carcinoma. Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis.

Signs & Symptoms

Signs or symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes or by other conditions. Some tumors may not cause signs or symptoms. Check with your doctor if you have any of these problems.

A non-functional pancreatic NET may grow for a long time without causing signs or symptoms. It may grow large or spread to other parts of the body before it causes signs or symptoms, such as:

  • Diarrhea
  • Indigestion
  • A lump in the abdomen
  • Pain in the abdomen or back
  • Yellowing of the skin and whites of the eyes
  • Signs and symptoms of a functional pancreatic NET

The signs and symptoms of a functional pancreatic NET depend on the type of hormone being made.

  • Too much gastrin may cause:
    • Stomach ulcers that keep coming back
    • Pain in the abdomen, which may spread to the back. The pain may come and go and it may go away after taking an antacid.
    • The flow of stomach contents back into the esophagus (gastroesophageal reflux)
    • Diarrhea
  • Too much insulin may cause:
    • Low blood sugar. This can cause blurred vision, headache, and feeling lightheaded, tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry.
    • Fast heartbeat
  • Too much glucagon may cause:
    • Skin rash on the face, stomach, or legs
    • High blood sugar. This can cause headaches, frequent urination, dry skin and mouth, or feeling hungry, thirsty, tired, or weak.
    • Blood clots in the lung can cause shortness of breath, cough, or pain in the chest. Blood clots in the arm or leg can cause pain, swelling, warmth, or redness of the arm or leg.
    • Diarrhea
    • Weight loss for no known reason
    • Sore tongue or sores at the corners of the mouth
  • Too much vasoactive intestinal peptide (VIP) may cause:
    • Very large amounts of watery diarrhea
    • This can cause feeling thirsty, making less urine, dry skin and mouth, headaches, dizziness, or feeling tired.
    • Low potassium level in the blood. This can cause muscle weakness, aching, or cramps, numbness and tingling, frequent urination, fast heartbeat, and feeling confused or thirsty.
    • Cramps or pain in the abdomen
    • Weight loss for no known reason
  • Too much somatostatin may cause:
    • High blood sugar. This can cause headaches, frequent urination, dry skin and mouth, or feeling hungry, thirsty, tired, or weak.
    • Diarrhea
    • Steatorrhea (very foul-smelling stool that floats).
    • Gallstones
    • Yellowing of the skin and whites of the eyes
    • Weight loss for no known reason

Treatment

There are six types of standard treatment. Your OHC doctor will help you determine the best care plan for you.

  • Surgery: An operation may be done to remove the tumor
  • Chemotherapy: a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing
  • Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing
  • Hepatic arterial occlusion uses drugs, small particles, or other agents to block or reduce the flow of blood to the liver through the hepatic artery (the major blood vessel that carries blood to the liver). This is done to kill cancer cells growing in the liver. The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine.
  • Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells.
  • Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for pancreatic NETs may include treatment for the following:
    • Stomach ulcers may be treated with drug therapy such as proton pump inhibitor drugs such as omeprazole, lansoprazole, or pantoprazole; histamine blocking drugs such as cimetidine, ranitidine, or famotidine; or somatostatin-type drugs such as octreotide.
    • Diarrhea may be treated with intravenous (IV) fluids with electrolytes such as potassium or chloride or somatostatin-type drugs such as octreotide.
    • Low blood sugar may be treated by having small, frequent meals or with drug therapy to maintain a normal blood sugar level.
    • High blood sugar may be treated with drugs taken by mouth or insulin by injection.