Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)

Types of Pancreatic Cancer

Cancers that develop within the pancreas fall into two major categories: (1) cancers of the endocrine pancreas (the part that makes insulin and other hormones) are called "islet cell" or "pancreatic neuroendocrine" cancers and (2) cancers of the exocrine pancreas (the part that makes enzymes). Islet cell cancers are rare and typically grow slowly compared to exocrine pancreatic cancers. Islet cell tumors often release hormones into the bloodstream and are further characterized by the hormones they produce (insulin, glucagon, gastrin, and other hormones). Cancers of the exocrine pancreas develop from the cells that line the system of ducts that deliver enzymes to the small intestine and are commonly referred to as pancreatic adenocarcinomas. Adenocarcinoma of the pancreas comprises most of all pancreatic ductal cancers and is the main subject of this review.

Pancreatic neuroendocrine tumors (NETs or islet cell tumors) facts*

*Pancreatic neuroendocrine tumor facts medical author:

  • Pancreatic neuroendocrine tumors are cancer cells that developed from hormone-making cells (islet cells) in the pancreas.
  • Pancreatic NETs have two major categories: endocrine and exocrine. Pancreatic NETs may be benign (noncancerous) or malignant (cancerous).
  • Pancreatic NETs may be functional (make extra amounts of hormones) or nonfunctional (do not make extra amounts of hormones); however, most pancreatic NETs are functional tumors.
  • Functional pancreatic NET types produce different kinds of hormones (for example, gastrin, insulin and glucagon); the main types are gastrinoma, insulinoma, and glucagonoma, with a few other rare types.
  • Experts don't know exactly the cause of pancreatic neuroendocrine tumors, but they speculate that genetics may play a role.
  • Risk factors include family members with NETs, such as multiple endocrine neoplastic types (MEN1 and MEN2) and/or neurofibromatosis type 1 (NF-1).
  • Signs and symptoms of pancreatic neuroendocrine tumors vary considerably. Some patients have no symptoms while other patients develop symptoms very slowly; symptoms that do develop usually depend on the type and volume of hormone being made by the individual pancreatic NET type, but most pancreatic NET types symptoms include diarrhea, indigestion, a lump in the abdomen, jaundice, and pain in the abdomen and/or back.
  • Physicians use physical exam and history plus lab tests and imaging to diagnose pancreatic NETs; some of the test that may be used are blood chemistry, chromogranin A test, CT scan, MRI scan, somatostatin receptor scintigraphy, ERCP (endoscopic retrograde cholangiopancreatography), ultrasound, angiogram, bone scan, laparotomy (surgical incision in the wall of the abdomen), and biopsy. Other tests are used to determine the specific type of pancreatic NET (for example, fasting serum gastrin, insulin, glucagon, somatostatin, and glucose tests).
  • Treatment options and prognosis depend upon the type of cancer cell, its location, it spread, if MEN1 is present, and the patient's age and general health.
  • Staging of pancreatic NETs is not particularly helpful as treatment is not based on stage but is based on where the cancers are found (one or several places in the pancreas or if it is spread to the lymph nodes or other parts of the body).
  • Spread and metastasis of pancreatic NETs can occur through the cancer cells moving within tissues, by the lymph system, and/or through the blood.
  • Recurrent pancreatic neuroendocrine tumors are tumors that have recurred after they had been treated and may come back in the pancreas or one of the other parts of the body.
  • Six types of standard treatment are cited: surgical, chemotherapy, hormone therapy, hepatic arterial occlusion or chemoembolization, targeted therapy, and supportive care.
  • Some patients may benefit from participating in clinical trials that test new types of treatment for pancreatic NETs.
  • Treatments for specific pancreatic NETs and for recurrent pancreatic NETs (for example gastrinoma, insulinoma, glucagonoma, and others) are detailed as to the complex treatment protocols that may involve combinations of surgical and medical therapies.
Medically Reviewed by a Doctor on 6/30/2016

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