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Endocrine (Neuroendocrine) Tumors
Accounting for less than 5% of all pancreatic tumors are endocrine tumors, also called neuroendocrine or islet cell tumors. The islet cells of the pancreas produce hormones including insulin, glucagon and somatostatin. A major function of these hormones is to control the amount of sugar in the blood. Endocrine tumors may be benign or malignant and tend to be slower growing than exocrine tumors. In addition, these tumors are categorized as functional (produce hormones) or nonfunctional (produce no hormones). The majority of functional endocrine tumors are benign. However, 90% of nonfunctional endocrine tumors are malignant, or cancerous. The following table describes the different types of pancreatic endocrine tumors.
| Types |
Description |
| Gastrinoma (Zollinger-Ellison Syndrome) |
Gastrinomas are often associated with recurrent ulcers, abdominal pain and diarrhea. Diagnosis is dependent on elevated gastrin hormone levels and imaging studies. When this tumor is inherited as part of a genetic syndrome called Multiple Endocrine Neoplasia Type 1 (MEN1) (see below), there may be multiple tumors located within the head of the pancreas and/or the duodenum. Most gastrinomas are malignant or have the ability to become malignant. Improved early screening techniques and increased awareness are leading to increased diagnoses when tumors are still benign. |
| Glucagonoma |
Glucagonomas are most commonly located in the body and tail of the pancreas. They are usually large, often metastasize and approximately 70% are malignant. This type of pancreatic cancer may be associated with a severe skin rash, diabetes and signs of depression or confusion. |
| Insulinoma |
Insulinomas are the most common islet cell tumors and tend to be small and hard to find. They are usually benign and cause low blood sugar. |
| Multiple Endocrine Neoplasia Type-1 (MEN1) (Wermer Syndrome) |
MEN1, or Wermer Syndrome, often involves multiple tumors in the pancreas and parathyroid glands; the tumors in the pancreas may be malignant. Symptoms, such as fatigue, muscle weakness or pain, constipation, kidney stones and thinning of the bones generally appear in individuals in their 30’s and 40’s. |
| Nonfunctional Islet Cell Tumor |
Nonfunctional islet cell tumors do not cause defined clinical symptoms such as overproduction of one or more pancreatic hormone (such as insulin). Most are malignant. |
| Somatostatinoma |
Somatostatinomas can occur anywhere in the pancreas and may also occur at the ampulla of Vater, where the pancreatic duct enters the duodenum. Like most pancreatic endocrine tumors, the potential to become malignant varies. If not removed, this type of tumor can spread to other parts of the body. |
| Vasoactive Intestinal Peptide-Releasing Tumor (VIPoma or Verner-Morrison Syndrome) |
VIPoma, or Verner-Morrison Syndrome, tumors are usually located in the body and tail of the pancreas. They are often associated with severe diarrhea and the resulting loss of large amounts of potassium (hypokalemia). Two-thirds of VIPomas are found in women. The syndrome is also known as Watery Diarrhea and Hypokalemia Achlorhydria (WDHA) Syndrome. |
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