Neuroendocrine tumors of the pancreas
Neuroendocrine tumors of the pancreas are among the most common neuroendocrine tumors, but make up only about 3% of pancreatic tumors. It is important to emphasize: there is no link between pancreatic adenocarcinoma and these tumors.
Neuroendocrine tumors of the pancreas are characterized by slow growth, but like other neuroendocrine tumors, they have malignant potential.
Most neuroendocrine tumors of the pancreas are not inherited. However, about one-tenth of patients carry a change in the genetic material that leads to an increased risk of neuroendocrine tumors and sometimes also other endocrine problems and tumors in other organs. An assessment of this risk is made by the attending physician.
Like other neuroendocrine tumors, the neuroendocrine tumors in the pancreas are classified as functional and non-functional. The most common functional tumors in the pancreas are insulinoma and gastrinoma:
The most common functional neuroendocrine tumor in the pancreas. These are usually isolated, small lesions (about 90% of which are not metastatic), well defined, and may be found throughout the pancreas at a similar rate.
These lesions secrete insulin (the hormone responsible for getting sugar from the blood into the cells). The uncontrolled secretion of insulin causes low blood glucose levels (hypoglycemia), which can lead to cognitive disorders to the point of loss of consciousness and convulsions, and sensations such as tremor and sweating. The diagnosis of insulinoma is made by blood tests, and depending on their results, further diagnosis of the tumor is made by various means.
A functional neuroendocrine tumor located at the top of the pancreas and often in the duodenum in an area known as the "gastrinoma triangle".
The tumor secretes the hormone gastrin, which causes acidity in the stomach that can lead to multiple/recurrent ulcers. Gastrin also interferes with the absorption of various substances in the digestive tract, so one of the prominent symptoms of gastrinoma is diarrhea.
Gastrinoma is usually unrelated to genetic syndromes, but its rate in inherited syndromes is relatively high compared to neuroendocrine tumors of the pancreas, and particularly in MEN1 syndrome.