Insulinoma

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Disease Overview

Insulinoma is a type of pancreatic neuroendocrine tumor (pancreatic NET), which refers to a group of rare tumors that form in the hormone-making cells of the pancreas. Insulinomas, specifically, produce too much insulin, a hormone that reduces the level of sugar in the blood by helping it move into cells. As a result, people with insulinomas generally have very low blood sugar levels which can be associated with anxiety, confusion, hunger, a fast heart rate, and sweating. In severe cases, it can lead to seizures, coma or even death.[11357][11359][11360] Ninty percent of insulinomas are benign (noncancerous). In most cases, the underlying cause of insulinoma is unknown. However, people with specific genetic syndromes such as multiple endocrine neoplasia type I, Von Hippel-Lindau syndrome, Neurofibromatosis type 1, and tuberous sclerosis are at risk of insulinomas and other endocrine tumors.[11358][11357] Treatment generally includes surgery to remove the tumor.[11359][11360]



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National Organization for Rare Disorders