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Most common tumor causing hypoglycemia.
Most common type of islet-cell tumor.
Insulinomas are insulin producing tumors that arise from the beta cells of the pancreas.
Incidence 1 in 4 per 100,000.
Median age is 50 years at the time of surgery.
57% of patients are women.
Most cases are sporadic and tend to occur in patients older than 40 years.
Greater than 90% are benign, can be sporadic, or occur as part of MEN 1 syndrome.
Patients with multiple insulinomas are at an increased risk of having the multiple endocrine neoplasia type 1 syndrome.
Up to 6% of insulinomas are associated with multiple endocrine neoplasia type I, an autosomal dominant disorder characterized by two or more tumors of the parathyroid gland, pituitary gland, or pancreatic islet cells.
Nearly all patients with insulinomas present with hypoglycemia symptoms, which can be progressive, and often provoked by a fasting state.
Hypoglycemic symptoms of insulinoma rarely occur in a postprandial state.
Prevention of hypoglycemia is the primary goal in its management, which can be achieved by debulking the primary tumor and metastatic disease by surgery and/or liver directed therapies.
About 90 percent are benign and amenable to surgical removal.
5 year overall survival rate of approximately 50% when metastases are present at diagnosis.
The tumor grade, influence his overall survival, low-grade tumors have a better outcome than intermediate and high-grade tumors.
In most cases surgical resection of the tumor is the treatment of choice.
Resection is curable in nearly 90% of patients.
8-9% may experience postoperative hypoglycemia and up to 6% may experience recurrent hypoglycemia in the future.
Insulinoma is the most common type of functional pancreatic neuroendocrine tumor, accounting for 4 to 20% of resected cases.
Insulinoma almost always located in the pancreas and is more indolent than other functional tumors with metastasis occurring in less than 10% of cases, and they usually associated with tumors that measures 2 cm or more in diameter.
Medication management for patients who refuse surgery or are they too high risk for surgery include using diazoxide or somatostatin analogues.
Diazoxide decreases insulin secretion by activating potassium channels in pancreatic islet cells and can decrease hypoglycemic events by approximately 50 to 59%.
Diazoxide side effects include thrombocytopenia, hirsutism, and fluid overload.
Somatostatin analogues used in the management of insulinoma hypoglycemia has a response rate ranging from 58 to 67%, but can be associated with exacerbation.
Everolimus may control hypoglycemia of insulinoma.
Peptide receptor radionuclide therapy has been increasingly used for insulinoma induced hypoglycemia.