Multiple endocrine neoplasia are defined by type. Type 1 affects the parathyroid glands, the pancreatic islets and the anterior pituitary. Associated tumors include lipomas, angiofibromas, or those located in the adrenal gland cortex. Type 2 A is defined by medullary thyroid carcinoma, pheochromocytoma (about 50%), and hyperparathyroidism caused by parathyroid gland hyperplasia (about 20%). Type 2B is defined by medullary thyroid tumor and pheochromocytoma. Associated abnormalities include mucosal neuromas, medullated corneal nerve fibers, and marfanoid habitus.
Insulinomas are rare, insulin-secreting tumors that cause low blood sugar levels that derive from the beta cells of the pancreas. About 99% of insulinomas arise in the pancreas, with 1% arising outside it, but always nearby, such as the duodenum. Nearly 95% are benign. Although called insulinomas, they are often capable of secreting other hormones in addition to insulin. These tumors also occur as part of the type 1 Multiple Endocrine Neoplasia syndrome.
Commonly Prescribed (On-Label) Drugs: None
Off-Label Prescription Drug Breakthrough Option: Diazoxide
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