This disease is inherited in an autosomal dominant fashion.
Menin is coded by the MEN 1 gene and is a tumor suppressor gene. This mutation on chromosome 11 leads to tumor growth.
This mutation on chromosome 11 leads to MEN 1 syndrome.
Tumors of the pancreas as well as duodenum are one of the culprits of MEN1. There are several types of tumors in the pancreas including gastrinomas, insulinomas, VIPomas, and glucagonomas. Depending on the type of pancreatic tumor, patients can present in a variety of ways including ulcers, hypoglycemia, or hyperglycemia.
Pituitary tumors are characteristic of MEN1 and are most commonly prolactinomas. Growth hormone tumors can also occur. Enlargement of a pituitary tumor can compress on the optic chiasm causing bitemporal hemianopia.
Parathyroid tumors are characteristically involved with MEN1 and present as hypercalcemia. Many times, patients will present with kidney stones as a consequence of prolonged hypercalcemia.
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