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Cancer Medicine :: Multiple Endocrine Neoplasia Type 1

Multiple Endocrine Neoplasia Type 1

MEN1 is suspected when a person has at least two of the most common tumors listed.

Parathyroid

Pancreatic

Pituitary

If a person has a family history of MEN1, he or she is suspected of also having MEN1 if diagnosed with a parathyroid, pancreatic, or pituitary tumor. Genetic testing for mutations in the MEN1 gene is available for people suspected to have MEN1. A mutation in the MEN1 gene is found in about 80% to 90% of families diagnosed with MEN1. Approximately 65% of people with two or more tumors associated with MEN1, but no family history, will have a mutation in the MEN1 gene. 

What are the screening options for MEN1?

Current suggested screening for people who are known or suspected to have MEN1 includes:

Genetic Testing

Genetic testing is available. It should be considered for children or young adults who are members of a family diagnosed with MEN1 and an identified mutation of the MEN1 gene to determine which children/young adults should have the screening studies described below. In a family with an identified mutation of the MEN1 gene, children with a genetic test showing no mutation (expected to be 50% of children born to an individual affected with MEN1) may not need the screening tests described below. 

Diagnostic Studies

Regular blood tests (every one to three years) for serum prolactin, insulin-like growth factor 1 (IGF-1) level, fasting glucose, insulin, and proinsulin, beginning at age 5 to 10 years.

A yearly ionized or albumin-corrected calcium level test, beginning at age 8

Regular blood tests for fasting gastrin level and fasting and meal stimulated pancreatic polypeptide (PP), fasting VIP, and glucagon, beginning at age 20.

Magnetic resonance imaging(MRI) scan of the brain, every three to five years, beginning between ages 5 to 10, or at any time the results of the tests for serum prolactin or insulin-like growth factor is abnormal.

MRI or computed tomography (CT) scan of the chest and abdomen, every two to four years, beginning at age 20 or when the serum gastrin, PP, or VIP is noted to be abnormal.

Screening guidelines may change over time as new technologies are developed and more is learned about MEN1. It is important to talk with your doctor about appropriate screening tests.

What are the treatment options for the endocrine tumors?

Most of these tumors are treated with surgery or by taking a medicine that suppresses growth or function of the tumor. Parathyroid tumors, almost always benign, should be surgically removed when the albumin-corrected serum calcium level is greater than 12 mg/dl, there is significant bone loss, or kidney damage or stones develop. The most challenging treatment issues relate to the pancreatic islet cell tumors. In addition to its role in normal digestion, the pancreas regulates the level of blood glucose through insulin production. Removal of the pancreas will cause diabetes mellitus, a condition that can lead to significant health problems. Doctors must balance the benefits of pancreatic removal in a person with MEN1 (such as the prevention of development of cancer spread) against the risks of diabetes mellitus. Carcinoid tumors are typically removed by surgery as soon as possible. 

Pituitary tumors producing prolactin (a hormone) are most commonly managed with dopamine agonists (drugs that imitate the action of dopamine; a naturally occurring substance produced in the brain); tumors that produce growth hormones or adrenocorticoptropin hormones or non-functioning tumors are most commonly treated with surgery. Two hormonal agents, a somatostatin analogue and a growth hormone antagonist, have been successfully used to treat growth hormone excess in patients who are not cured by surgery.

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