MEN Type - 1

Multiple endocrine neoplasia type 1 (MEN1) is a rare genetic disorder that mainly affects the endocrine glands NIH external link. Located in different parts of the body, these glands control the production of hormones that direct many body processes, including growth, digestion, and sexual function.

Causes of MEN Type - 1 #

• MEN1 is an inherited disorder most often caused by a mutation in the MEN1 gene.

• The gene provides instructions for producing a protein called menin, known to play a role in keeping cells from growing and dividing too fast.

• MEN1 is an autosomal dominant disorder.

• This means that only one parent needs to have the defective gene to pass the disorder on to a child.

• If one parent has the MEN1 gene, each child has a 1 in 2 (50%) chance of having the disorder.

• In about 1 in 10 cases, the mutation is not inherited from either parent but develops on its own.

• This is a natural, random process that can occur in anyone.

Symptoms of MEN Type - 1 #

• Kidney stones

• Muscle weakness

• Tiredness

• Increased thirst and urination

• Depression NIH external link

• Aches and pains in bones and joints

• Digestive problems and constipation

• Stomach ulcers

• Acid reflux

• Abdominal pain

• Frequent diarrhea

• Low blood glucose

• Enlarged and swollen hands and feet

Diagnosis of MEN Type - 1 #

Your doctor will diagnose you as having MEN1 if you meet one of these three criteria

• Two or more MEN1-related tumors (tumors in parathyroid glands, pituitary gland, and pancreas, or other part of the digestive tract)

• One MEN1-related tumor and a first-degree relative (a parent, brother or sister, or child) who has been clinically diagnosed as having MEN1

• A MEN1 mutation, even if you have no signs or symptoms of MEN1

Blood tests #

These tests will help your doctor monitor levels of hormones and other substances linked to MEN1-related tumors. Examples include calcium and parathyroid hormone, prolactin, gastrin, and markers for certain tumors.

Imaging tests #

• Your doctor may order imaging tests to monitor the size and growth of existing tumors and to detect new ones, including tumors that don’t release hormones or cause symptoms.

• These tests include magnetic resonance imaging (MRI), computed tomography (CT) scan, and ultrasound NIH external link.

• Other tests that look for abnormal hormone receptors—proteins that attach to certain hormones—on the surface of tumors can help detect tumors that may not be visible on an MRI or CT scan.

Treatment of MEN Type - 1 #

• Parathyroid tumors are most often treated with surgery to remove the affected glands. If surgery is not possible, your doctor may prescribe medicines to control calcium levels.

• Tumors of the pancreas and digestive tract are often treated with medicines to control symptoms such as too much stomach acid.

• Other treatment options include surgery to remove the tumor(s), freezing or burning tumors that have spread to the liver without removing them, and, more rarely, systemic chemotherapy treatment with anticancer drugs that travel through the blood to cells all over your body.

• Pituitary gland tumors are most often treated with medicines and/or surgery.

• Radiation therapy may also be used, but more rarely.

• Treating multiple tumors - People with MEN1 often develop many tumors at the same time. As a result, treatment is more complicated than among people who have a single tumor or very few tumors. Sometimes, MEN1-related tumors may be larger, more aggressive, and resistant to treatment than other tumors.