Multiple endocrine neoplasia type 1 MEN, or also called Wermer’s syndrome, is part of a group of disorders that affect the endocrine system, the network of hormone-producing glands in the body. The hormones are the chemical messengers that travel through the bloodstream and regulate the cells and tissue function throughout the whole body.
Multiple endocrine neoplasia includes tumors in two or more endocrine glands. Tumors can also appear in other organs and tissues of the body. The tumor growths can be benign or malignant. Some cases can result in death if the tumors become cancerous.
There are two forms of multiple endocrine neoplasia. They are called type 1 and type 2. Because of their similar names they are often confused, but they are not the same. They differ by the gene that is involved, the type of hormones that are made and the symptoms they cause.
There are many different types of tumors that can be related to multiple endocrine neoplasia. However, most frequent tumors in multiple endocrine neoplasia type 1 MEN1 are: tumors of the parathyroid gland, the pituitary gland and the pancreas.
Hyperthyroidism is the most common symptom of this disorder. Overactivity of the parathyroid gland interrupts the normal balance of calcium in the blood that can cause: kidney stones, osteoporosis, hypertension, lack of appetite, sickness, weakness, exhaustion and depression.
Neoplasia in the pituitary gland usually causes too much secretion of prolactin and suppression of gonadotropins, which causes a decrease of testosterone. Tumor in the pituitary gland can be quite big and compress the nearby tissues.
Pancreatic tumors that can be associated with MEN-1 neoplasia are usually formed in the beta cells of the islets of Langerhans and cause too much emission of insulin what can result in hypoglycemia. A lot of other tumors can appear as well. Some of them are: tumor in the alpha cells that causes over-secretion of glucagon what can result in hyperglycemia, a rash called necrolytic migratory erythema and weight loss; tumor of the non-beta islet cells or gastrinoma that causes too much oozing of the hormone gastrin what can eventually lead to Zollinger-Ellison syndrome.
The disorder can appear at any age in both men and women equally.
The symptoms that will appear depend on in which gland the tumor appeared and they differ from person to person. Some of the symptoms that can be experienced are:
- Abdominal pain
- Black stool
- Bloating after eating
- Burning feeling in the upper abdomen
- Aching discomfort in the upper abdomen
- Lack of sexual interest
- Loss of appetite
- Lack of menstrual periods (women)
- Loss of body or facial hair (men)
- Pain in the muscles
- Sickness and vomiting
- Confusion or mental changes
- Being sensitive to the cold
- Involuntarily weight loss
- Vision problems
Multiple endocrine neoplasia type 1 MEN occurs when the gene that carries the code for protein called menin is defected. The disorder causes tumors to appear on various glands. The tumors may not appear at the same time.
The risk is higher if there is a history of the disorder in the family.
The complication that exists is that the tumors can keep coming back. It is recommendable for a person experiencing this kind of disorder to have regular examinations at the doctor.
Treatment options for multiple endocrine neoplasia type 1 MEN involve surgery to remove the affected gland or medications for pituitary tumors that help the hormone prolactin to be released.
Also, a hormone replacement therapy can be given when a certain gland has been removed or the gland doesn’t produce enough of the specific protein.
There is no certain way to prevent this disorder from occurring, but it is advisable to know the family history of the disease so examining and screening of close relatives is recommendable.