Multiple Endrocine Neoplasia type 1, abbreviated as MEN1, is a hereditary condition that causes tumors in the glands of the endocrine system. A rare disorder, around one in 30,000 people has developed MEN1. About 10% of those cases are patients with a new mutation of the gene responsible for causing it. Also called Wermer syndrome, growths typically develop within the pituitary gland, pancreatic islet cells, and parathyroid gland. MEN1 can also produce carcinoid tumors, cortical tumors, and (rarely) masses in the adrenal glands. In some cases, tumors may be found in other areas of the digestive tract.
MEN1 masses form when there is enlargement and over-activity in the affected gland. Most tumors associated with this disorder are noncancerous. However, around one-third of carcinoid and islet cell growths are malignant and spread to other areas of the body, usually the liver, lungs, and lymph nodes. Benign tumors can still create problems when they cause glands to produce too much hormone.
Symptoms associated with the condition vary depending on the location of the tumors. Most of the time, they stem from the overproduction of some type of hormone. Patients with a mass in the parathyroid gland might experience:
Pancreatic tumors are often due to the overproduction of insulin and may produce symptoms such as:
Pituitary tumors sometimes put pressure on the optic nerve, causing symptoms related to changes in vision and headaches. Carcinoid tumors are more likely to show up in the lungs, stomach, thymus or pancreas, and they produce symptoms like flushing, wheezing, and diarrhea.
MEN1 treatment plans are developed around symptoms and the location of the tumor. Usually, therapies involve various medications to regulate hormone production or surgery to remove the tumor. The condition will be monitored regularly to help medical professionals adjust treatment as needed.