What is Wermer Syndrome?

Wermer syndrome, also known as multiple endocrine neoplasia type 1 or MEN1, is a relatively rare genetic disorder that typically causes multiple endocrine gland tumors, as well as tumors in the duodenum and stomach. Multiple tumors generally form in the pituitary, pancreas, and parathyroid glands of people with Wermer syndrome. These tumors can increase endocrine hormone production, leading to hormonal imbalances and a range of health effects. MEN1 is considered hereditary, and children, parents, or siblings of a person who has it are believed to have a 50% chance of developing it themselves. Symptoms of Wermer syndrome, which can vary widely on a case-by-case basis, do not typically appear until adulthood, and can usually be managed well enough to prevent serious complications from arising.

Most people who develop MEN1 have a family history of the disease, since it is usually hereditary. Parents, siblings, and children of a person diagnosed with this disease are usually also tested for the gene mutation that causes it. Both men and women can develop this disease, and symptoms can appear at any age, though they usually appear in adulthood. The disease seems to occur equally through all races and geographic regions. About one in 30,000 people develop this disease, so it's considered rare.

Multiple endorcine neoplasia type 1 generally causes the growth of multiple tumors in the body's endocrine glands. These glands include the pancreas, parathyroid gland, and pituitary gland. Tumors in the endocrine glands can cause these glands to become overactive, leading to a wide range of health problems due to hormone imbalance.

Tumors in the parathyroid glands can cause hyperparathryoidism, a condition that causes the parathyroid glands to produce excessive amounts of their hormone. High levels of parathyroid hormone in the body can raise blood calcium levels, leading to weakening of bones, kidney stones, and damage to kidney tissue. Other symptoms can include fatigue, weakness, pain in the muscles or bones, indigestion, and constipation.

Pancreatic complications can arise due to tumors in the pancreas and duodenum. Tumors in this area can elevate levels of insulin and glucagon, the hormones that help regulate blood sugar. Elevated insulin levels can lead to diabetes, while elevated glucagon levels can cause hypoglycemia, or dangerously low blood sugar. Stomach tumors can also develop, leading to severe ulceration of the stomach lining and diarrhea. Pituitary tumors in people with Wermer syndrome can cause excessive bone growth and reproductive problems.

Treatment for the tumors associated with Wermer syndrome generally involves surgery. The tumors and, in some cases, the glands themselves may be removed. Radiation therapy is sometimes administered to stop the growth of tumors or reduce their size.