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Multiple Endocrine Neoplasia Type 1 (MEN1) Syndrome

  • Posted on- Aug 04, 2015
  • 2580 Views

Multiple endocrine neoplasia type 1 (MEN1) is a genetic condition linked with tumors of the endocrine glands. Formerly it was known as Wermer syndrome. Tumors of the parathyroid gland, islet cells of the pancreas and pituitary gland are commonly seen in Multiple endocrine neoplasia type 1 patients. Other tumors include adrenal, cortical carcinoid and tumors of the digestive system.

Some non-endocrine tumors are also associated with MEN1 including:

  • Tumors of blood vessels and fibrous tissue
  • Tumors on the skin
  • Fatty tumors called lipomas
  • Smooth muscle tumors called leiomyomas
  • Tumors from nervous system tissue

Tumors in MEN1 patients are mostly benign in nature. But approximately one-third of islet cell tumors are cancerous (can spread to other parts of the body). However, all tumors can produce excess hormones and there is a wide range of clinical syndromes associated with increased hormone production including:

Causes of Multiple endocrine neoplasia type 1

Since MEN1 is a hereditary condition, the cancer risk and some other features can easily be passed on from generation to generation. A change in MEN1 gene can increase the risk of endocrine tumors. 9 out of 10 people who inherit the MEN1 mutation will develop symptoms(s) of MEN1. Those who fall under exceptions are found to have germline mutations in proteins called cyclin-dependent kinase inhibitors that promote cell growth and division. Currently, worldwide research is going on to gather more information on MEN1.

Screening for Multiple endocrine neoplasia type 1

People who suspect MEN1 can undergo genetic testing. It is recommended for children and younger adults who have a family background of MEN1. In a family where children do not show MEN1 gene mutation may not need the screening tests.

Treatment for Multiple endocrine neoplasia type 1

Most of the tumors are removed with surgery or treated by taking medicines which tames the growth of the tumor. Benign parathyroid tumors are surgically removed. However, pancreatic islet cells tumors pose a challenge to the endocrinologists. The pancreas regulates the level of blood glucose through insulin production along with assisting digestion and removing them opens the opportunity for diabetes mellitus. It will then become inevitable for the patient to take pancreatic enzyme supplements to promote digestion. Endocrinologists must strike a balance between a MEN1 patient and the risks of diabetes mellitus.