Multiple Endocrine Neoplasia Type 1 (MEN 1)

Overview

Mayo Clinic has extensive experience treating multiple endocrine neoplasia type 1 (MEN 1), a rare, complex genetic disorder that can result in various tumors throughout the body. Mayo physicians evaluate 10 to 15 new patients annually, in addition to providing follow-up care to scores of patients who have MEN 1.

Mayo Clinic specialists work as a team to listen, counsel and treat patients who have this complicated disease. Specialists involved with care of MEN 1 patients include endocrinologists, endocrine surgeons, gastroenterologists, neurosurgeons, oncologists, neuro-oncologists and geneticists. Ongoing research projects at Mayo examine the best screening methods and treatment options for MEN 1 patients.

Diagnosis

Mayo specialists diagnose MEN 1 in probands (first family member to be identified) by documenting two of the three major manifestations (parathyroid, pancreatic neuroendocrine or pituitary tumors) or one major proband manifestation in a family member. Specialists confirm the presence of MEN 1 through genetic testing which is positive in more than 90 percent of MEN 1 patients. The disease can manifest in many ways. Multiple imaging and diagnostic methods are necessary to evaluate the disease and its progression. Genetic screening and consultation is available for individuals suspected of being at risk for MEN 1. Read more about MEN 1 diagnosis.

Treatment Options

Treatment depends on the problem or symptom. Pituitary tumors, primary hyperparathyroidism, pancreatic tumors and other MEN 1-related conditions require different treatment approaches. In the disease's early stages, Mayo specialists may consider medical and surgical therapy to manage the tumors. Mayo Clinic is experienced in procedures such as endonasal pituitary surgery, surgery for primary hyperparathyroidism, and pancreatoduodenal surgery for MEN 1.

When the cancer has spread, radiation, chemotherapy and other specialty care such as radiofrequency ablation may be used. Read more about multiple endocrine neoplasia.

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About Multiple Endocrine Neoplasia Type 1

MEN 1 is an inherited disorder that can cause tumors in at least eight endocrine and non-endocrine tissues. Children of an affected parent have a 50 percent chance of being predisposed to the disease. The gene associated with MEN 1 is found on the long arm of chromosome 11 (11q13), a tumor suppressor gene. The incidence of MEN 1 varies from 0.2 to 2 per 100,000 people. Major clinical symptoms of MEN 1 include primary hyperparathyroidism (seen in more than 95 percent of patients), pancreatic endocrine tumors (seen in 50 to 75 percent), and pituitary tumors (seen in 30 to 55 percent). The disease rarely expresses itself prior to age 10. Most often, the condition appears between the ages of 20 and 40.