Multiple Endocrine Neoplasia Type 1 Overview
Learn About Multiple Endocrine Neoplasia Type 1
Multiple endocrine neoplasia (MEN) type I is a disease in which one or more of the endocrine glands are overactive or forms a tumor. It is passed down through families.
Endocrine glands most commonly involved include:
- Pancreas
- Parathyroid
- Pituitary
Wermer syndrome; MEN I
MEN I is caused by a defect in a gene that carries the code for a protein called menin. The condition causes tumors of various glands to appear in the same person, but not necessarily at the same time.
The disorder may occur at any age, and it affects men and women equally. A family history of this disorder raises your risk.
Symptoms vary from person to person, and depend on which gland is involved. They may include:
- Abdominal pain
- Anxiety
- Black, tarry stools
- Bloated feeling after meals
- Burning, aching, or hunger discomfort in the upper abdomen or lower chest that is relieved by antacids, milk, or food
- Decreased sexual interest
- Fatigue
- Headache
- Lack of menstrual periods (in women)
- Loss of appetite
- Loss of body or facial hair (in men)
- Mental changes or confusion
- Muscle pain
- Nausea and vomiting
- Sensitivity to the cold
- Unintentional weight loss
- Vision problems
- Weakness
Surgery to remove the diseased gland is often the treatment of choice. Medicines such as cabergoline and bromocriptine may be used instead of surgery for pituitary tumors that release the hormone prolactin.
The parathyroid glands, which control calcium production, can be removed. However, it is difficult for the body to regulate calcium levels without these glands, so a total parathyroid removal is not done first in most cases.
Medicine is available to decrease the excess stomach acid production caused by some tumors (gastrinomas), and to reduce the risk of ulcers.
Hormone replacement therapy is given when entire glands are removed or do not produce enough hormones.
University Surgical Associates Llp
Mashaal Dhir is a General Surgeon and a Surgical Oncologist in Syracuse, New York. Dr. Dhir and is rated as an Experienced provider by MediFind in the treatment of Multiple Endocrine Neoplasia Type 1. His top areas of expertise are Pancreatic Cancer, Familial Pancreatic Cancer, Hyperparathyroidism, Pancreatectomy, and Small Bowel Resection.
Upstate Urology, Inc.
Gennady Bratslavsky is an Urologist and a Surgical Oncologist in Syracuse, New York. Dr. Bratslavsky and is rated as an Experienced provider by MediFind in the treatment of Multiple Endocrine Neoplasia Type 1. His top areas of expertise are Renal Cell Carcinoma (RCC), Pheochromocytoma, Prostate Cancer, Nephrectomy, and Reconstructive Urology Surgery.
St Peters Hospital Of The City Of Albany
Ronnie Luyun is an Oncologist and a Hematologist in Albany, New York. Dr. Luyun and is rated as an Experienced provider by MediFind in the treatment of Multiple Endocrine Neoplasia Type 1. His top areas of expertise are Chondrosarcoma, Osteosarcoma, Paget Disease of the Breast, and Breast Cancer.
Pituitary and parathyroid tumors are usually noncancerous (benign), but some pancreatic tumors may become cancerous (malignant) and spread to the liver. These can lower life expectancy.
The symptoms of peptic ulcer disease, low blood sugar, excess calcium in the blood, and pituitary dysfunction usually respond well to appropriate treatment.
The tumors can keep coming back. Symptoms and complications depend on which glands are involved. Regular check-ups by your provider are essential.
Contact your provider if you notice symptoms of MEN I or have a family history of this condition.
Screening close relatives of people affected with this disorder is recommended.
Summary: Multiple Endocrine Neoplasia type 1 (MEN1) is an autosomal dominant disease with a high degree of penetrance (\>80% of patients). It is caused by the presence of the MEN1 mutation located on chromosome 11q13. The prevalence of this mutation is estimated at approximately 1/30,000. This hereditary syndrome is characterized by the presence of tumours of the endocrine system (adenoma of the parathyroi...
Summary: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder due to mutations in the tumor suppressor gene MEN1 with the corresponding gen product menin. MEN1 is characterized by the occurrence of parathyroid, pancreatic islet and anterior pituitary tumors which can release excessive amounts of hormones (= functional active tumors). Other tumors (e.g. carcinoid tumors, adrenocortic...
Published Date: March 31, 2024
Published By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
National Comprehensive Cancer Network website. Clinical practice guidelines in oncology (NCCN guidelines): neuroendocrine and adrenal tumors. Version 1.2023. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Updated August 2, 2023. Accessed May 7, 2024.
Newey PJ, Thakker RV. Multiple endocrine neoplasia. In: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. Williams Textbook of Endocrinology. 14th ed. Philadelphia, PA: Elsevier; 2020:chap 42.
Newey PJ, Thakker RV. Multiple endocrine neoplasia type 1. In: Robertson RP, ed. DeGroot's Endocrinology. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 133.
Nieman LK, Spiegel AM. Polyglandular disorders. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 212.