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:
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:
Surgery to remove the diseased gland is often the treatment of choice. A medicine called 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.
William Simonds is an Endocrinologist and a Hospital Medicine doctor in Bethesda, Maryland. Dr. Simonds has been practicing medicine for over 42 years and is rated as an Elite doctor by MediFind in the treatment of Multiple Endocrine Neoplasia Type 1. He is also highly rated in 9 other conditions, according to our data. His top areas of expertise are Multiple Endocrine Neoplasia Type 1, Multiple Endocrine Neoplasia, Parathyroid Adenoma, and Parathyroid Cancer. Dr. Simonds is currently accepting new patients.
Smita Jha is a Hospital Medicine specialist and an Endocrinologist in Bethesda, Maryland. Dr. Jha has been practicing medicine for over 16 years and is rated as a Distinguished doctor by MediFind in the treatment of Multiple Endocrine Neoplasia Type 1. She is also highly rated in 10 other conditions, according to our data. Her top areas of expertise are Melorheostosis, Multiple Endocrine Neoplasia Type 1, Multiple Endocrine Neoplasia, and Parathyroid Cancer. Dr. Jha is currently accepting new patients.
Richard Santen is an Endocrinologist and a Hematologist Oncology doctor in Charlottesville, Virginia. Dr. Santen has been practicing medicine for over 58 years and is rated as an Advanced doctor by MediFind in the treatment of Multiple Endocrine Neoplasia Type 1. He is also highly rated in 15 other conditions, according to our data. His top areas of expertise are Breast Cancer, Menopause, Multiple Endocrine Neoplasia, Hormone Replacement Therapy (HRT), and Orchiectomy. Dr. Santen is currently accepting new patients.
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.
Background: Parathyroid disorders are very common in the general population and include disorders of parathyroid excess, deficiency, or defects in parathyroid hormone (PTH) signaling. PTH, the main secretory product of parathyroid glands is responsible for regulation of calcium-phosphate homeostasis.
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: January 25, 2022
Published By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, 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 1.2022. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Updated May 23, 2022. Accessed August 23, 2022.
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.
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Thakker RV. Multiple endocrine neoplasia type 1. In: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Adult and Pediatric. 7th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 148.