Multiple endocrine neoplasia
Last updated 18 Aug 2020
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Multiple endocrine neoplasia is a group of disorders that affect the body's network of hormone-producing glands called the endocrine system. Hormones are chemical messengers that travel through the bloodstream and regulate the function of cells and tissues throughout the body. Multiple endocrine neoplasia typically involves tumours (neoplasia) in at least two endocrine glands; tumours can also develop in other organs and tissues. These growths can be noncancerous (benign) or cancerous (malignant). If the tumours become cancerous, the condition can be life-threatening.
Description
Multiple endocrine neoplasia is a group of disorders that affect the body's network of hormone-producing glands called the endocrine system. Hormones are chemical messengers that travel through the bloodstream and regulate the function of cells and tissues throughout the body. Multiple endocrine neoplasia typically involves tumours (neoplasia) in at least two endocrine glands; tumours can also develop in other organs and tissues. These growths can be noncancerous (benign) or cancerous (malignant). If the tumours become cancerous, the condition can be life-threatening.
The major forms of multiple endocrine neoplasia are called type 1, type 2, and type 4. These types are distinguished by the genes involved, the types of hormones made, and the characteristic signs and symptoms.
Many different types of tumours are associated with multiple endocrine neoplasia. Type 1 frequently involves tumours of the parathyroid glands, the pituitary gland, and the pancreas. Tumours in these glands can lead to the overproduction of hormones. The most common sign of multiple endocrine neoplasia type 1 is overactivity of the parathyroid glands (hyperparathyroidism). Hyperparathyroidism disrupts the normal balance of calcium in the blood, which can lead to kidney stones, thinning of bones, nausea and vomiting, high blood pressure (hypertension), weakness, and fatigue.
The most common sign of multiple endocrine neoplasia type 2 is a form of thyroid cancer called medullary thyroid carcinoma. Some people with this disorder also develop a pheochromocytoma, which is an adrenal gland tumour that can cause dangerously high blood pressure. Multiple endocrine neoplasia type 2 is divided into three subtypes: type 2A, type 2B (formerly called type 3), and familial medullary thyroid carcinoma (FMTC). These subtypes differ in their characteristic signs and symptoms and risk of specific tumours; for example, hyperparathyroidism occurs only in type 2A, and medullary thyroid carcinoma is the only feature of FMTC. The signs and symptoms of multiple endocrine neoplasia type 2 are relatively consistent within any one family.
Multiple endocrine neoplasia type 4 appears to have signs and symptoms similar to those of type 1, although it is caused by mutations in a different gene. Hyperparathyroidism is the most common feature, followed by tumours of the pituitary gland, additional endocrine glands, and other organs.
Inheritance patterns
The source lists the following possible inheritance patterns: Autosomal dominant. More than one pattern can apply in different cases.
Related genes
Other names
Adenomatosis, familial endocrine
Endocrine neoplasia, multiple
Familial endocrine adenomatosis
MEA
MEN
Multiple endocrine adenomatosis
Multiple endocrine neoplasms
Related patient information and medicines
Patient information leaflet
Hyperparathyroidism
However, in a very small number of people, it can be the result of an inherited condition called familial isolated hyperparathyroidism. Also, rarely, it may be inherited as part of a collection of problems (a syndrome) called multiple endocrine neoplasia (MEN) which affects the parathyroids, the pancreas and the pituitary gland. Secondary.
Patient information leaflet
Prolactinoma
A prolactinoma is a non-cancerous growth in the pituitary gland that makes a hormone called prolactin. Tucked away deep inside your brain is a gland called your pituitary gland. A prolactinoma occurs when some of the cells in the pituitary gland (the ones producing prolactin) multiply more than usual to form a small growth (tumour) in the.
Patient information leaflet
Can I take Losartan and Rybelsus together?
Pancreatitis Stop taking Rybelsus and seek immediate medical help if you experience severe, persistent stomach pain (which may reach through to your back), as this can be a sign of an inflamed pancreas (pancreatitis). Thyroid cancer Do not take Rybelsus if you or any of your family have ever had medullary thyroid carcinoma (MTC) or Multiple.
Patient information leaflet
Can I take Diazepam and Tirzepatide together?
Thyroid Cancer Risk Do not use tirzepatide if you or any of your family have ever had a type of thyroid cancer called medullary thyroid carcinoma (MTC) or Multiple Endocrine Neoplasia syndrome type 2 (MEN 2). Severe Gastrointestinal Disease Tirzepatide slows stomach emptying and is not recommended for patients with severe gastrointestinal.
Patient information leaflet
Can I take Metformin and Semaglutide together?
History of pancreatitis Stop taking semaglutide and seek immediate medical help if you experience severe,... Thyroid cancer Do not take semaglutide if you or any of your family have ever had medullary thyroid carcinoma (MTC) or Multiple Endocrine Neoplasia syndrome type 2 (MEN 2). Contact your doctor immediately if you notice a lump or swelling in.
Patient information leaflet
Myeloma
Myeloma is a cancer that affects cells, called plasma cells, in the bone marrow. Bone marrow is the spongy tissue at the centre of some bones that produces the body's blood cells. As the cancerous plasma cells fill the bone marrow, you are not able to make enough normal blood cells. This can lead to anaemia, bleeding problems and infections. Other.
Further reading and references
- Multiple endocrine neoplasia; MedlinePlus, National Library of Medicine.
- Concolino P, Costella A, Capoluongo E. Multiple endocrine neoplasia type 1 (MEN1): An update of 208 new germline variants reported in the last nine years. Cancer Genet. 2016 Jan-Feb;209(1-2):36-41. doi: 10.1016/j.cancergen.2015.12.002. Epub 2015 Dec 14.
- Eng C, Plitt G. Multiple Endocrine Neoplasia Type 2. 1999 Sep 27 [updated 2023 Aug 10]. In: Adam MP, Bick S, Mirzaa GM, Wallace SE, Amemiya A, editors. GeneReviews(R) [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2026. Available from http://www.ncbi.nlm.nih.gov/books/NBK1257/
- Giusti F, Marini F, Brandi ML. Multiple Endocrine Neoplasia Type 1. 2005 Aug 31 [updated 2022 Mar 10]. In: Adam MP, Bick S, Mirzaa GM, Wallace SE, Amemiya A, editors. GeneReviews(R) [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2026. Available from http://www.ncbi.nlm.nih.gov/books/NBK1538/
- Lee M, Pellegata NS. Multiple endocrine neoplasia type 4. Front Horm Res. 2013;41:63-78. doi: 10.1159/000345670. Epub 2013 Mar 19.
- Moline J, Eng C. Multiple endocrine neoplasia type 2: an overview. Genet Med. 2011 Sep;13(9):755-64. doi: 10.1097/GIM.0b013e318216cc6d.
- Pellegata NS. MENX and MEN4. Clinics (Sao Paulo). 2012;67 Suppl 1(Suppl 1):13-8. doi: 10.6061/clinics/2012(sup01)04.
- Raue F, Rondot S, Schulze E, Szpak-Ulczok S, Jarzab B, Frank-Raue K. Clinical utility gene card for: multiple endocrine neoplasia type 2. Eur J Hum Genet. 2012 Jan;20(1). doi: 10.1038/ejhg.2011.142. Epub 2011 Aug 24. No abstract available.
- Thakker RV, Newey PJ, Walls GV, Bilezikian J, Dralle H, Ebeling PR, Melmed S, Sakurai A, Tonelli F, Brandi ML; Endocrine Society. Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). J Clin Endocrinol Metab. 2012 Sep;97(9):2990-3011. doi: 10.1210/jc.2012-1230. Epub 2012 Jun 20.
- Thakker RV. Multiple endocrine neoplasia type 1 (MEN1) and type 4 (MEN4). Mol Cell Endocrinol. 2014 Apr 5;386(1-2):2-15. doi: 10.1016/j.mce.2013.08.002. Epub 2013 Aug 8.
- Thakker RV. Multiple endocrine neoplasia type 1 (MEN1). Best Pract Res Clin Endocrinol Metab. 2010 Jun;24(3):355-70. doi: 10.1016/j.beem.2010.07.003.
- Wells SA Jr, Asa SL, Dralle H, Elisei R, Evans DB, Gagel RF, Lee N, Machens A, Moley JF, Pacini F, Raue F, Frank-Raue K, Robinson B, Rosenthal MS, Santoro M, Schlumberger M, Shah M, Waguespack SG; American Thyroid Association Guidelines Task Force on Medullary Thyroid Carcinoma. Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma. Thyroid. 2015 Jun;25(6):567-610. doi: 10.1089/thy.2014.0335.
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