Multiple endocrine neoplasia
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Multiple endocrine neoplasia
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First Aid
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Multiple endocrine neoplasia p. 339
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Contributors
Harry Delaney, MBChB
Sam Gillespie, BSc
The multiple endocrine neoplasias, or MEN for short, are a group of inherited diseases which cause tumors to grow in the endocrine glands of the body.
The endocrine glands affected in multiple endocrine neoplasia are the pituitary gland, the thyroid gland, the parathyroid glands, the adrenal glands and the pancreas.
So in multiple endocrine neoplasias there are tumors that form in these glands that lead to overproduction of hormones.
Let’s start by going through the glands that are affected and what they do.
The pituitary gland is a pea-sized gland found at the base of the brain that makes hormones to control many of the other endocrine glands in the body.
These hormones include thyroid stimulating hormone which acts on the thyroid to make thyroid hormone, adrenal corticotropic hormone that acts on the adrenal glands to make cortisol, follicle stimulating hormone and luteinizing hormone which acts on the ovaries and testes to make estrogen in women and testosterone in men.
So it’s like the king of the endocrine glands telling them how much hormone to produce.
The pituitary also makes growth hormone which makes you grow, prolactin which stimulates milk production in women, oxytocin which triggers milk release, antidiuretic hormone which help the kidneys reabsorb water, and melanocyte stimulating hormone helps the melanocytes create more melanin or pigment.
Summary
Multiple endocrine neoplasias, or MEN for short, are a group of inherited diseases that cause tumors to grow in the endocrine glands of the body. MEN tumors are classified into two broad categories based on their underlying mutations. There is MEN1 caused by a mutation in the MEN1 gene, and MEN2, which is caused by a mutation in the RET gene.
People with MEN1 present with tumors in the pituitary gland or parathyroid glands and pancreatic tumors. MEN2 is further divided into two subtypes: MEN2A and MEN2B. MEN2A is characterized by medullary thyroid cancer, pheochromocytoma, and parathyroid tumors; whereas MEN2B presents with medullary thyroid cancer, pheochromocytoma, and mucosal neuromas.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
- "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
- "Multiple endocrine neoplasia type 2: An overview" Genetics in Medicine (2011)
- "Concepts for screening and diagnostic follow-up in multiple endocrine neoplasia type 1 (MEN1)" Experimental and Clinical Endocrinology & Diabetes (2000)
- "Multiple endocrine neoplasia type 2: An overview" Genetics in Medicine (2011)
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