Multiple endocrine neoplasia type 1
Updates to Article Attributes
Multiple endocrine neoplasia type 1 (MEN1), also known as Wermer syndrome, is an autosomal dominant genetic disease that results in proliferative lesions in multiple endocrine organs, particularly the pituitary gland, islet cells of the pancreas, and parathyroid glands.
There are other multiple endocrine neoplasia syndromes and these are discussed separately.
Epidemiology
Associations
In addition to the characteristic lesions involving the pituitary, parathyroid, and pancreas, numerous other lesions are encountered with greater frequency in patients with MEN1. These include:
-
adrenal cortical lesions
cortisol-secreting adenomas
adrenal carcinomas (rare)
hepatic focal nodular hyperplasia 5
Clinical presentation
Primary hyperparathyroidism is the commonest presentation, followed by pancreatic islet cell tumourneuroendocrine tumours with associated hypersecretion syndromes; gastrinomas are most common and associated with Zollinger-Ellison syndrome 7.
MEN type 1MEN1 is an autosomal dominant syndrome characterizedcharacterised by 1-7:
-
prolactinoma (most common)
30% of patients
-
islet cellpancreatic neuroendocrine tumoursof the pancreasgastrinoma (most common: >50%), followed by insulinoma (4-6%), and glucagonoma (<3%) 9
50-80% of patients
-
asignificant cause of mortality
-
parathyroid proliferative diseases 7
-
parathyroid hyperplasia (most common)
hyperparathyroidism
is seen in 80(80-95%of patients)
parathyroid carcinoma (rare)
-
Handy mnemonics for recalling MEN type 1MEN1:
Pathology
Genetics
The abnormality is related to MEN1, a tumour suppressor gene located on chromosome 11q13 which produces menin, a nuclear protein important for the regulation of gene expression.
Treatment and prognosis
Treatment is directed to each individual manifestation. These are therefore discussed separately.
Pancreatic malignancy is the leading cause of mortality in MEN type 1MEN1.
History and etymology
Harvey Cushing, was the first to publish a case of MEN1 with the classical triad in 1927. MEN1 was first characterizedcharacterised as a discrete pathological entity by an American-Austrian physician Paul Wermer et al., whilst working at Presbyterian Hospital, as part of a Columbia University team in New York, USA in 1954 3,8. The MEN1 gene was discovered in 1997 8.
See also
-<p><strong>Multiple endocrine neoplasia type 1 (MEN1)</strong>, also known as <strong>Wermer syndrome</strong>, is an autosomal dominant genetic disease that results in proliferative lesions in multiple endocrine organs, particularly the <a href="/articles/pituitary-gland">pituitary gland</a>, islet cells of the <a href="/articles/pancreas">pancreas</a> and <a href="/articles/parathyroid-glands">parathyroid glands</a>. </p><p>There are other <a href="/articles/multiple-endocrine-neoplasia-syndromes">multiple endocrine neoplasia </a><a href="/articles/multiple-endocrine-neoplasia-syndromes">syndromes</a> and these are discussed separately. </p><h4>Epidemiology</h4><h5>Associations</h5><p>In addition to the characteristic lesions involving the pituitary, parathyroid, and pancreas, numerous other lesions are encountered with greater frequency in patients with MEN1. These include: </p><ul>-<li><a href="/articles/lipoma">lipomas</a></li>-<li><a href="/articles/angiofibroma">angiofibromas</a></li>-<li>-<a href="/articles/adrenal-cortex">adrenal cortical</a> lesions<ul>-<li><a href="/articles/adrenal-adenoma">adrenal adenomas</a></li>-<li><a href="/articles/adrenocortical-hyperplasia">adrenocortical hyperplasia</a></li>-<li>cortisol-secreting adenomas</li>-<li>-<a href="/articles/adrenal-cortical-carcinoma-1">adrenal carcinomas</a> (rare)</li>- +<p><strong>Multiple endocrine neoplasia type 1 (MEN1)</strong>, also known as <strong>Wermer syndrome</strong>, is an autosomal dominant genetic disease that results in proliferative lesions in multiple endocrine organs, particularly the <a href="/articles/pituitary-gland">pituitary gland</a>, <a href="/articles/pancreas">pancreas</a>, and <a href="/articles/parathyroid-glands">parathyroid glands</a>. </p><p>There are other <a href="/articles/multiple-endocrine-neoplasia-syndromes">multiple endocrine neoplasia syndromes</a> and these are discussed separately. </p><h4>Epidemiology</h4><h5>Associations</h5><p>In addition to the characteristic lesions involving the pituitary, parathyroid, and pancreas, numerous other lesions are encountered with greater frequency in patients with MEN1. These include: </p><ul>
- +<li><p><a href="/articles/lipoma">lipomas</a></p></li>
- +<li><p><a href="/articles/angiofibroma">angiofibromas</a></p></li>
- +<li>
- +<p><a href="/articles/adrenal-cortex">adrenal cortical</a> lesions</p>
- +<ul>
- +<li><p><a href="/articles/adrenal-adenoma">adrenal adenomas</a></p></li>
- +<li><p><a href="/articles/adrenocortical-hyperplasia">adrenocortical hyperplasia</a></p></li>
- +<li><p>cortisol-secreting adenomas</p></li>
- +<li><p><a href="/articles/adrenal-cortical-carcinoma-1">adrenal carcinomas</a> (rare)</p></li>
-<li><a href="/articles/carcinoid-tumour-2">carcinoid tumours</a></li>-<li>hepatic <a href="/articles/focal-nodular-hyperplasia">focal nodular hyperplasia</a> <sup>5</sup>-</li>-<li>-<a href="/articles/breast-neoplasms">breast carcinoma</a> <sup>8</sup>-</li>-<li>-<a href="/articles/meningioma">meningiomas</a> <sup>8</sup>-</li>-</ul><h4>Clinical presentation</h4><p><a href="/articles/primary-hyperparathyroidism">Primary hyperparathyroidism</a> is the commonest presentation, followed by pancreatic islet cell tumour with associated hypersecretion syndromes; <a href="/articles/gastrinoma">gastrinomas</a> are most common and associated with <a href="/articles/zollinger-ellison-syndrome">Zollinger-Ellison syndrome</a> <sup>7</sup>. </p><p>MEN type 1 is an autosomal dominant syndrome characterized by <sup>1-7</sup>:</p><ul>-<li>-<a href="/articles/pituitary-adenoma">pituitary adenomas</a><ul>-<li>-<a href="/articles/prolactinoma">prolactinoma</a> (most common)</li>-<li>30% of patients</li>- +<li><p><a href="/articles/carcinoid-tumour-2">carcinoid tumours</a></p></li>
- +<li><p>hepatic <a href="/articles/focal-nodular-hyperplasia">focal nodular hyperplasia</a> <sup>5</sup></p></li>
- +<li><p><a href="/articles/breast-neoplasms">breast carcinoma</a> <sup>8</sup></p></li>
- +<li><p><a href="/articles/meningioma">meningiomas</a> <sup>8</sup></p></li>
- +</ul><h4>Clinical presentation</h4><p><a href="/articles/primary-hyperparathyroidism">Primary hyperparathyroidism</a> is the commonest presentation, followed by pancreatic neuroendocrine tumours with associated hypersecretion syndromes; <a href="/articles/gastrinoma">gastrinomas</a> are most common and associated with <a href="/articles/zollinger-ellison-syndrome">Zollinger-Ellison syndrome</a> <sup>7</sup>. </p><p>MEN1 is an autosomal dominant syndrome characterised by <sup>1-7</sup>:</p><ul>
- +<li>
- +<p><a href="/articles/pituitary-adenomapitnet">pituitary adenomas</a></p>
- +<ul>
- +<li><p><a href="/articles/prolactinoma">prolactinoma</a> (most common)</p></li>
- +<li><p>30% of patients</p></li>
-<a href="/articles/endocrine-tumours-of-the-pancreas">islet cell tumours of the pancreas</a><ul>-<li>-<a href="/articles/gastrinoma">gastrinoma</a> (most common) followed by <a href="/articles/glucagonoma">glucagonoma</a>-</li>-<li>50-80% of patients</li>-<li>a significant cause of mortality</li>- +<p><a href="/articles/endocrine-tumours-of-the-pancreas">pancreatic neuroendocrine tumours</a></p>
- +<ul>
- +<li><p><a href="/articles/gastrinoma">gastrinoma</a> (most common: >50%), followed by <a href="/articles/insulinoma" title="Insulinoma">insulinoma</a> (4-6%), and <a href="/articles/glucagonoma">glucagonoma</a> (<3%) <sup>9</sup></p></li>
- +<li><p>50-80% of patients</p></li>
- +<li><p>significant cause of mortality</p></li>
-<a title="Parathyroid proliferative disease" href="/articles/parathyroid-proliferative-disease">parathyroid proliferative diseases</a> <sup>7</sup><ul>- +<p><a href="/articles/parathyroid-proliferative-disease" title="Parathyroid proliferative disease">parathyroid proliferative diseases</a> <sup>7</sup></p>
- +<ul>
-<a href="/articles/parathyroid-hyperplasia">parathyroid hyperplasia</a> (most common)<ul><li>-<a href="/articles/hyperparathyroidism">hyperparathyroidism</a> is seen in 80-95% of patients</li></ul>- +<p><a href="/articles/parathyroid-hyperplasia">parathyroid hyperplasia</a> (most common)</p>
- +<ul><li><p><a href="/articles/hyperparathyroidism">hyperparathyroidism</a> (80-95%)</p></li></ul>
-<li><a href="/articles/parathyroid-adenoma">parathyroid adenoma</a></li>-<li>-<a href="/articles/parathyroid-carcinoma">parathyroid carcinoma</a> (rare)</li>- +<li><p><a href="/articles/parathyroid-adenoma">parathyroid adenoma</a></p></li>
- +<li><p><a href="/articles/parathyroid-carcinoma">parathyroid carcinoma</a> (rare)</p></li>
-</ul><p>Handy mnemonics for recalling MEN type 1: </p><ul><li><a title="PPP (mnemonic for MEN1)" href="/articles/men1-triad-mnemonic">PPP or PiParPanc</a></li></ul><h4>Pathology</h4><h5>Genetics</h5><p>The abnormality is related to <em>MEN1</em>, a tumour suppressor gene located on chromosome 11q13 which produces menin, a nuclear protein important for the regulation of gene expression. </p><h4>Treatment and prognosis</h4><p>Treatment is directed to each individual manifestation. These are therefore discussed separately.</p><p>Pancreatic malignancy is the leading cause of mortality in MEN type 1. </p><h4>History and etymology</h4><p>Harvey Cushing, was the first to publish a case of MEN1 with the classical triad in 1927. MEN1 was first characterized as a discrete pathological entity by an American-Austrian physician <strong>Paul Wermer</strong> et al., whilst working at Presbyterian Hospital, as part of a Columbia University team in New York, USA in 1954 <sup>3,8</sup>. The MEN1 gene was discovered in 1997 <sup>8</sup>.</p><h4>See also</h4><ul>-<li><strong>MEN1 (Wermer syndrome)</strong></li>-<li>-<a href="/articles/multiple-endocrine-neoplasia-type-ii-1">MEN2</a><ul>-<li>-<a href="/articles/multiple-endocrine-neoplasia-type-iia-1">MEN2a</a> (Sipple syndrome)</li>- +</ul><p>Handy mnemonics for recalling MEN1: </p><ul><li><p><a href="/articles/men1-triad-mnemonic" title="PPP (mnemonic for MEN1)">PPP or PiParPanc</a></p></li></ul><h4>Pathology</h4><h5>Genetics</h5><p>The abnormality is related to <em>MEN1</em>, a tumour suppressor gene located on chromosome 11q13 which produces menin, a nuclear protein important for the regulation of gene expression. </p><h4>Treatment and prognosis</h4><p>Treatment is directed to each individual manifestation. These are therefore discussed separately.</p><p>Pancreatic malignancy is the leading cause of mortality in MEN1. </p><h4>History and etymology</h4><p>Harvey Cushing was the first to publish a case of MEN1 with the classical triad in 1927. MEN1 was first characterised as a discrete pathological entity by an American-Austrian physician <strong>Paul Wermer</strong> et al., whilst working at Presbyterian Hospital, as part of a Columbia University team in New York, USA in 1954 <sup>3,8</sup>. The MEN1 gene was discovered in 1997 <sup>8</sup>.</p><h4>See also</h4><ul>
- +<li><p><strong>MEN1 (Wermer syndrome)</strong></p></li>
-<a href="/articles/multiple-endocrine-neoplasia-type-iib">MEN2b</a> (mucosal neuroma syndrome)</li>-<li><a href="/articles/familial-medullary-thyroid-carcinoma">familial medullary thyroid carcinoma</a></li>- +<p><a href="/articles/multiple-endocrine-neoplasia-type-ii-1">MEN2</a></p>
- +<ul>
- +<li><p><a href="/articles/multiple-endocrine-neoplasia-type-iia-1">MEN2a</a> (Sipple syndrome)</p></li>
- +<li><p><a href="/articles/multiple-endocrine-neoplasia-type-iib">MEN2b</a> (mucosal neuroma syndrome)</p></li>
- +<li><p><a href="/articles/familial-medullary-thyroid-carcinoma">familial medullary thyroid carcinoma</a></p></li>
-<li><a href="/articles/multiple-endocrine-neoplasia-type-iv">MEN4</a></li>-<li><a href="/articles/carney-complex">Carney complex</a></li>- +<li><p><a href="/articles/multiple-endocrine-neoplasia-type-iv">MEN4</a></p></li>
- +<li><p><a href="/articles/carney-complex">Carney complex</a></p></li>
References changed:
- 9. Sadowski S & Triponez F. Management of Pancreatic Neuroendocrine Tumors in Patients with MEN 1. Gland Surg. 2015;4(1):63-8. <a href="https://doi.org/10.3978/j.issn.2227-684X.2014.12.01">doi:10.3978/j.issn.2227-684X.2014.12.01</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/25713781">Pubmed</a>