Multiple endocrine neoplasia type 1
Updates to Article Attributes
Multiple endocrine neoplasia type I (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.
Clinical presentation
Primary hyperparathyroidism is the commonest presentation, followed by pancreatic islet cell tumour with associated hypersecretion syndromes; gastrinomas are most common and associateassociated with Zollinger-Ellison syndrome 7.
Pathology
The abnormality is related to a tumour suppressor gene located in chromosome 11q13. MEN type I is an autosomal dominant syndrome characterised by 1-7:
-
pituitary adenomas
- prolactinoma (most common)
- 30% of patients
-
islet cell tumours of the pancreas
- gastrinoma (most common) followed by glucagonoma
- 50-80% of patients
- a significant cause of mortality
- proliferative parathyroid gland lesions 7
-
parathyroid hyperplasia (most common)
- hyperparathyroidism is seen in 80-95% of patients
- parathyroid adenoma
- parathyroid carcinoma (rare)
-
parathyroid hyperplasia (most common)
Handy mnemonics for recalling MEN type I:
- PPP or PiParPanc
Associations
In addition to the aforementioned characteristic lesions involving the pituitary, parathyroid and pancreas, numerous other lesions are encountered with greater frequency in patients with MEN1. These include:
- lipomas
- angiofibromas
-
adrenal cortical lesions
- adrenal adenomas
- adrenocortical hyperplasia
- cortisol-secreting adenomas
- adrenal carcinomas (rare)
- carcinoid tumours
- hepatic focal nodular hyperplasia 5
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 I.
History and etymology
MEN1 was first characterised by P Wermer et al. in 1954 3.
See also
- MEN1 (Wermer syndrome)
-
MEN2
- MEN2a (Sipple syndrome)
- MEN2b (mucosal neuroma syndrome)
- familial medullary thyroid carcinoma
- MEN4
- Carney complex
-<p><strong>Multiple endocrine neoplasia type I (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>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 associate with <a href="/articles/zollinger-ellison-syndrome">Zollinger-Ellison syndrome</a> <sup>7</sup>. </p><h4>Pathology</h4><p>The abnormality is related to a tumour suppressor gene located in chromosome 11q13. MEN type I is an autosomal dominant syndrome characterised by <sup>1-7</sup>:</p><ul>- +<p><strong>Multiple endocrine neoplasia type I (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>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><h4>Pathology</h4><p>The abnormality is related to a tumour suppressor gene located in chromosome 11q13. MEN type I is an autosomal dominant syndrome characterised by <sup>1-7</sup>:</p><ul>
-<li><a href="/articles/carcinoid-tumours-1">carcinoid tumours </a></li>- +<li><a href="/articles/carcinoid-tumour-2">carcinoid tumours </a></li>