Multiple endocrine neoplasia type IIa
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- updated 11 Jun 2023:
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Multiple endocrine neoplasia (MEN) type IIa, also known as Sipple syndrome, accounts for most cases of MEN2 and is characterised by:
phaeochromocytomas: in 50% of patients, often bilateral, and can be extra-adrenal
medullary thyroid cancer: 100% of patients, aggressive, and may secrete calcitonin
parathyroid hyperplasia: only seen in 20% of patients, and often presents with hypercalcaemia and renal calculi
Mnemonic:
PMP
Pathology
Genetics
A small proportion of individuals have a RET D631 proto-oncogene mutation. RET mutation is different from the RET translocation in papillary thyroid carcinoma. ChromosomeThe chromosome locus is 10q11.2. 3
See also
-<p><strong>Multiple endocrine neoplasia (MEN) type IIa</strong>, also known as <strong>Sipple syndrome</strong>, accounts for most cases of <a href="/articles/multiple-endocrine-neoplasia-type-ii-1">MEN2</a> and is characterised by:</p><ul>-<li><p><a href="/articles/phaeochromocytoma-1">phaeochromocytomas</a>: in 50% of patients, often bilateral, and can be extra-adrenal</p></li>-<li><p><a href="/articles/medullary-thyroid-carcinoma-1">medullary thyroid cancer</a>: 100% of patients, aggressive, and may secrete <a href="/articles/calcitonin">calcitonin</a></p></li>-<li><p><a href="/articles/parathyroid-hyperplasia">parathyroid hyperplasia</a>: only seen in 20% of patients, and often presents with <a href="/articles/hypercalcaemia">hypercalcaemia</a> and <a href="/articles/renal-calculi">renal calculi</a></p></li>-</ul><p>Mnemonic: </p><ul><li><p><strong>PMP</strong></p></li></ul><h4>Pathology</h4><h5>Genetics</h5><p>A small proportion of individuals have a <em>RET</em> D631 proto-oncogene mutation. RET mutation is different from the RET translocation in papillary thyroid carcinoma. Chromosome locus is 10q11.2.<sup> 3</sup></p><h4>See also</h4><ul>-<li><p><a href="/articles/multiple-endocrine-neoplasia-type-1-2">MEN1</a> (Wermer syndrome)</p></li>-<li>-<p><a href="/articles/multiple-endocrine-neoplasia-type-ii-1">MEN2</a> (multiple endocrine adenomatosis)</p>-<ul>-<li><p><strong>MEN2a</strong> (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>-</ul>-</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>- +<p><strong>Multiple endocrine neoplasia (MEN) type IIa</strong>, also known as <strong>Sipple syndrome</strong>, accounts for most cases of <a href="/articles/multiple-endocrine-neoplasia-type-ii-1">MEN2</a> and is characterised by:</p><ul>
- +<li><p><a href="/articles/phaeochromocytoma-1">phaeochromocytomas</a>: in 50% of patients, often bilateral, and can be extra-adrenal</p></li>
- +<li><p><a href="/articles/medullary-thyroid-carcinoma-1">medullary thyroid cancer</a>: 100% of patients, aggressive, and may secrete <a href="/articles/calcitonin">calcitonin</a></p></li>
- +<li><p><a href="/articles/parathyroid-hyperplasia">parathyroid hyperplasia</a>: only seen in 20% of patients, and often presents with <a href="/articles/hypercalcaemia">hypercalcaemia</a> and <a href="/articles/renal-calculi">renal calculi</a></p></li>
- +</ul><p>Mnemonic: </p><ul><li><p><strong>PMP</strong></p></li></ul><h4>Pathology</h4><h5>Genetics</h5><p>A small proportion of individuals have a <em>RET</em> D631 proto-oncogene mutation. RET mutation is different from the RET translocation in papillary thyroid carcinoma. The chromosome locus is 10q11.2.<sup> 3</sup></p><h4>See also</h4><ul>
- +<li><p><a href="/articles/multiple-endocrine-neoplasia-type-1-2">MEN1</a> (Wermer syndrome)</p></li>
- +<li>
- +<p><a href="/articles/multiple-endocrine-neoplasia-type-ii-1">MEN2</a> (multiple endocrine adenomatosis)</p>
- +<ul>
- +<li><p><strong>MEN2a</strong> (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>
- +</ul>
- +</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>
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