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</div><div><span>Multiple endocrine neoplasia (MEN) II</span></div></div>
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</a><h1 class="with-also" itemprop="name">Multiple endocrine neoplasia (MEN) II</h1>
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</noscript></div><div class="main"><div id="ency_summary"><p>Multiple endocrine neoplasia, type II (MEN II) is a disorder passed down through families in which one or more of the endocrine glands are overactive or form a tumor. Endocrine glands most commonly involved include:</p><ul><li>Adrenal gland (about half the time)</li><li>Parathyroid gland (20% of the time)</li><li>Thyroid gland (almost all the time) </li></ul><p>Multiple endocrine neoplasia, type I (<a test="test" href="./000398.htm">MEN I</a>) is a related condition. </p></div><section><div class="section"><div class="section-header"><div class="section-title"><h2>Causes</h2></div><div class="section-button"><button type="submit" aria-controls="section-1" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-1"><p>The cause of MEN II is a defect in a gene called RET. This defect causes many tumors to appear in the same person, but not necessarily at the same time.</p><p>Involvement of the adrenal gland is most often with a tumor called a <a test="test" href="./000340.htm">pheochromocytoma</a>. </p><p>Involvement of the thyroid gland is most often with a tumor called <a test="test" href="./000374.htm">medullary carcinoma of the thyroid</a>.</p><p>Tumors in the thyroid, adrenal, or parathyroid glands may occur years apart.</p><p>The disorder may occur at any age, and affects men and women equally. The main risk factor is a family history of MEN II.</p><p>There are two subtypes of MEN II. They are MEN IIa and IIb. MEN IIb is less common.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Symptoms</h2></div><div class="section-button"><button type="submit" aria-controls="section-2" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-2"><p>The symptoms may vary. However, they are similar to those of:</p><ul><li><div><a test="test" href="./000374.htm">Medullary carcinoma of the thyroid</a></div></li><li><div><a test="test" href="./000340.htm">Pheochromocytoma</a></div></li><li><div><a test="test" href="./001188.htm">Parathyroid adenoma</a></div></li><li><div><a test="test" href="./001189.htm">Parathyroid hyperplasia</a></div></li></ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Exams and Tests</h2></div><div class="section-button"><button type="submit" aria-controls="section-3" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-3"><p>To diagnose this condition, the health care provider looks for a mutation in the RET gene. This can be done with a blood test. Additional tests are done to determine which hormones are being overproduced.</p><p>A physical exam may reveal:</p><ul><li>
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Enlarged lymph nodes in the neck
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</li><li><a test="test" href="./003090.htm">Fever</a></li><li>
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High blood pressure (<a test="test" href="./000468.htm">hypertension</a>)</li><li><a test="test" href="./003077.htm">Rapid heart rate</a></li><li><a test="test" href="./007265.htm">Thyroid nodules</a></li></ul><p>Imaging tests used to identify tumors may include:</p><ul><li><a test="test" href="./003789.htm">Abdominal CT scan</a></li><li>
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Imaging of the kidneys or ureters
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</li><li><a test="test" href="./003830.htm">MIBG scintiscan</a></li><li><a test="test" href="./003796.htm">MRI of abdomen</a></li><li><a test="test" href="./003829.htm">Thyroid scan</a></li><li><a test="test" href="./003776.htm">Ultrasound of the thyroid</a></li></ul><p>Blood and other tests are used to see how well certain glands in the body are working. They may include:</p><ul><li><a test="test" href="./003699.htm">Blood calcitonin</a> level
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</li><li><a test="test" href="./003470.htm">Blood alkaline phosphatase</a></li><li><a test="test" href="./003477.htm">Blood calcium</a></li><li>Blood parathyroid hormone level</li><li><a test="test" href="./003478.htm">Blood phosphorus</a></li><li><a test="test" href="./003613.htm">Urine catecholamines</a></li><li><a test="test" href="./003613.htm">Urine metanephrine</a></li></ul><p>Other tests or procedures that may be done include:</p><ul><li>Adrenal biopsy</li><li>Electrocardiogram (<a test="test" href="./003868.htm">ECG</a>)</li><li>Thyroid biopsy</li></ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Treatment</h2></div><div class="section-button"><button type="submit" aria-controls="section-4" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-4"><p>Surgery is needed to remove a pheochromocytoma, which can be life threatening due to the hormones it makes.</p><p>For medullary carcinoma of the thyroid, the thyroid gland and surrounding lymph nodes must be totally removed. Thyroid hormone replacement therapy is given after surgery.</p><p>If a child is known to carry the RET gene mutation, surgery to remove the thyroid before it becomes cancerous is considered. This should be discussed with a physician who is very familiar with this condition. It would be done at an early age (before age 5) in people with known MEN IIa, and before age 6 months in people with MEN IIb. </p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Outlook (Prognosis)</h2></div><div class="section-button"><button type="submit" aria-controls="section-5" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-5"><p>Pheochromocytoma is most often not cancerous (benign). Medullary carcinoma of the thyroid is a very aggressive and potentially fatal cancer, but early diagnosis and surgery can often lead to a cure. Surgery does not cure the underlying MEN II.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Possible Complications</h2></div><div class="section-button"><button type="submit" aria-controls="section-6" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-6"><p>The spread of cancerous cells is a possible complication.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>When to Contact a Medical Professional</h2></div><div class="section-button"><button type="submit" aria-controls="section-7" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-7"><p>Contact your provider if you notice symptoms of MEN II or if someone in your family receives such a diagnosis.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Prevention</h2></div><div class="section-button"><button type="submit" aria-controls="section-8" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-8"><p>Screening close relatives of people with MEN II may lead to early detection of the syndrome and related cancers. This may allow for steps to prevent complications. </p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Alternative Names</h2></div><div class="section-button"><button type="submit" aria-controls="section-Alt" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-Alt"><p>Sipple syndrome; MEN II; Pheochromocytoma - MEN II; Thyroid cancer - pheochromocytoma; Parathyroid cancer - pheochromocytoma</p></div></div></section><section><div class="section sec-mb"><div class="section-header"><div class="section-title"><h2>Images</h2></div><div class="section-button"><button type="submit" aria-controls="section-tnails" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-Ref"><p>Gild ML, Tsang V, Clifton-Bligh RJ, Robinson BG. Multiple endocrine neoplasia types 2 and 3, and medullary thyroid carcinoma. In: Robertson RP, ed. <em>DeGroot's Endocrinology</em>. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 134.</p><p>National Comprehensive Cancer Network website. Clinical practice guidelines in oncology (NCCN guidelines): neuroendocrine and adrenal tumors. Version 1.2023. <a href="https://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf" target="_blank">www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf</a>. Updated August 2, 2023. Accessed May 7, 2024.</p><p>Newey PJ, Thakker RV. Multiple endocrine neoplasia. In: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. W<em>illiams Textbook of Endocrinology</em>. 14th ed. Philadelphia, PA: Elsevier; 2020:chap 42.</p><p>Nieman LK, Spiegel AM. Polyglandular disorders. In: Goldman L, Cooney KA, eds. <em>Goldman-Cecil Medicine</em>. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 212.</p></div></div></section>
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