Multiple endocrine neoplasia (MEN) II
Sipple syndrome; MEN II; Pheochromocytoma - MEN II; Thyroid cancer - pheochromocytoma; Parathyroid cancer - pheochromocytomaMultiple 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:
- Adrenal gland (about half the time)
- Parathyroid gland (20% of the time)
- Thyroid gland (almost all the time)
Multiple endocrine neoplasia, type I (MEN I) is a related condition.
MEN I
Multiple endocrine neoplasia (MEN) type I is a disease in which one or more of the endocrine glands are overactive or forms a tumor. It is passed do...
Read Article Now Book Mark ArticleCauses
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.
Involvement of the adrenal gland is most often with a tumor called a pheochromocytoma.
Pheochromocytoma
Pheochromocytoma is a rare tumor of adrenal gland tissue. It results in the release of too much epinephrine and norepinephrine, hormones that contro...
Read Article Now Book Mark ArticleInvolvement of the thyroid gland is most often with a tumor called medullary carcinoma of the thyroid.
Medullary carcinoma of the thyroid
Medullary carcinoma of the thyroid is cancer of the thyroid gland that starts in cells that release a hormone called calcitonin. These cells are cal...
Read Article Now Book Mark ArticleTumors in the thyroid, adrenal, or parathyroid glands may occur years apart.
The disorder may occur at any age, and affects men and women equally. The main risk factor is a family history of MEN II.
There are two subtypes of MEN II. They are MEN IIa and IIb. MEN IIb is less common.
Symptoms
The symptoms may vary. However, they are similar to those of:
- Medullary carcinoma of the thyroid
Medullary carcinoma of the thyroid
Medullary carcinoma of the thyroid is cancer of the thyroid gland that starts in cells that release a hormone called calcitonin. These cells are cal...
Read Article Now Book Mark Article - Pheochromocytoma
Pheochromocytoma
Pheochromocytoma is a rare tumor of adrenal gland tissue. It results in the release of too much epinephrine and norepinephrine, hormones that contro...
Read Article Now Book Mark Article - Parathyroid adenoma
Parathyroid adenoma
A parathyroid adenoma is a noncancerous (benign) tumor of the parathyroid glands. The parathyroid glands are located in the neck, near or attached t...
Read Article Now Book Mark Article - Parathyroid hyperplasia
Parathyroid hyperplasia
Parathyroid hyperplasia is the enlargement of all 4 parathyroid glands. The parathyroid glands are located in the neck, near or attached to the back...
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Exams and Tests
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.
A physical exam may reveal:
- Enlarged lymph nodes in the neck
- Fever
Fever
Fever is the temporary increase in the body's temperature in response to a disease or illness. A child has a fever when the temperature is at or abov...
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High blood pressure (hypertension)
Hypertension
Blood pressure is a measurement of the force exerted against the walls of your arteries as your heart pumps blood to your body. Hypertension is the ...
Read Article Now Book Mark Article - Rapid heart rate
Rapid heart rate
A bounding pulse is a strong throbbing felt over one of the arteries in the body. It is due to a forceful heartbeat.
Read Article Now Book Mark Article - Thyroid nodules
Thyroid nodules
A thyroid nodule is a growth (lump) in the thyroid gland. The thyroid gland is located at the front of the neck, just above where your collarbones m...
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Imaging tests used to identify tumors may include:
- Abdominal CT scan
Abdominal CT scan
An abdominal CT scan is an imaging method. This test uses x-rays to create cross-sectional pictures of the belly area. CT stands for computed tomog...
Read Article Now Book Mark Article - Imaging of the kidneys or ureters
- MIBG scintiscan
MIBG scintiscan
An MIBG scintiscan is a type of imaging test. It uses a radioactive substance (called a tracer). A scanner finds or confirms the presence of pheoch...
Read Article Now Book Mark Article - MRI of abdomen
MRI of abdomen
An abdominal magnetic resonance imaging scan is an imaging test that uses powerful magnets and radio waves. The waves create pictures of the inside ...
Read Article Now Book Mark Article - Thyroid scan
Thyroid scan
A thyroid scan uses a radioactive iodine tracer to examine the structure and function of the thyroid gland. This test is often done together with a ...
Read Article Now Book Mark Article - Ultrasound of the thyroid
Ultrasound of the thyroid
A thyroid ultrasound is an imaging method to see the thyroid, a gland in the neck that regulates metabolism (the many processes that control the rate...
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Blood and other tests are used to see how well certain glands in the body are working. They may include:
- Blood calcitonin level
Blood calcitonin
The calcitonin blood test measures the level of the hormone calcitonin in the blood.
Read Article Now Book Mark Article - Blood alkaline phosphatase
Blood alkaline phosphatase
Alkaline phosphatase (ALP) is a protein found in all body tissues. Tissues with higher amounts of ALP include the liver, bile ducts, and bone. A blo...
Read Article Now Book Mark Article - Blood calcium
Blood calcium
The calcium blood test measures the level of calcium in the blood. This article discusses the test to measure the total amount of calcium in your blo...
Read Article Now Book Mark Article - Blood parathyroid hormone level
- Blood phosphorus
Blood phosphorus
The phosphorus blood test measures the amount of phosphate in the blood.
Read Article Now Book Mark Article - Urine catecholamines
Urine catecholamines
Catecholamines are chemicals made by nerve tissue (including the brain) and the adrenal gland. The main types of catecholamines are dopamine, norepin...
Read Article Now Book Mark Article - Urine metanephrine
Urine metanephrine
Catecholamines are chemicals made by nerve tissue (including the brain) and the adrenal gland. The main types of catecholamines are dopamine, norepin...
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Other tests or procedures that may be done include:
- Adrenal biopsy
- Electrocardiogram (ECG)
ECG
An electrocardiogram (ECG) is a test that records the electrical activity of the heart.
Read Article Now Book Mark Article - Thyroid biopsy
Treatment
Surgery is needed to remove a pheochromocytoma, which can be life threatening due to the hormones it makes.
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.
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.
Outlook (Prognosis)
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.
Possible Complications
The spread of cancerous cells is a possible complication.
When to Contact a Medical Professional
Contact your provider if you notice symptoms of MEN II or if someone in your family receives such a diagnosis.
Prevention
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.
References
Gild ML, Tsang V, Clifton-Bligh RJ, Robinson BG. Multiple endocrine neoplasia types 2 and 3, and medullary thyroid carcinoma. In: Robertson RP, ed. DeGroot's Endocrinology. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 134.
National Comprehensive Cancer Network website. Clinical practice guidelines in oncology (NCCN guidelines): neuroendocrine and adrenal tumors. Version 1.2023. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Updated August 2, 2023. Accessed May 7, 2024.
Newey PJ, Thakker RV. Multiple endocrine neoplasia. In: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. Williams Textbook of Endocrinology. 14th ed. Philadelphia, PA: Elsevier; 2020:chap 42.
Nieman LK, Spiegel AM. Polyglandular disorders. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 212.
Endocrine glands - illustration
Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
Endocrine glands
illustration
Endocrine glands - illustration
Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
Endocrine glands
illustration
Review Date: 3/31/2024
Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.