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Multiple endocrine neoplasia (MEN) II

Sipple syndrome; MEN II

 

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:

  • Adrenal gland (about half the time)
  • Parathyroid gland (20% of the time)
  • Thyroid gland (almost all of the time)

See also: MEN I

Causes

 

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 usually with a tumor called a pheochromocytoma.

Involvement of the thyroid gland is usually with a tumor called medullary carcinoma of the thyroid.

Tumors 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 -- 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
  • Pheochromocytoma
  • Parathyroid adenoma
  • Parathyroid hyperplasia

 

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 examination may reveal:

  • Enlarged lymph nodes in the neck
  • Fever
  • High blood pressure
  • Rapid heart rate
  • Thyroid nodules

Imaging tests used to identify tumors may include:

  • Abdominal CT scan
  • Imaging of the kidneys or ureters
  • MIBG scintiscan
  • MRI of abdomen
  • Thyroid scan
  • Ultrasound of the thyroid

Blood tests are used to see how well certain glands in the body are working. They may include:

  • Calcitonin level
  • Blood alkaline phosphatase
  • Blood calcium
  • Blood parathyroid hormone level
  • Blood phosphorus
  • Urine catecholamines
  • Urine metanephrine

Other tests or procedures that may be done include:

  • Adrenal biopsy
  • Electrocardiogram (ECG)
  • Thyroid biopsy
  • Parathyroid 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 usually not cancerous (benign). While medullary carcinoma of the thyroid is a very aggressive and potentially fatal cancer, 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

 

Call your health care 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, allowing potentially preventive intervention.

 

 

References

Kronenberg HM. Polyglandular disorders. In: Goldman L, Ausiello D, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 239.

National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Neuroendocrine Tumors. National Comprehensive Cancer Network; 2011. Version 1. 2011.

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      Tests for Multiple endocrine neoplasia (MEN) II

       
         

        Review Date: 3/23/2014

        Reviewed By: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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