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    Multiple endocrine neoplasia (MEN) II

    Sipple syndrome; MEN II

    Multiple endocrine neoplasia, type II (MEN II) is disorder passed down through familiesin 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.

    A tumor in the adrenal gland is called a pheochromocytoma.

    A tumor in thethyroid gland is a 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 pheochromocytoma.

    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). Medullary carcinoma of the thyroid is a very aggressive and potentially fatal cancer. However, early diagnosis and surgery can often lead to a cure.

    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.

    References

    Kronenberg HM. Polyglandular disorders. In: Goldman L, Ausiello D, eds. 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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    • Endocrine glands

      illustration

      • Endocrine glands

        illustration

      A Closer Look

        Tests for Multiple endocrine neoplasia (MEN) II

          Review Date: 3/14/2012

          Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.

          The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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