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Multiple endocrine neoplasia (MEN) I

Wermer syndrome; 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 down through families.

Endocrine glands most commonly involved include:

  • Pancreas
  • Parathyroid
  • Pituitary

Causes

 

MEN I is caused by a defect in a gene that carries the code for a protein called menin. The condition causes tumors of various glands to appear in the same person, but not necessarily at the same time.

The disorder may occur at any age, and it affects men and women equally. A family history of this disorder raises your risk.

 

Symptoms

 

Symptoms vary from person to person, and depend on which gland is involved. They may include:

  • Abdominal pain
  • Anxiety
  • Black, tarry stools
  • Bloated feeling after meals
  • Burning, aching, or hunger discomfort in the upper abdomen or lower chest that is relieved by antacids, milk, or food
  • Decreased sexual interest
  • Fatigue
  • Headache
  • Lack of menstrual periods (in women)
  • Loss of appetite
  • Loss of body or facial hair (in men)
  • Mental changes or confusion
  • Muscle pain
  • Nausea and vomiting
  • Sensitivity to the cold
  • Unintentional weight loss
  • Vision problems
  • Weakness

 

Exams and Tests

 

The health care provider will perform a physical exam and ask questions about your medical history and symptoms. The following tests may be done:

  • Blood cortisol level
  • CT scan of the abdomen
  • CT scan of the head
  • Fasting blood sugar
  • Genetic testing
  • Insulin test
  • MRI of the abdomen
  • MRI of the head
  • Parathyroid biopsy
  • Serum adrenocorticotropic hormone
  • Serum calcium
  • Serum follicle stimulating hormone
  • Serum gastrin
  • Serum glucagon
  • Serum luteinizing hormone
  • Serum parathyroid hormone
  • Serum prolactin
  • Serum thyroid stimulating hormone
  • Ultrasound of the neck

 

Treatment

 

Surgery to remove the diseased gland is often the treatment of choice. A medicine called bromocriptine may be used instead of surgery for pituitary tumors that release the hormone prolactin.

The parathyroid glands, which control calcium production, can be removed. However, it is difficult for the body to regulate calcium levels without these glands, so a total parathyroid removal is not done first in most cases.

Medicine is available to decrease the excess stomach acid production caused by some tumors (gastrinomas), and to reduce the risk of ulcers.

Hormone replacement therapy is given when entire glands are removed or do not produce enough hormones.

 

Outlook (Prognosis)

 

Pituitary and parathyroid tumors are usually noncancerous ( benign ), but some pancreatic tumors may become cancerous (malignant) and spread to the liver. These can lower life expectancy.

The symptoms of peptic ulcer disease, low blood sugar, excess calcium in the blood, and pituitary dysfunction usually respond well to appropriate treatment.

 

Possible Complications

 

The tumors can keep coming back. Symptoms and complications depend on which glands are involved. Regular check-ups by your health care provider are essential.

 

When to Contact a Medical Professional

 

Call your provider if you notice symptoms of MEN I or have a family history of this condition.

 

Prevention

 

Screening close relatives of people affected with this disorder is recommended.

 

 

Open References

References

National Comprehensive Cancer Network. Clinical practice guidelines in oncology: neuroendocrine tumors. National Comprehensive Cancer Network; 2014. Updated May 25, 2016. Version 1.2015. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf . Accessed April 6, 2016.

Nieman LK, Spiegel AM. Polyglandular disorders. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 231.

Thakker RV. Multiple endocrine neoplasia type 1. In: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Adult and Pediatric . 7th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 148.

 
  • Endocrine glands

    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 pace of chemical activity 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

      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 pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).

      Endocrine glands

      illustration

    A Closer Look

     

      Self Care

       

        Tests for Multiple endocrine neoplasia (MEN) I

         
         

        Review Date: 3/16/2016

        Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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