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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Multiple endocrine neoplasia type 1


Other Names for this Disease

  • Endocrine adenomatosis multiple
  • MEN 1
  • Wermer syndrome
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Treatment

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How might multiple endocrine neoplasia, type 1 be treated?

People with multiple endocrine neoplasia, type 1 (MEN1) are usually managed with regular screening to allow for early diagnosis and treatment of endocrine tumors. This screening begins in early childhood and continues for life.[1]

Recommended screening includes specific types of imaging studies every 3-5 years:[1] Annual blood tests are also recommended, which evaluate the levels of certain substances that can be elevated if an MEN1-associated tumor is present:[1]
  • Prolactin concentrations, which can be used to screen for pituitary tumors, are measured beginning at age 5.
  • Calcium concentrations, which can be used to screen for parathyroid tumors, are measured beginning at age 8.
  • Gastrin concentrations, which can be used to screen for gastrinomas (a specific type of gastro-entero-pancreatic tract tumor) are measured beginning at age 20.

When a tumor is detected through screening, the best treatment options depend on many factors, including the size, location, and type of tumor; and whether or not the tumor is "functional" (releasing hormones). Many tumors are treated with surgery. If a tumor is functional, removal of the affected endocrine gland often resolves health problems that may be present as a result of elevated hormones. In some cases, functional tumors can be treated with medications that block the function or lower the levels of the overproduced hormone. Chemotherapy or radiation therapy may also be used to to shrink or destroy tumors.[1][2]

Last updated: 12/4/2014

References
  1. Pagon RA, Adam MP, Ardinger HH, et al. Multiple Endocrine Neoplasia Type 1. GeneReviews. September 2012; http://www.ncbi.nlm.nih.gov/books/NBK1538/?report=printable.
  2. Multiple Endocrine Neoplasia, Type 1. NORD. May 2012; http://rarediseases.org/rare-disease-information/rare-diseases/byID/1229/viewFullReport.


Management Guidelines

  • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.

Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Multiple endocrine neoplasia type 1. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
Other Names for this Disease
  • Endocrine adenomatosis multiple
  • MEN 1
  • Wermer syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.