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Multiple Endocrine Neoplasia Type 1 (MEN1)

  • Living reference work entry
  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 35 Accesses

Synonyms

Multiple endocrine adenomatosis; Wermer syndrome

Definition

Multiple endocrine neoplasia type 1 (MEN1), first discovered by Erdheim in 1903 and then defined by Underdahl and Werner about five decades later, is a rare autosomal dominant disorder characterized by both endocrine and non-endocrine neoplasms. It is caused by pathogenic mutations in MEN1, a gene which encodes for menin, a protein which has a number of diverse functions including regulation of cell division, cellular proliferation, and genomic stability. The classic triad of endocrine lesions developing in the context of MEN1 is parathyroid proliferations, anterior pituitary neuroendocrine tumors, and pancreaticoduodenal neuroendocrine tumors; in general, the clinical diagnosis of MEN1 syndrome is suspected when a patient presents with tumors affecting two of the three gland locations described above. Other endocrine lesions including foregut-origin neuroendocrine tumors such as gastric enterochromaffin-like cell...

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References and Further Readings

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Authors and Affiliations

  1. Department of Laboratory Medicine and Pathobiology, Faculty of Medicine, University of Toronto, Toronto, ON, Canada

    Anjelica Hodgson, Sara Pakbaz & Ozgur Mete

  2. Department of Pathology, University Health Network, Toronto, ON, Canada

    Anjelica Hodgson, Sara Pakbaz & Ozgur Mete

  3. Endocrine Oncology Site, The Princess Margaret Cancer Centre, Toronto, ON, Canada

    Ozgur Mete

Authors
  1. Anjelica Hodgson
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  2. Sara Pakbaz
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  3. Ozgur Mete
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Correspondence to Ozgur Mete .

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Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Hodgson, A., Pakbaz, S., Mete, O. (2020). Multiple Endocrine Neoplasia Type 1 (MEN1). In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5223-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5223-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

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