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Multiple Endocrine Neoplasia Type 2 (MEN2)

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

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

  • 36 Accesses

Synonyms

Familial medullary thyroid carcinoma; Mucosal neuroma syndrome; Sipple syndrome

Definition

Multiple endocrine neoplasia type 2 (MEN2), first described by Sipple in 1961, is an autosomal dominant syndrome with two types (2A and 2B). MEN2A is more common and is characterized by medullary thyroid carcinoma, pheochromocytoma, and primary hyperparathyroidism due to parathyroid adenomas. Cutaneous lichen amyloidosis is also a rare manifestation. MEN2A may be categorized into four subtypes as follows: (i) classical MEN2A, (ii) MEN2A with cutaneous lichen amyloidosis, (iii) MEN2A with Hirschsprung disease, and (iv) familial medullary thyroid cancer. The far less common but more aggressive MEN2B (also known as MEN3) is characterized by medullary thyroid carcinoma, pheochromocytoma, a “Marfanoid” body habitus, as well as mucosal or gastrointestinal neuromas.

Clinical Features

  • Incidence

    MEN2 occurs in about 1 in 200,000 live births.

  • Age

    In MEN2B, medullary thyroid carcinoma may develop...

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References and Further Reading

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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 2 (MEN2). In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5224-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5224-1

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  • Print ISBN: 978-3-319-28845-1

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