Merck Manual

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Overview of Multiple Endocrine Neoplasias (MEN)

(Familial Endocrine Adenomatosis; Multiple Endocrine Adenomatosis)

By

Lawrence S. Kirschner

, MD, PhD, The Ohio State University;


Pamela Brock

, MS, CGC, The Ohio State University

Reviewed/Revised Jun 2023
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Topic Resources

The multiple endocrine neoplasia (MEN) syndromes comprise 4 genetically distinct familial diseases involving adenomatous hyperplasia and malignant tumors in several endocrine glands.

Clinical features depend on the glandular elements involved. The phenotype of MEN 4 is similar to that of MEN 1 but lacks the cutaneous abnormalities seen in the latter disorder.

Table

Each syndrome is inherited as an autosomal dominant trait with a high degree of penetrance, variable expressivity, and production of seemingly unrelated effects by a single mutant gene. The specific mutation is not always known.

Symptoms and signs develop at any age. Proper management includes early identification of affected individuals within a kindred and surgical removal of the tumors when possible. Although these syndromes are genetically and clinically distinct, significant overlap exists (see table ).

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