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Overview of Multiple Endocrine Neoplasias (MEN)

(Familial Endocrine Adenomatosis; Multiple Endocrine Adenomatosis)

By Patricia A. Daly, MD, Medical Director for Diabetes;Visiting Assistant Professor of Clinical Medicine, Valley Health System;University of Virginia ; Lewis Landsberg, MD, Irving S. Cutter Professor of Medicine and Dean Emeritus, Northwestern University Feinberg School of Medicine

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The multiple endocrine neoplasia (MEN) syndromes comprise 3 genetically distinct familial diseases involving adenomatous hyperplasia and malignant tumors in several endocrine glands.

  • MEN 1 involves primarily hyperplasia or sometimes adenomas of the parathyroid glands (with resultant hyperparathyroidism) and tumors of the pancreatic islet cells and/or pituitary gland.

  • MEN 2A involves primarily medullary thyroid carcinoma, pheochromocytoma, hyperplasia or sometimes adenomas of the parathyroid glands (with resultant hyperparathyroidism), and occasionally cutaneous lichen amyloidosis.

  • MEN 2B involves primarily medullary thyroid carcinoma, pheochromocytoma, multiple mucosal and intestinal neuromas, and marfanoid habitus.

Clinical features depend on the glandular elements involved.

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: Conditions Associated With MEN Syndromes).

Conditions Associated With MEN Syndromes

Condition

MEN 1

MEN 2A

MEN 2B

95%

10–20%

30–80%

15–42%

> 95%

> 95%

40–50%

50%

Mucosal neuromas

≈ 100%

≈ 100%

MEN = multiple endocrine neoplasia.

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