Multiple endocrine neoplasia 1 D44.8

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 11.12.2022

Synonym(s)

Endocrine adenomatosis multiple; endocrine polyadenomatosis; familial polyadenomatosis; MEA type I; MEN-1 syndrome; MEN I, MEN 1; MEN-I Syndrome; MEN Type I; Multiple endocrine adenomatosis; Multiple endocrine adenomatosis type I; multiple endocrine neoplasia type 1; Polyadenomatosis familial; Polyglandular adenoma syndrome; wormer syndrome

History
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Erdheim, 1903; Wermer, 1954

Definition
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Disease pattern belonging to the multiple endocrine neoplasms with combination of peptic ulcers, hyperplastic or tumorous changes of the endocrine pancreas(insulinoma, gastrinoma, glucagonoma, VIPoma), parathyroid gland (mostly adenomatous hyperplasia), anterior pituitary gland (mostly Cushing's syndrome) and possibly thyroid gland (follicular or papillary carcinoma). Frequent comnoinaiton with multiple lipomas.

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Occurrence/Epidemiology
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Panethnic. Prevalence: 0.5-1/100.000 inhabitants/year.

Etiopathogenesis
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Autosomal dominant inheritance. Mutation of the MEN1 gene (menin gene), a tumor suppressor gene mapped to gene locus 11q13, with consecutive loss of an autosome in the tumor with hemizygosity.

Manifestation
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First symptoms appear during puberty or in adulthood (20.-50. LJ). Women are affected about twice as often as men.

Clinical features
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Zollinger-Ellison syndrome with diarrhea. Increased gastrin secretion, gastric ulcers (gastrinomas in 60% of pat.), hypertrophic gastropathy Ménétrier.

Primary hyperparathyroidism: parathyroid hormone increased (in 90-97% of pat.).

Hyperinsulinism (20% insulinomas) or pancreatic neoplasms (in 30-80% of cases).

Pituitary tumors (15-50%).

Struma nodosa, multiple lipomas (also liposarcomas) in the subcutaneous region (not obligatory).

Angiofibromas, bronchial adenomas (not obligate), colonic polyposis (not obligate, rare).

Progression/forecast
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Limited life expectancy (exitus lethalis in 50% of patients around the age of 50).

Literature
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Berglund G et al (2003) Quality of life in patients with multiple endocrine neoplasia type 1 (MEN 1). Fam Cancer 2: 27-33
Brandi ML, Marx SJ, Aurbach GD, Fitzpatrick LA (1987) Familial multiple endocrine neoplasia type I: a new look on pathophysiology. Endocrinol Rev 8: 391-405
Doherty GM et al (2003) Multiple endocrine neoplasia type 1: duodenopancreatic tumours. J Internal Med 253: 590-598
Norton JA et al (2003) Current surgical management of Zollinger-Ellison syndrome (ZES) in patients without multiple endocrine neoplasia-type 1 (MEN1). Surg Oncol 12: 145-151
Wermer P (1954) Genetic aspect of adenomatosis of endocrinic glands. On J Med 16: 363-371