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Multiple Endocrine Neoplasia Type 1 (MEN 1)

An autosomal dominant inherited syndrome with parathyroid, pancreatic, and pituitary tumors.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Endokrinoloji department. Book Appointment →

What is Multiple Endocrine Neoplasia Type 1 (MEN 1)?

Multiple endocrine neoplasia type 1 (MEN 1, Wermer syndrome) is an autosomal dominant inherited disease due to mutations in the MEN1 gene (encoding the menin protein). It is defined by the classic triad of parathyroid hyperplasia, pancreatic neuroendocrine tumors, and pituitary adenomas.

Primary hyperparathyroidism is the most common finding of MEN 1 and is seen in more than 95% of patients. Among pancreatic neuroendocrine tumors, gastrinoma is the most common and leads to Zollinger-Ellison syndrome. Pituitary adenomas are most often prolactinomas.

Early diagnosis and regular screening are critical in extending life expectancy and reducing morbidity.

Symptoms

Symptoms of hypercalcemia due to hyperparathyroidism
Peptic ulcer, diarrhea (gastrinoma)
Hypoglycemia (insulinoma)
Amenorrhea-galactorrhea due to prolactinoma
Findings of acromegaly (GH-secreting adenoma)
Findings of Cushing's syndrome
Family history of endocrine tumors

Risk Factors

Family history of MEN 1 (autosomal dominant transmission)
MEN1 gene mutation
History of multiple endocrine tumors
Hyperparathyroidism at an early age
History of gastrinoma or insulinoma
Pituitary adenoma together with parathyroid disease
Young patients with hyperparathyroidism in the family

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • When hyperparathyroidism is detected under age 40
  • For genetic counseling if there is an individual diagnosed with MEN 1 in the family
  • When Zollinger-Ellison syndrome is suspected
  • In the presence of multiple endocrine tumors
  • Combination of pituitary adenoma and hypercalcemia

Treatment Methods

01
Subtotal or total parathyroidectomy in parathyroid hyperplasia
02
Acid suppression with PPI and surgical resection for gastrinoma
03
Surgical enucleation in insulinoma
04
Cabergoline/bromocriptine in prolactinoma
05
Lifelong clinical, biochemical, and imaging screening
06
Genetic testing and screening of family members

Which Department to Visit?

You can visit our Endokrinoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.