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Multiple Endocrine Neoplasia Syndromes

Inherited tumor syndromes affecting multiple endocrine glands.

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 Internal Medicine department. Book Appointment →

What is Multiple Endocrine Neoplasia Syndromes?

Multiple endocrine neoplasia (MEN) syndromes are autosomal dominant inherited genetic disorders that lead to tumors or hyperplasia in multiple endocrine organs. There are three main types: MEN 1, MEN 2A, and MEN 2B.

MEN 1 (Wermer syndrome): characterized by parathyroid hyperplasia (90%), pancreatic neuroendocrine tumors (60-70%), and pituitary adenomas (30-40%). It is associated with mutations in the MEN1 gene.

MEN 2A (Sipple syndrome): medullary thyroid cancer (95%), pheochromocytoma (50%), and primary hyperparathyroidism (20-30%). MEN 2B: medullary thyroid cancer, pheochromocytoma, mucosal neuromas, and a marfanoid habitus. Both are linked to mutations in the RET proto-oncogene.

Symptoms

MEN 1: hypercalcemia, peptic ulcer (gastrinoma), hypoglycemia (insulinoma)
MEN 2A: thyroid nodule, hypertensive episodes, hypercalcemia
MEN 2B: lip and tongue neuromas, marfanoid appearance
Family history of endocrine tumors at a young age
Concurrent pathology in multiple endocrine glands

Risk Factors

Family history of MEN syndrome
Known carrier status for MEN1 or RET mutations
Medullary thyroid cancer at a young age
Bilateral pheochromocytoma
Multiple parathyroid adenomas

When to See a Doctor?

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

  • If MEN syndrome runs in the family, seek genetic counseling and testing
  • If tumors are detected in multiple endocrine glands
  • If thyroid cancer or pheochromocytoma develops at a young age
  • If a positive genetic test is found, regular screening should begin

Treatment Methods

01
Genetic counseling and family screening
02
MEN 2: prophylactic thyroidectomy (timing based on the RET mutation type)
03
MEN 1: parathyroidectomy, surgery or surveillance for pancreatic tumors
04
Pheochromocytoma: adrenalectomy after alpha-blockade
05
Lifelong regular biochemical and imaging surveillance

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.