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Multiple Endocrine Neoplasia Types Causes Symptoms Diagnosis Treatment
Frequently Asked Questions
What is multiple endocrine neoplasia
Multiple endocrine neoplasia is a group of inherited disorders characterized by the development of tumors in two or more endocrine glands, most commonly involving the parathyroid, pancreas, pituitary, thyroid and adrenal glands.
What are the main types of MEN
The main types are MEN 1, MEN 2A, MEN 2B and Familial Medullary Thyroid Carcinoma. Each type involves a different combination of endocrine tumors.
What causes MEN syndrome
MEN is caused by inherited genetic mutations. MEN 1 is caused by mutation in the MEN1 gene, while MEN 2 syndromes are caused by mutations in the RET proto-oncogene.
What tumors occur in MEN 1
MEN 1 causes tumors of the parathyroid glands, pancreatic neuroendocrine tumors such as gastrinoma and insulinoma, and pituitary adenomas, most commonly prolactinomas.
What is the most common manifestation of MEN 1
Primary hyperparathyroidism due to parathyroid hyperplasia is the most common manifestation of MEN 1.
What are the key features of MEN 2A
MEN 2A is characterized by medullary thyroid carcinoma, pheochromocytoma and primary hyperparathyroidism.
What distinguishes MEN 2B from MEN 2A
MEN 2B includes aggressive medullary thyroid carcinoma, pheochromocytoma, mucosal neuromas and a marfanoid body habitus.
Why is calcitonin important in MEN
Calcitonin is secreted by medullary thyroid carcinoma and is used as a tumor marker for diagnosis and monitoring in MEN 2.
What is the role of genetic testing in MEN
Genetic testing identifies MEN1 or RET mutations and allows early diagnosis, family screening and preventive treatment such as prophylactic thyroidectomy.
Why must pheochromocytoma be treated before thyroid surgery in MEN 2
Pheochromocytoma must be removed first because uncontrolled catecholamine release during thyroid surgery can cause fatal hypertensive crisis.
What is Zollinger Ellison syndrome in MEN
Zollinger Ellison syndrome is caused by gastrin-secreting tumors in MEN 1 leading to severe peptic ulcer disease and chronic diarrhea.
What is prophylactic thyroidectomy in MEN
Prophylactic thyroidectomy is the preventive removal of the thyroid gland in RET mutation carriers to stop development of medullary thyroid carcinoma.
Can MEN be cured
MEN cannot be cured genetically, but early detection and timely surgery and medical treatment can prevent complications and allow long-term survival.
Is MEN inherited
Yes, MEN is inherited in an autosomal dominant pattern, meaning a child has a 50 percent chance of inheriting the condition if one parent is affected.
What is the most important screening test in MEN 2
Serum calcitonin and RET genetic testing are the most important screening tools for MEN 2.
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