Multiple endocrine neoplasia Guide, Meaning , Facts, Information and Description

Multiple endocrine neoplasia (MEN) consists of three syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. The presence of any one tumor type does not automatically have a patient labelled as MEN, but a search of the other at-risk areas is usually undertaken, especially when there are suggestive clinical signs.

MEN syndromes are inherited as autosomal dominant disorders. Medullary carcinoma of the thyroid may occur as an autosomal dominant in the absence of other features.

Table of contents

1 MEN type 1 2 MEN type 2A and 2B 2.1 MEN type 2A 2.2 MEN type 2B

MEN type 1

1. Parathyroid hyperplasia/tumour causing hyperparathyroidism.
2. Islet cell tumour causing hypoglycaemia (insulinoma) and Zollinger-Ellison syndrome (gastrinoma).
3. Pituitary adenoma which may cause pituitary hormone excess.

MEN type 2A and 2B

The next two MEN syndrome types have their basis in molecular genetics. Individuals can be tested for this genetic disorder reliably even when asymptomatic patients. The mutation is in the ret oncogene.

MEN type 2A

1. Medullary carcinoma of thyroid which is associated with increased calcitonin secretion. A test for elevated calcitonin should be done after pentagastrin injection and calcium infusion, to ensure that all affected patients are detected.
2. Pheochromocytoma
3. Parathyroid hyperplasia/tumour causing hyperparathyroidism.

MEN type 2B

1. Phaeochromocytoma
2. Medullary carcinoma of thyroid which is associated with increased calcitonin secretion. A test for elevated calcitonin should be done after pentagastrin injection and calcium infusion, to ensure that all affected patients are detected.
3. Mucosal neuromas which are usually situated in the gastrointestinal tract.
4. Marfanoid habitus

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