MULTIPLE ENDOCRINE NEOPLASIA (MEN) SYNDROMES

The Correct answer is C.Medullary carcinoma of the thyroid is a component of MEN I

MULTIPLE ENDOCRINE NEOPLASIA (MEN) SYNDROMES

There are 3 MEN syndromes consisting of neoplastic or hyperplastic lesions of multiple endocrine glands, transmitted as autosomal dominants.

Typically, familial tumors are multicentric and bilateral. Hence, in patients with endocrine neoplasia involving only a single gland (e.g. parathyroid adenoma), the tumour is likely sporadic rather than familial. While sporadic hyperparathryoidism is usually due to a single parathyroid adenoma, hyperparathyroidism of MEN syndromes is due to diffuse hyperplasia involving all glands. In kindred suspected of MEN-I, hyperparathyroidism precedes other endocrine neoplasia in almost all cases, so simply following serum calcium at yearly intervals is probably sufficient.
Of the different neoplasms involved in MEN syndromes, medullary thyroid carcinoma (MTC) is the most likely to have a genetic background (25% of MTC), a fact that merits looking for cases of MTC in other family members and looking for pheochromocytoma in patients with MTC.
In MEN-2B syndrome, mucosal neuromas are present in almost all affected patients. The neuromas appear as small bumps about the lips, tongue, and buccal mucosa. They generally precede thyroid medullary carcinoma or pheochromocytoma. Hence one should always look for mucosal neuromas in kindred suspected of MEN-2B.

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