endocrine and metabolic disorders
Multiple Endocrine Neoplasia, Type 2B (MEN 2B)
Key Points
Multiple endocrine neoplasia, type 2B (MEN 2B) has a mutation of the same gene as in MEN 2A and manifests similarly except for the absence of hyperparathyroidism, the presence of more aggressive medullary thyroid carcinoma and the presence of multiple mucosal neuromas and a marfanoid habitus. Patients should have genetic testing forRETproto-oncogene mutations, serumcalcitoninmeasurement, blood or urine tests for pheochromocytoma, and imaging studies of the neck and adrenal glands. Pheochromocytoma is excised and prophylactic thyroidectomy is done. 