[Surgical aspects of multiple endocrine neoplasia type 2]

Surgical aspects of multiple endocrine neoplasia type 2 Abstract. The multiple endocrine neoplasia type 2 (MEN 2) is an autosomal-dominant hereditary tumor-syndrome. The subtypes show clinically specific disease manifestations. In MEN 2a, medullary thyroid carcinoma, pheochromocytoma, and primary hyperparathyroidism occur, while in familial medullary thyroid carcinoma, the medullary thyroid carcinoma is the only clinical manifestation. In MEN 2b medullary thyroid carcinoma and pheochromocytoma are the clinically dominant manifestations. All MEN-2-specific disease is treated primarily surgically. It is of utmost importance to identify the individual gene carrier in order to enable timely intervention before malignancy develops (prophylactic surgery) or to reach surgical cure as well as to identify relatives that are MEN 2 gene-carriers. Typical MEN 2 manifestations and clinical signs important for surgery are outlined. In case MEN 2 is suspected, it may be vital to exclude underlying pheochromocytoma before diagnostic or surgical interventions are started to forego hyperadrenergic crisis unprepared.