Adrenal Tumors in MEN1 Syndrome and the Role of Menin in Adrenal Tumorigenesis

Abstract

Most of the adrenal tumors are benign adrenocortical adenoma (AA)and pheochromocytomas (Pheo) originatingfrom the adrenal medulla, but rarely malignant adrenocortical carcinomas (ACC) can be also found. Adrenal tumors causinghormonal overproduction such as aldosterone-producing and cortisol-producingtumors are also rare, whereas nonhyperfunctioning adenomas occur more frequently.1 During the last decades an extensive use of advanced imaging techniques (computer tomography, magnetic resonance imaging, endoscopic ultrasound) has led to an increased incidence of accidentally discovered adrenal masses, Le., incidentalomas.2, 3, 4 The prevalence of incidentalomas is up to 9% of all autopsy cases. The majority of these tumors are hormonally inactive and are of adrenocortical origin, but pheochromocytomas and hormonally active adrenocortical tumors associated with the development of Cushing’s syndrome or primary aldosteronism can also be found in some patient.1,4