Multiple endocrine neoplasia, type 1 (MEN1) is an autosomal dominant hereditary syndrome caused by germline mutations of the menin gene that predisposes the development of endocrine and non-endocrine tumours with variable penetrance. However, only limited data regarding adrenal involvement in MEN1 is available. Gatta-Cherifi et al. analysed the records of 715 MEN1 patients in a multicentre database and compared it with those found in a multicentre control cohort of 155 sporadic adrenal incidentalomas.

They found that adrenal pathology in MEN1 differed from that observed in sporadic incidentalomas. They suggest that in MEN1, endocrine investigations should focus on steroid hormone secretion, the occurrence of ACC and lifelong imaging of adrenal tumours. A significant increase in size of adrenal lesions eventually associated with a change in spontaneous attenuation values at CT scanning or lipid content at MRI should prompt towards surgery. Gatta-Cherifi et al. (2012) European Journal of Endocrinology 166 269-279.

Read the full article at DOI: 10.1530/EJE-11-0679.