Multiple endocrine neoplasia type 1

Received 10 March 2007; received in revised form 6 July 2007; accepted 30 August 2007. published online 08 November 2007.

Abstract

The co-occurrence of parathyroid hyperplasia with pancreatic endocrine tumours and/or pituitary adenoma is classified as Multiple Endocrine Neoplasia type 1 (MEN-1) and is caused by a germ-line mutation in MEN-1 gene encoding a tumour suppressor protein, menin. This review presents clinical expressions, diagnosis and management of the MEN-1 syndrome. Properties and mechanisms of menin functions are also reviewed.

Keywords: MEN-1, Menin, Primary hyperparathyroidism, Gastrinoma, Insulinoma, Pituitary tumours

Department of Nephrology, Endocrinology and Metabolic Diseases, Medical University of Silesia, Katowice, ul. Francuska 20/24, 40-027 Katowice, Poland

Corresponding author. Tel.: +48 322552695; fax: +48 322553726.

PII: S0953-6205(07)00262-2

doi:10.1016/j.ejim.2007.08.004

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