MULTIPLE ENDOCRINE NEOPLASMS TYPE-1 (MEN- 1) - MOLECULAR-GENETICS, MORPHOLOGY AND PROGNOSIS

The syndrome of multiple endocrine neoplasia type 1 (MEN 1) is an auto somal dominant tumour disease of the neuroendocrine system with manife stations in the parathyroids, pancreas, duodenum and pituitary gland a nd rarely also in the stomach and thymus. Recently, the MEN 1 gene loc us has been mapped to the long arm of chromosome 11. This gene most li kely belongs to the tumour suppressor genes, the allelic loss of which causes tumour development. The pancreatic and duodenal tumours may me tastasize, but usually have a low malignant potential. Clinically, mos t MEN 1 patients present between the age of 20 and 35 with hyperparath yroidism and/or Zollinger-Ellison syndrome.