Multiple endocrine neoplasia type 1 (MEN 1) is an inherited disease of
the neuroendocrine cell system affecting primarily the parathyroids,
pancreas, duodenum and the anterior pituitary. The pancreatic and duod
enal tumours may metastasize, but generally have a low malignant poten
tial. The diagnosis of MEN 1 is usually made in the second decade of l
ife and based on the involvement of at least two organs and a family h
istory. The recent discovery of the MEN 1 locus on the centromeric leg
ion of the long arm of chromosome 11 may become a further diagnostic c
riterion, The use of flanking DNA markers permits presymptomatic testi
ng for MEN I in affected families.