Total pancreatectomy in the MEN-1 syndrome

A family with the multiple endocrine neoplasia Type 1 (MEN-1) syndrome, followed over three generations, is presented. In the first generation, one family member died after a history indicating Zollinger-Ellison syndrome. In the second generation endocrine pancreatic tumours were diagnosed or suspected in three out of five members. In the third generation, five out of seven members were investigated and four of these had positive tests at hormonal screening. Of particular diagnostic value in this family was the determination of peripheral serum levels of proinsulin and C-peptide, which were used as tumour markers both for diagnosis and in the postoperative follow-up. Two members in the third generation have been successfully managed by total pancreatectomy. A MEN-1 patient with malignant pancreatic tumours should be considered for total pancreatectomy if the pancreatic disease has caused high morbidity and mortality within the family. This is especially pertinent if the patient has multiple malignant tumours.