Pancreatico-duodenal
tumors are the second most common endocrinopathy in
multiple endocrine neoplasia syndrome type 1, and have a pronounced effect on life expectancy as the principal cause of disease-related death. Previous discussions about surgical management have focused mainly on syndromes of
hormone excess and, in particular, the management of
multiple endocrine neoplasia syndrome type 1-related
Zollinger-Ellison syndrome. Since hormonal syndromes tend to occur late and indicate the presence of
metastases, screening with
biochemical markers and endoscopic ultrasound is recommended for early detection of pancreatico-duodenal
tumors, and with early surgery before
metastases have developed. Surgery is recommended in patients with or without hormonal syndromes in the absence of disseminated liver
metastases. The suggested operation includes distal 80% subtotal pancreatic resection together with enucleation of
tumors in the head of the pancreas, and in cases with
Zollinger-Ellison syndrome, excision of duodenal
gastrinomas together with clearance of regional
lymph node metastases. This strategy, with early and aggressive surgery before
metastases have developed, is believed to reduce the risks for
tumor recurrence and malignant progression.