Duodenal
gastrinomas are now more frequently recognized as the source of hypergastrinemia in patients with
Zollinger-Ellison syndrome. The cell lineage of duodenal
gastrinomas may differ from that of pancreatic
gastrinomas, which accounts for variations in their clinical behavior. Attempts to localize the submucosal
tumors are difficult and are limited by their small size. Intraoperative endoscopic transillumination, selective intra-arterial
secretin injection, and duodenotomy with mucosal eversion are currently the most sensitive and reliable methods of localization. Endoscopic ultrasonography and
somatostatin scintigraphy further enhance the accuracy of preoperative localization of these
tumors. Current information based on cure rates and survival data mandates a primary surgical approach in patients with either the sporadic or the
multiple endocrine neoplasia type 1-associated form of the disease. Thus, wide local resection of duodenal
gastrinomas with removal of all
tumor-bearing lymphatic tissue and
acid inhibitory
pharmacotherapy (
proton pump inhibition) may yield 5-year survival rates of 80% to 90%. Similarly, in patients with pancreatic and duodenal
gastrinomas as a manifestation of
multiple endocrine neoplasia type 1, the additional enucleation of pancreatic lesions with or without distal
pancreatectomy has resulted in cure rates of 67% to 100%.