Recent advances in the localization and surgical management of duodenal gastrinomas

Abstract

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%.