Multiple endocrine neoplasia type 1 with primary hyperparathyroidism, pancreatic insulinoma and portal gastrinoma (case report)

It is presented case report of multiple endocrine neoplasia type 1 (Wermer’s syndrome). This syndrome is characterized by a large variability of clinical manifestations. In our case, there was a combination of primary hyperparathyroidism and simultaneous neuroendocrine tumors of the pancreatic tail and portal area. We have not found similar cases with portal gastrinoma in the available literature. Simultaneous subtotal parathyroidectomy, distal pancreatectomy with excision of portal insulinoma and partially extrahepatic bile ducts followed by hepaticojejunostomy were performed. This observation demonstrates the modern diagnostic and surgical methods in patients with MEN-1 syndrome and neuroendocrine tumors.