Open AccessMultiple endocrine neoplasia type 1 with primary hyperparathyroidism, pancreatic insulinoma and portal gastrinoma (case report)
Author(s) -
V. G. Ignatyuk,
Timur Britvin,
Д. В. Подрез,
Л. Е. Гуревич
Publication year - 2018
Publication title -
annaly hirurgičeskoj gepatologii
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.138
H-Index - 1
eISSN - 2408-9524
pISSN - 1995-5464
DOI - 10.16931/1995-5464.2018357-63
Subject(s) - gastrinoma , insulinoma , multiple endocrine neoplasia , men1 , medicine , zollinger ellison syndrome , endocrine system , hyperparathyroidism , pancreas , neuroendocrine tumors , gastroenterology , primary hyperparathyroidism , hormone , biology , biochemistry , secretion , gastrin , gene
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.