Pancreatic Neuroendocrine Tumor Producing Insulin and Vasopressin
- 171 Downloads
The objective of the study is to report a rare case of pancreatic neuroendocrine tumor (pNET) producing insulin and vasopressin. We describe the clinical presentation and management of a metastatic pNET with refractory hypoglycemia and progressive severe hyponatremia. A 52-year-old patient had abdominal pain leading to the diagnosis of a tumor that was initially presumed to be splenic in origin. Investigations ultimately identified a pancreatic mass that on biopsy proved to be a pNET. Eventually, he developed extensive liver metastases, and with tumor progression, he manifested hypoglycemia and severe hyponatremia. He was managed with multiple therapies including somatostatin analogue, peptide-receptor-radionuclide-therapy (PRRT), diazoxide, and everolimus; none of these therapeutic modalities was successful in controlling functional and structural progression of the tumor. Ultimately, the pNET proved fatal and autopsy confirmed widely metastatic disease that stained strongly and diffusely for vasopressin, a feature not seen in the previous liver biopsy. This case illustrates the challenges of diagnosis and management of aggressive insulin-producing pNETs and highlights the potential concomitant ectopic production of vasopressin leading to refractory hyponatremia.
KeywordsPancreatic neuroendocrine tumors Neuroendocrine tumors Insulin Vasopressin
Compliance with Ethical Standards
Conflict of Interest
The authors declare that they have no conflict of interest.
- 9.Gomez-Perez FJ, Cuevas-Ramos D, Valdes PA, Aguilar-Salinas CA, Mehta R, Rull JA. Beta-cell adenomas without hyperinsulinemia with use of highly specific insulin radioimmunoassays: case report and review of literature. Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists. 2010;16(4):660–663.CrossRefGoogle Scholar
- 13.Biondi CA, Gartside MG, Waring P, Loffler KA, Stark MS, Magnuson MA, et al. Conditional Inactivation of the Men1 Gene Leads to Pancreatic and Pituitary Tumorigenesis but Does Not Affect Normal Development of These Tissues. Molecular and Cellular Biology. 2004;24(8):3125–3131.CrossRefPubMedPubMedCentralGoogle Scholar
- 19.Louiset E, Contesse V, Groussin L, Cartier D, Duparc C, Perraudin V, et al. Expression of vasopressin receptors in ACTH-independent macronodular bilateral adrenal hyperplasia causing Cushing's syndrome: molecular, immunohistochemical and pharmacological correlates. J Endocrinol. 2008;196(1):1–9.CrossRefPubMedGoogle Scholar