Pancreatic Neuroendocrine Tumor Producing Insulin and Vasopressin

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Alshaikh, Omalkhaire M
Yoon, Ju-
Chan, Bryan A
Krzyzanowska, Monika K
Butany, Jagdish
Asa, Sylvia L
Ezzat, Shereen
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2018
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Abstract

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.

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Endocrine Pathology
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© 2018 Springer US. This is an electronic version of an article published in Endocrine Pathology, Volume 29, Issue 1, pp 15–20. Endocrine Pathology is available online at: http://link.springer.com/ with the open URL of your article.
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Clinical sciences
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