Hypoglycemia in a patient with a polyhormonal pancreatic neuroendocrine tumor with evidence of endocrine progenitors

Zachary B. Simons, Rachel C. Morgan, Laurel Rose, Jennifer B. Nelson, Sarah A. Tersey, Raghavendra G. Mirmira

Research output: Contribution to journalArticle

Abstract

A 55-year-old woman with a large polyhormonal neuroendocrine tumor with unusual pathology is described. The patient presented with intermittent neuroglycopenic symptoms between more protracted asymptomatic periods occurring over the preceding 4 years. During a diagnostic 72-hour inpatient fast, she exhibited hypoglycemia at 70 hours after initiation. On computed tomography scan, a 6-cm mass was identified at the pancreatic head. The patient underwent a pylorus-preserving pancreaticoduodenectomy, and pathology was positive for cells staining for pancreatic polypeptide, insulin, and occasional double hormone (insulin plus pancreatic polypeptide)-positive cells. In addition, the tumor exhibited broad staining for ALDH1A3, a new marker of endocrine progenitors. This case serves to highlight the clinical and pathologic variability of insulin-producing tumors and raises the potential for cells in these tumors to exhibit hormone interconversion and progenitor-like states.

Original languageEnglish (US)
Pages (from-to)172-177
Number of pages6
JournalJournal of the Endocrine Society
Volume2
Issue number2
DOIs
StatePublished - Feb 2018

Keywords

  • Hypoglycemia
  • Insulin
  • Neuroendocrine tumor
  • Pancreatic polypeptide

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism

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