A Variant Pancreatic Insulinoma in MEN1 Syndrome Characterized by Normoglycemia/Normoinsulinemia but Abnormal C-Peptide and Abnormal Proinsulin Levels

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Introduction: Insulinoma associated w/ hyperinsulinemia is frequent in functioning pancreatic NET in MEN 1. We report on familial variant insulinoma w/ normoglycemia/norminsulinemia, but abnormal C-peptide and proinsulin.

Aim(s): To draw attention to variant pancreatic insulinoma in MEN 1 syndrome, characterized by normoglycemia/normoinsulinemia but abnormal C-peptide & proinsulin.

Materials and methods: A 45-year-old pt. (index Pt.) and his 18-year-old daughter with hyperparthyroidism and prolactinoma underwent determination of gastrin (normal), VIP (normal), CgA, CT pancreas and extended fast monitoring glucose, insulin, C-peptide (CP) and proinsulin (PI). Genetic studies were also done. Brother of index pt. and three of his children also have MEN 1.

Conference: 8th Annual ENETSConcerence (2011)

Presenting Author:

Authors: Ahmed M, Al Qaraawi A, Al Faifi J,

Keywords: MEN 1 syndrome, insulinoma, genetic analysis,