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A Pancreatic Polypeptide-Producing Pancreatic Tumor Causing WDHA SyndromeAmrilleva V.a · Slater E.P.a · Waldmann J.a · Bonorden D.b · Fendrich V.a
Departments of aSurgery and bGastroenterology, Philipps University Marburg, Marburg, Germany Corresponding Author
Volker Fendrich, MD
Department of Surgery, Philipps University Marburg
DE–35043 Marburg (Germany)
Tel. +49 6421 2866441, Fax +49 6421 2862105, E-Mail firstname.lastname@example.org
We report the case of a 46-year-old female patient with WDHA (watery diarrhea/hypokalemia/achlorhydria) syndrome caused by a pancreatic polypeptide-producing tumor in the head of the pancreas. Whereas VIP and other pancreatic endocrine hormones were in the normal range, only serum levels of pancreatic polypeptide were elevated. Imaging studies identified a pancreatic tumor in the head of the gland. After laparotomy, the tumor of 3 cm in size was enucleated. Final pathology documented a pancreatic endocrine tumor with immunohistochemical staining demonstrating the presence of pancreatic polypeptide. The patient remained cured after a follow-up of more than three years. The present case illustrates that, although rare, WDHA syndrome may be associated with a pancreatic polypeptide-secreting endocrine tumor of the pancreas.
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