VIPoma

PMID: 29939520
Source: StatPearls
Publication date: 2025-07-24
Year: 2025

Abstract

Vasoactive intestinal peptide tumors (VIPomas) are rare neuroendocrine tumors characterized by unregulated secretion of vasoactive intestinal peptide (VIP), leading to a distinct clinical syndrome. Werner and Morrison first described them in 1958 as a pancreatic tumor resulting in watery diarrhea and hypokalemia, coining the term Verner-Morrison syndrome after its discoverers. While VIPomas are unusual, they encompass 2% of pancreatic neuroendocrine tumors. They secrete excessive VIP, which primarily affects the gastrointestinal (GI) system and can cause effects secondary to the secretion of water and electrolytes from the GI epithelial cells. The tumor usually presents as a firm, homogenous, and well-circumscribed lesion with histology consistent with that of other well-differentiated neuroendocrine tumors. VIPoma is characterized by fasting-persistent watery diarrhea accompanied by hypokalemia and dehydration, collectively referred to as WDHA syndrome (eg, watery diarrhea, hypokalemia, and achlorhydria). VIPoma symptoms may often overlap with more common GI conditions like laxative abuse, colitis, malabsorption, or inflammatory bowel disease. Therefore, the diagnosis of VIPoma is dependent upon VIP levels greater than 250 pg/mL. Multiphase contrast-enhanced computed tomography scans in conjunction with somatostatin receptor scintigraphy are imaging techniques that aid in diagnosis and tumor localization. Management is primarily complete surgical resection with somatostatin analogs for medical optimization and symptom control. VIPomas can cause life-threatening electrolyte abnormalities, and most tumors are considered malignant with a median survival of 8 years (dependent on tumor grade, stage, and resectability). Ultimately, the complications of this tumor are related to the symptoms associated with the primary tumor's excessive secretion of VIP, in addition to the complications inherent to surgical resection and metastasis. This course highlights the clinical presentation, diagnosis, management, and treatment of VIPomas.