VIPoma: Mechanisms, clinical presentation, diagnosis and treatment (Review)

Abstract

Vasoactive intestinal peptide (VIP) secreting tumour (VIPoma) is a rare neuroendocrine tumour that most often originates from pancreatic islet cells and affects one in ten million individuals per year. In adults, it develops most commonly in the fortieth year of life with a sparse female predominance. Excessive VIP secretion induces refractory watery diarrhoea, hypokalemia and achlorhydria. Other symptoms include hyperglycemia (20-50%), hypercalcaemia (25-50%), hypochlorhydria (20-50%) and flushing (15-30%). VIP plasma levels are increased in almost all patients with VIPoma, and, together with profusing secretory diarrhoea, it is sufficient to establish the diagnosis. Moreover, the majority of VIPomas are malignant or have already metastasised at the time of diagnosis. The treatment of the neoplasm comprises medical management and surgery. While surgery remains the gold standard of treatment, peptide receptor radionuclide therapy represents one of the most effective and well-tolerated treatment options. The average survival rate of patients with VIPoma is 96 months. The objective of this review was to summarise all features of pancreatic VIPoma, as well as present novel treatment approaches for this rare neoplasm.

Rare Cases of Pediatric Vasoactive Intestinal Peptide Secreting Tumor With Literature Review: A Challenging Etiology of Chronic Diarrhea

“…Everolimus and sunitinib are two promising MTTs as advanced therapy, participating as mammalian target of rapamycin (mTOR) inhibitor and vascular endothelial growth factor (VEGF) pathway inhibitor, respectively. These MTT demonstrate certain potential on metastatic diseases while sustaining a better progression-free survival (1). Successful treatment with sunitinib for adult has been reported, and the ENETS guideline has approved everolimus as a therapeutic option (46,47).…”

“…SSA is the mainstay of medical treatment, which could attain up to 83% of sustained response in all-aged study ( 5 ). SSA binds to the somatostatin receptors, facilitating the regulation of hormone secretion and the inhibition of tumor growth ( 1 ). Yet the application was less described in children.…”

“…Vasoactive intestinal peptide (VIP) secreting tumors (VIPoma) comprise several types of neuroendocrine tumor (NET), secreting VIP, which results in the “WDHA syndrome”: watery diarrhea, hypokalemia, and achlorhydria ( 1 ). Other presentations include hyperglycemia, metabolic acidosis, flushing, abdominal distention disproportionate to the nutritional state, and growth arrest ( 1 – 3 ). The estimated annual incidence is around one in 10 million people, yet the incidence of the pediatric population is unclear ( 3 ).…”

Vasoactive Intestinal Peptide-Secreting Pheochromocytoma: A Case Report and Review of Literature

“…1 Patients commonly present with a profound secretory diarrhea (often !3 L/day) that persists with fasting (54.5%), electrolyte abnormalities such as hypomagnesemia or hypokalemia (45.6%), and hypochlorhydria/achlorhydria (42.4%). 1 Other possible features include hypotension from vasodilation, a hypokalemic nonanion gap metabolic acidosis from bicarbonate wasting, hyperglycemia (20%-50%) secondary to increased glycogenolysis, and hypercalcemia (25%-50%) likely due to dysregulation of bone metabolism. 1,8 VIP-secreting pheochromocytomas have only been reported in case reports, and studies with larger series are not available (Table 2).…”

“…1 Other possible features include hypotension from vasodilation, a hypokalemic nonanion gap metabolic acidosis from bicarbonate wasting, hyperglycemia (20%-50%) secondary to increased glycogenolysis, and hypercalcemia (25%-50%) likely due to dysregulation of bone metabolism. 1,8 VIP-secreting pheochromocytomas have only been reported in case reports, and studies with larger series are not available (Table 2). These tumors were generally large on diagnosis and presented with classical symptoms of excess catecholamine release (hypertension, paroxysmal headaches, and palpitations) or symptoms of WDHA syndrome.…”