Vasoactive Intestinal Peptide Tumor

Abstract

Background and aim. Vasoactive intestinal peptide tumor is a rare neuroendocrine neoplasm which causes voluminous watery diarrhea via hypersecretion of electrolytes and water from the intestinal mucosa through a vasoactive intestinal peptide-mediated, cyclic AMP-dependent mechanism. The acid base imbalance generated by the loss of water and electrolytes leads to severe dehydration and potential renal failure, which can ultimately result in death if left untreated. This paper aims to review the clinical, histological, radiological, and diagnostic features of this disease as well as the therapeutic modalities in treating this condition. Methods. A review of literature was performed using MEDLINE, Pubmed, and Cochrane databases in collection of data using MeSH terms including vasoactive intestinal peptide, VIPoma, and WDHA. Results and conclusion. Vasoactive intestinal peptide tumor is a rare neoplasm associated with significant morbidity and mortality through secretion of water and electrolytes in the gastrointestinal tract. The nonspecific clinical presentation of this neoplasm can pose diagnostic challenges, as these tumors can be easily misdiagnosed as other conditions, ranging from laxative overdose to the presence of a carcinoid secreting tumor. Nevertheless, a number of imaging and laboratory studies can facilitate the correct evaluation and diagnosis of VIPoma. Following proper diagnosis, VIPomas are treated by either medical or surgical modalities depending on the existence and extent of metastasis.

Vasoactive Intestinal Peptide-Secreting Pancreatic Neuroendocrine Tumor: A Case Report

“…VIP is a 28-amino acid neuropeptide that causes large voluminous watery diarrhea by its vasodilator effect on intestinal smooth muscle and stimulating the production of CAMP in enterocytes, which induces the secretion of water and electrolytes, especially potassium and bicarbonate, along with water into the intestinal lumen [ 3 ].…”

“…Treatment with somatostatin analog octreotide quickly resolves diarrhea. The main action of the drug is the direct inhibitory effect on the production of hormones from the tumor [ 3 ]. Inoperable tumors are managed by palliation therapy, including cytoreductive surgery for symptom control along with biologics and chemotherapy.…”

Pancreatic VIPoma as a Differential Diagnosis in Chronic Pediatric Diarrhea: A Case Report and Review of the Literature

“…In the pediatric population, VIPomas most frequently arise from the bronchus, colon, liver, and neural crest-derived tissues (e.g. sympathetic nerve chain, pituitary, thyroid, and adrenal glands), and rarely originate from the pancreas [ 16 , 17 ].…”

“…A strong clinical suspicion is necessary in order to request specific metabolic tests, such as VIP, gastrin, and glucagon (along with an electrolyte panel including potassium, sodium, and chlorine) [ 16 , 17 ]. Most reported cases present with VIP serum levels between 60 and 2,100 pg/mL, yet values higher than 200 pg/mL are diagnostic.…”