Year: 2024
Type: article
Abstract: VIPomas are rare, functional neuroendocrine tumors (NET) that are usually located in the pancreas and lead to a clinical syndrome known as Verner-Morrison syndrome.Verner-Morrison syndrome is characterized by watery diarrhea, hypokalemia, and achlorhydria (WDHA) due to the excessive release of vasoactive intestinal peptides (VIPs). 1 VIPoma symptoms are difficult to manage and may have lifethreatening consequences independent of the oncological status.Surgical resection is crucial in the treatment of primary tumors, and somatostatin analogs are used as the main treatment for symptom control when surgical resection is not possible.2 VIP is a polypeptide of 28 amino acids that induces adenylate cyclase and cyclic adenosine monophosphate production.Pathophysiologically, VIPomas are characterized by an excessive and irregular release of VIP, which leads to increased fluid and electrolyte secretion into the intestinal lumen, resulting in secretory diarrhea and hypokalemia.3 Cytotoxic chemotherapy, peptide receptor radionuclide therapy, sunitinib, and everolimus may be considered for treatment in cases that cannot be controlled using somatostatin analogs.In a study that compared the effects of sunitinib, a tyrosine kinase inhibitor, with those of placebo treatment, increased progressionfree survival and overall survival rates were noted with the use of sunitinib.4 VIPoma has a low incidence in the population and, therefore, no treatment standards are reported in the literature comprising both prospective and evidence-based studies.The objective of VIPoma treatment is not limited to tumor growth control and includes the eradication of lifethreatening symptoms as well.
Source: Journal of Oncological Sciences
Institutions: Süleyman Demirel Üniversitesi, Istinye University
Language: English
Subfield: Epidemiology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4403988942