Vasoactive intestinal peptide secreting tumour: An overview

Abstract

Vasoactive intestinal peptide (VIP) secreting tumour (VIPoma) is a rare functional neuroendocrine tumour that typically arises from pancreatic islet cells. These present as sporadic, solitary pancreatic neoplasias with an estimated incidence of one in ten million individuals per year. Only around 5% of VIPomas are associated with multiple endocrine neoplasia type I syndrome. Excessive VIP secretion produces a clinical syndrome characterized by refractory watery diarrhoea, hypokalemia and metabolic acidosis. These coupled with elevated plasma levels of VIP are diagnostic. The majority of VIPomas are malignant and have already metastasized at the time of diagnosis (60%). Metastases occur most frequently in the liver, or regional lymph nodes, lungs, kidneys and bones. Some reports of skin metastases have been documented. Complete surgical resection continues to be the only potentially curative treatment. However, when the neoplasia cannot be excised completely, surgical debulking may provide palliative benefit. Other palliative options have included recently the peptide receptor radionuclide therapy which has shown to be effective and well-tolerated. This article will review all aspects of pancreatic VIPomas highlighting aspects such as clinical presentation, diagnosis and management.

A case of vasoactive intestinal peptide-secreting tumor (VIPoma) arising from <i>MEN1</i> inactivation which recurred 15 years after the initial resection

“…Hyperglycemia or impaired glucose tolerance reportedly affects 20-50% of patients with VIPoma [10]. Multiple mechanisms have been proposed to explain this association such as the direct glycogenolytic and gluconeogenic activity of VIP in the liver and the inhibitory effect of hypokalemia on insulin release from pancreatic beta cells [2]. Glycemic control appeared to be correlated with the tumor burden of the VIPoma at the time of recurrence, suggesting that VIPoma was involved in the worsening of glycemic control in this case.…”

“…In addition, lanreotide treatment was maintained as postoperative adjuvant therapy. Although SSAs have not yet been proven to prevent the relapse of VIPoma, we expected that lanreotide would minimize the chance of further relapse because of SSAs' potential antiproliferation and tumor stabilization effects [2,9].…”

“…They can be broadly classified into non-functional and functional (hormone-producing) tumors, including gastrinoma, insulinoma, glucagonoma, somatostatinoma, or vasoactive intestinal peptide-secreting tumor (VIPoma). VIPoma is extremely rare, with an estimated incidence of less than 1 in 10 million individuals per year in the general population [2]. Most VIPomas are sporadic, whereas 5% are associated with multiple endocrine neoplasia type 1 (MEN1) syndrome [3].…”

A Rare Presentation of Lymph Node Metastasis of VIPoma After Three Years of Resection: A Case Report

“…VIPoma is one of the rarest forms of pancreatic NETs with an incidence of less than 0.22 cases per 100,000 [ 4 ]. It is usually diagnosed at late stages due to its insidious onset and the non-specific nature of the symptoms [ 5 ]. The disease usually presents itself with intractable watery diarrhea, electrolyte abnormalities such as hyponatremia and hypokalemia, and metabolic acidosis which initially lead to investigation of other etiologies of watery diarrhea such as colitis and small bowel disorders [ 3 ].…”

Pediatric Neuroendocrine Neoplasms: Rare Malignancies with Incredible Variability

“…Over 95% of insulinomas can be diagnosed by serial plasma glucose and insulin levels during a 72 h fast showing hyperinsulinemic hypoglycemia [ 73 ]. Similarly, somatostatin can be measured to aid in the diagnosis of somatostatinoma and VIP can be measured in the plasma to diagnose a VIPoma in conjunction with hypokalemia and achlorhydria [ 74 , 75 ]. Functional pituitary NENs can be detected by elevated levels of growth hormone, ACTH, IGF1, or prolactin [ 31 ].…”