Pancreatic Pathology Online

Pathology of VIPomas (Vasoactive Intestinal Peptide-Secreting Tumours) of the Pancreas

Dr Sampurna Roy MD        July 2016

 

Vasoactive intestinal peptide-secreting tumours (VIPomas) are rare islet cell tumours of the pancreas that can result in life-threatening biochemical abnormalities.

These tumours synthesize and secrete vasoactive intestinal polypeptide (VIP).

VIPomas give rise to the Verner-Morrison syndrome, which is characterized by explosive and profuse watery diarrhea, accompanied by hypokalemia and hypochlorhydia.  [Watery diarrhea, hypokalemia, and achlorhydria - WDHA ].

The loss of water may be as high as 5 liters per day, with an attendant excretion of up to 300 mmol potassium.

Severe dehydration with debility are frequent complications, which, together with the intractable diarrhea, has led to the term pancreatic cholera.

These profound effects are due to the excessive activation of adenyl cyclase in the mucosal cells of the small intestine by vasoactive intestinal peptide.

In turn, high levels of cAMP drive the active secretion of intracellular electrolytes, including potassium and water.

The tumours are usually solitary and benign,and there are a few reports of malignant behavior.

High levels of circulating vasoactive intestinal peptide and severe diarrhea have also been encountered in patients with a variety of nonpancreatic neoplasms containing different types of APUD cells, for example, ganglioneuroma, pheochromocytoma of the adrenal medulla, medullary thyroid carcinoma, and bronchogenic carcinoma.

In some patients the Verner-Morrison syndrome is caused by the multiple endocrine neoplasia (MEN) syndrome, Type 1.

This familial disorder is characterized by neoplasms in several endocrine organs simultaneously, especially adenomatous tumours of the pituitary and parathyroid glands.

 

Further reading:

VIPoma syndrome.

The morphology and neuroendocrine profile of pancreatic epithelial VIPomas and extrapancreatic, VIP-producing, neurogenic tumors.

Radiofrequency ablation has a valuable therapeutic role in metastatic VIPoma.

Large cell carcinoma with calcitonin and vasoactive intestinal polypeptide-associated Verner-Morrison syndrome.

Pancreatic VIPomas: subject review and one institutional experience.

Diagnosis and treatment of VIPoma: a case report and literature review in China.

Vipoma of the pancreas. Apropos of a case and review of the literature.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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