Diagnosis and treatment of VIPoma in a female patient

We report a case of VIPoma in an 83-year-old female patient, who presented with frequent and excessive diarrhoea, muscle weakness, and severe hypokalaemia. Abdominal computed tomography (CT) revealed a 4x6 cm mass in the body of the pancreas. Laboratory analysis showed elevated levels of both vasoactive intestinal polypeptide (VIP; 153 pmol/l) and pancreatic polypeptide (161 pmol/l). In view of the patient's age, physical condition, and tumour size, surgical resection was not performed. The patient was treated with a long-acting octreotide, after which her symptoms diminished. After 24 months of follow-up, the patient remained in good physical condition without any further serious gastrointestinal symptoms. The VIPoma syndrome is caused by a neuroendocrine tumour, usually located in the pancreas, which secretes VIP, causing severe diarrhoea, dehydration and hypokalaemia. Treatment options include resection of the tumour, chemotherapy or the reduction of symptoms with somatostatin analogues. We provide an overview of the incidence, pathophysiology, diagnosis, treatment strategies, and prognosis of this rare syndrome.