Endocrine Abstracts

Case history: A 59-year-old male presented to gastroenterology with a two years of high volume persistent watery diarrhoea. He gave a past medical history of poorly controlled, insulin treated diabetes mellitus present for 8 years. He reported abdominal pain and diarrhoea that persisted despite fasting. A colonoscopy with biopsy were reported as normal. Diarrhoea, malaise, fatigue and weight loss persisted.

Investigations: Stool culture and faecal elastase were unremarkable. CT imaging revealed a large pancreatic mass involving the body and tail of the pancreas and liver lesions were noted. Tissue diagnosis confirmed an ENETS grade 2 pancreatic NET with strong synaptophysin expression, weak chromogranin A, Cytokeratins positive and Ki67 of 6%. Initial biochemistry: Na 128, K 2.8, Urea 9.1, Creatinine 142. Severe metabolic acidosis pH of 7.14 to 7.19, bicarbonate of 19 with worsening renal function was also noted. Gut peptides revealed Glucagon 48 (0–49 pmol/L), Gastrin 13 (0–39 pmol/L), P Polypeptide >500 (0–299 pmol/L), Somatostatin >1000 (0–149 pmol/L), VI Polypeptide (ViP) 102(0–29 pmol/L), Chromogranin A 130.5(0–6 nmol/L). Octreoscan confirmed avidity with disease limited to the pancreas and liver.

Results and treatment: Management required urgent electrolyte replacement, octreotide, initially given subcutaneously and correction of the acidosis with bicarbonate infusions. Diarrhoea persisted with bowel movements up to 8–10 times. Lanreotide autogel 120 mg injection was also started. Glucocorticoids were commenced to improve syndrome functionality. VRIII was used to maintain normoglycaemia. Transferred to HDU for central line access. To improve his nutritional status total parenteral nutrition was also commenced via PICC line. Electrolytes improved and gradually doses of Octreotide and steroid were weaned. He was sent home self-managing the octreotide, insulin and with ambulatory TPN in the community and for peptide receptor radionuclide therapy. To date he has received two cycles of treatment with Lutetium DOTATE with good response in terms of reduction of VIP levels, cessation of diarrhoea and reduction in tumour load.

Conclusions and points for discussion: ViPOMA is a rare pancreatic neuroendocrine tumour with an annual incidence of 1/10 million. It is also known as pancreatic cholera or the Verner Morrison syndrome or WDHA (watery diarrhoea hypokalaemia and achlorhydria) syndrome.Treatment is to initially prevent death from dehydration but also includes octreotide which inhibits the actions of ViP. Surgery offers cure where disease is localised. Peptide receptor radionuclide therapy which has also been shown to be highly effective.