A case is reported of a man with stage IV grade 2 pancreatic VIPoma. At diagnosis he had several features which may have expedited the diagnosis including refractory diarrhoea containing undigested foodstuffs, alcohol-related flushing, electrolyte abnormalities and intestinal oedema on imaging. Symptom control was challenging. There was no benefit from creon, loperamide or codeine and escalation to both short and long acting somatostatin analogues was futile. Consistent with a published case report, he gained rapid symptomatic benefit from the tyrosine kinase inhibitor sunitinib. The duration of benefit, however, was less than 6 months. Sunitinib is a symptom control option in somatostatin analogue-refractory VIPoma whilst other more definitive symptom or disease control options are explored. He received one dose of peptide radionuclide therapy but after this never left hospital due to deteriorating symptoms. Interferon-alpha, known to be of use in carcinoid syndrome, provided some brief symptomatic benefit but unfortunately by this time he was too far along a downward clinical trajectory and he died shortly after.
04 Dec 2017
UK and Ireland Neuroendocrine Tumour Society