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A 46-year-old male was referred to his local hospital with palpitations and a 6-month history of severe watery diarrhoea associated with weight loss; nausea; profound, refractory hypokalaemia (Potassium 2.6, NR 3.5–5.3mmol/l); and acute kidney injury. Bowel motions were large-volume, watery, and passed up to 10 times per day, with the absence of blood or mucous. He had no symptoms of flushing or wheezing, nor symptoms suggestive of hypo- or hyperglycaemia; however, reported a transient ‘eczema-like’ rash which resolved with later systemic treatment.
Figure 1 shows a near-diagnostic image from CT imaging, which reveals a lesion within the tail of an atrophic pancreas, with multiple hepatic metastases. It also demonstrates distended small and large bowel with fluid levels consistent with secretory diarrhoea. Liver lesions were octreotide-avid on octreoscan. Vasoactive Intestinal Polypeptide (VIP) was 784 (0–30pmol/l), glucagon 682 (0–50pmol/l), chromogranin A 268 (0–60pmol/l), chromogranin B 303 (0–150pmol/l) and pancreatic polypeptide 1972 (0–300pmol/l). Urinary 5-hydroxyindoleacetic acid was elevated at 66 (0–50µmmol/24h). Histology on liver biopsy was of a well-differentiated grade 1 neuroendocrine tumour.
A diagnosis of metastatic functioning pancreatic neuroendocrine tumour co-secreting VIP and glucagon was made. The rash was thought to be necrolytic migratory erythema.
This patient is being treated with life-long somatostatin analogue therapy (lanreotide 120mg subcutaneously every 28 days, with 100µg octreotide as required for diarrhoea). This treatment has resulted in full resolution of gastrointestinal symptoms and follow-up imaging at 6 months demonstrated stable disease with resolution of previous bowel findings. He remains under close review.
Conflict of interest: None declared.