Vasoactive intestinal polypeptide secreting pancreatic tumour (VIPoma) with liver metastases in a 46-year-old male, long term survival after orthotopic liver transplantation
Philip Johnston1, Joy Ardill2, Brian Johnston2 & David Mc Cance2
A 46-year-old male presented in 1981 with a 2 year history of profuse watery diarrhoea, three stone weight loss and fatigue. On examination he appeared gaunt with diffuse muscle weakness. Investigations revealed hypokalaemia (2.5 mmol/l; NR 3.54.5), achlorhydria and a raised vasoactive intestinal polypeptide (VIP) (1500ng/L; NR 0100). Abdominal CT showed a 5 cm pancreatic mass but with no focal liver pathology. A distal pancreatectomy was performed. Histology confirmed an islet cell carcinoma (VIPoma).
His symptoms recurred one year post surgery, at which time liver metastases were demonstrated radiologically. He responded initially to three courses of Streptozotocin but ultimately developed resistance. For fifteen years his symptoms were controlled by octreotide injections, initially Sandostatin (subcutaneously) and later Sandostatin LAR. The patient also underwent hepatic chemoembolisation.
By 1997, sixteen years after his initial surgery, treatment failure occurred with a profound deterioration clinically and debilitating diarrhoea. No evidence of extra hepatic disease was found. After extensive discussion he underwent orthotopic liver transplantation which resulted in resolution of his symptoms.
Recurrence was noted two years post transplant in the para-aortic lymph nodes but not in the liver. He remained mildly symptomatic with gradual deterioration of his general health and died 9 years after liver transplantation.
This case is one of the longest reported (25 years) survivors of a VIPoma after initial diagnosis. The case also has several notable features including the absence of liver metastases at diagnosis and the variety of treatment modalities used for symptom control including successful orthotopic liver transplantation.