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- Title
Vasoactive intestinal polypeptide secreting pancreatic tumour (VIPoma) with recurrent metastases in a 46 year old male, long term survival after orthoptic liver transplantation.
- Authors
Johnston, P. C.; Ardill, J. E.; Johnston, B. M.; McCance, D. R.
- Abstract
Case Report: A 46 year old male presented in 1981 with a two year history of profuse watery diarrhoea, three stone weight loss and fatigue. On examination he appeared gaunt with diffuse muscle weakness. Investigations revealed hypokalaemia - 2.5mmol/L (3.5-4.5), achlorydria and a raised vasoactive intestinal polypeptide (VIP) -500ng/L (0-100). Abdominal CT revealed a 5 cm pancreatic mass but with no focal liver pathology. A distal pancreatectomy was performed the histology of which 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 (subcut) 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 orthoptic 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 the longest reported (25 years) survival of a VIPoma after initial diagnosis. The case also has several notable features including the absence of liver metastases at diagnosis, the variety of modalities of treatment used for symptom control including successful orthoptic liver transplantation.
- Subjects
DIARRHEA; WEIGHT loss; FATIGUE (Physiology); MUSCLES; POLYPEPTIDES; LIVER transplantation
- Publication
Ulster Medical Journal, 2008, Vol 77, Issue 2, p139
- ISSN
0041-6193
- Publication type
Article