Surgery is the only chance of curative therapy. As these tumours tend to be malignant, their removal must involve nearby tissue that may have been invaded (such as parts of the pancreas, the spleen and occasionally the duodenum). If the liver has been extensively infiltrated with metastases then resection of the primary tumour with liver transplant is the only chance of cure.
The surgery is difficult, as the anaemia and blood clotting problems require careful monitoring and management during the operation. Total parenteral nutrition is required pre-operatively for the rash. Post-operatively, prophylaxis against blood clotting is required. Surgery should therefore be attempted only if there is a good prospect of cure. Cure rates are as low as 5%.
Alternatives include cryoreductive therapy (where the tumour cells are frozen in surgery to kill them) and embolisation (where small arteries feeding the tumours are blocked off, killing the cells).
Octreotide, a somatostatin analogue, can be used to render the patient safe before surgery. This prevents the cells from producing so much glucagon. Chemotherapy is also used, the main agents being 5-fluorouracil and streptozotocin. These can lead to a reduction in glucagon concentrations of up to 80%.
This is very variable, depending on the individual circumstances. The median survival time from diagnosis is 3 years but individuals have survived for up to 20 years.