Gastrointestinal endocrine tumours. Insulinoma.

Fundamental to establishing a diagnosis of insulinoma is first to consider the diagnosis when presented with the constellation of symptoms and signs that indicate hypoglycaemia. Prominent and most convincing are manifestations of neuroglycopenia. Although hypoglycaemia can be caused by a number of disorders, the combination of hypoglycaemia and endogenous hyperinsulinaemia is diagnostic of insulinoma. Our criteria now include a glucose level of 40 mg/dl with a concomitant insulin level of 6 microU/ml, a C-peptide level exceeding 200 pmol/l, and negative screen for sulphonlyurea. Ancillary diagnostic tests or the use of insulin surrogates may offer helpful confirmation. Localization is still evolving, but in our hands pre-operative ultrasound is the best and only pre-operative test that we obtain in the usual situation. Expertise and experience with other modalities at other institutions offer reasonable but more costly alternatives. Intraoperative ultrasonography provides significant benefit in both tumour localization and delineating important related anatomy. Insulinomas are virtually all located in the pancreas; 90% are benign, single, and are generally firmer than surrounding normal pancreas. Extensive exposure may be required to identify and remove safely the tumour. Enucleation is our preferred technique, but distal pancreatectomy for tumours in the body or tail is an excellent method as well. Pancreatoduodenectomy is rarely necessary. Complications most commonly relate to leak of pancreatic secretions, causing pseudocyst, abscess, or fistula. except in MEN 1 syndrome, excision of a benign insulinoma equates with disease cure, and patients are often extraordinarily grateful as the change in their lives may be profound.