Insulinomas are rare tumors and account for 90% of all endocrine pancr
eatic tumors. They typically present as a solitary tumor, but may occu
r in multiple sites (e.g. multiple endocrine neoplasia type I) or as a
malignant disease in 10% of cases and rarely as nesidioblastosis or i
slet cell adenomatosis. Neuroglucopenic symptoms lead to the diagnosis
; inadequate high insulin and C-peptide secretion with hypoglycemia in
the fasting test confirm the diagnosis. Preoperative localization is
not necessary prior to the first operation. The standard operation is
enucleation or, depending on size and location, resection. The treatme
nt of multiple tumors and islet cell hyperplasia with a high risk of r
ecurrence is problematic. Subtotal resection plus enucleation seems to
be better than selective tumor resection. In malignant insulinomas, m
ostly presenting with liver metastases, aggressive surgical therapy wi
th hepatectomy and debulking, chemoembolization and systemic chemother
apy are the modalities of choice.