Insulinoma is the most frequently encountered functioning endocrine pancrea
tic tumor in humans. In this overview we summarize morphological and clinic
al features of insulinomas, report about the proinsulin-insulin conversion
in normal and neoplastic B-cells, discuss the new classification, the crite
ria of malignancy, and the clonal composition of endocrine pancreatic tumor
s, and outline recent findings on the molecular pathology of these tumors.