Pancreatic endocrine tumours

Pancreatic endocrine tumours produce several characteristic clinical syndromes. Many tumours produce (but do not necessarily secrete) more than one hormone and are multicellular. They represents examples of tumour cell heterogeneity and are highly complex biological systems. The precursor processing and storage capacity and the secretory mechanism of peptides are probably defective in many tumours. The detection of the alpha-chain of glycoprotein hormones and, less often, of human chorionic gonadotropin or its beta-subunit is useful because these substance appear to be produced virtually exclusively by malignant pancreatic endocrine tumours. This finding raises the question of the relationship of an oncogene to the production of these substances in detectable amounts. Diagnostic procedures as proposed in Figure 6 have proved useful in our hands. In addition, systematic investigation of the production and secretion of hormones, including subunits of glycoprotein hormones, and of neuronespecific enolase may permit detection of small, potentially curable, tumour foci. Moreover, the course of incurable tumours and the effect of therapy can often be monitored.