Endocrine Abstracts

Pancreatic endocrine tumors (EPT) may occur sporadically or in association with the rare autosomal dominantly inherited tumor syndromes; multiple endocrine neoplasia type 1 (MEN1) and von Hipple Lindau (VHL). The genes causing these syndromes have been identified, and genotyping is possible which enables the laborious clinical investigations for diagnosis of lesions to be restricted to 50% of family members. For MEN1, no clinically useful genotype-phenotype correlation as been discovered and in a majority of patients the EPT will undergo malignant transformation. Timely identification and intervention by surgery before development of metastases currently represents the only cure of the disease. Thus, repeated extensive biochemical and radiological investigations for early recognition of small EPT in situ should be considered in asymptomatic gene carriers. Efficacies of genetic and hormonal screening programs as well as imaging will be discussed.