Endocrine Abstracts

Multiple Endocrine Neoplasia Type 1 (MEN1) and the von Hippel Lindau syndrome (VHL) are hereditary disease with an autosomal dominant inheritance. Among a wide variety of manifestations, both syndromes predispose patients to pancreatic neuro-endocrine neoplasms (PanNENs). Screening for presymptomatic diagnosis of PanNENs enables timely intervention with the intention to prevent metastasized disease and premature death. MEN1 and VHL lead to different manifestations and there are also distinct differences in occurrence and clinical course of PanNEN. Therefore the management of PanNEN occurring in both syndromes is different with consequences for the required multidisciplinary team. For both tumor syndromes screening for PanNEN should be based on clinical orientation, including general planning of screening and surveillance, utility of biochemical biomarkers, the optimal choice for imaging modality, and risk stratification for individual patients. Recent research gives more insight into the main aspects of MEN1 and VHL-related pancreatic manifestations and their clinical management. For care tailored to the needs of the individual patient and improving outcomes on an individual basis, studies are now needed to define predictors of tumor behavior and effects of more individualized interventions. Patients with MEN1 and VHL are therefore preferably treated in centers with specific expertise and dedication to collaborative research.