title: Challenges and controversies in management of pancreatic neuroendocrine tumours in patients with MEN1.
abstract: Multiple endocrine neoplasia type 1 (MEN1), an autosomal dominant disorder, is characterised by the occurrence of pancreatic neuroendocrine tumours (P-NETs) in association with parathyroid and pituitary tumours. P-NETs, which include gastrinomas, insulinomas, and non-functioning tumours, occur in more than 80% of MEN1 patients and account for 50% of disease-specific deaths. However, there is no consensus about the optimal methods for detecting and treating P-NETs in MEN1 patients, and extrapolations from approaches used in patients with non-familial (sporadic) P-NETs require caution because of differences, such as the younger age of onset, multi-focality of P-NETs, and concomitant presence of other tumours in MEN1 patients. Thus, the early detection of P-NETs by circulating biomarkers and imaging modalities, and their appropriate treatments by surgical approaches and/or radionuclide therapy, chemotherapy, and biotherapy pose challenges and controversies. These challenges and controversies will be reviewed and possible approaches proposed.
authors.affiliation: Academic Endocrine Unit, Radcliffe Department of Clinical Medicine, University of Oxford, Oxford, UK; Department of Diabetes and Endocrinology, Melbourne Health, Melbourne, VIC, Australia; Department of Diabetes and Endocrinology, Western Health, Melbourne, VIC, Australia; Department of Medicine, University of Melbourne, Melbourne, VIC, Australia.
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Yates
authors.affiliation: Academic Endocrine Unit, Radcliffe Department of Clinical Medicine, University of Oxford, Oxford, UK; Division of Cardiovascular and Diabetes Medicine, Ninewells Hospital and Medical School, University of Dundee, Dundee, UK.
authors.forename: Paul J
authors.initials: PJ
authors.lastname: Newey
authors.affiliation: Academic Endocrine Unit, Radcliffe Department of Clinical Medicine, University of Oxford, Oxford, UK. Electronic address: rajesh.thakker@ndm.ox.ac.uk.
authors.forename: Rajesh V
authors.initials: RV
authors.lastname: Thakker
mesh_terms: D015415:Biomarkers; D019468:Disease Management; D006801:Humans; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D018358:Neuroendocrine Tumors / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D010190:Pancreatic Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers