title: Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours.
abstract: Although recent epidemiological evidence indicates that the prevalence of non-functioning gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) is rising, a significant number of GEP-NETs still present with symptoms related to the secretion of biologically active substances leading to the development of distinct clinical syndromes. In the past, these syndromes were associated with substantial morbidity and mortality due to the lack of specific therapies; however, since the introduction of long-acting somatostatin analogues and medications such as proton pump inhibitors, their control has been greatly improved. As a result, nowadays, the main cause of morbidity and mortality in GEP-NETs is mostly directly related to tumour growth and the extent of metastatic disease. However, in some patients with functioning tumours and extensive disease, control of the secretory syndrome still remains problematic, necessitating the employment of several cytoreductive techniques, which may not always be sufficient. Recently, new agents directed against tumour growth, or exerting increased binding activity to receptors expressed in these tumours, or interfering with the synthetic pathway of some of the compounds secreted by these tumours, have been developed. Since there are no specific guidelines addressing the totality of the management of the secretory syndromes related to GEP-NETs, this review aims at critically analysing the medical management of previously recognised secretory syndromes; it also addresses areas of uncertainty, assesses the newer therapeutic developments and also addresses recently described but poorly characterised secretory syndromes related to GEP-NETs.
authors.affiliation: The Arden NET CoEWarwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism, University Hospitals of Coventry and Warwickshire NHS Trust, Coventry, UK Division of Translational and Experimental MedicineWarwick Medical School, University of Warwick, Coventry, UK Division of Endocrinology and Investigative MedicineImperial College London, Hammersmith Campus, London, UK g.dimitriadis@warwick.ac.uk.
authors.forename: Georgios K
authors.initials: GK
authors.lastname: Dimitriadis
authors.affiliation: The Arden NET CoEWarwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism, University Hospitals of Coventry and Warwickshire NHS Trust, Coventry, UK Division of Translational and Experimental MedicineWarwick Medical School, University of Warwick, Coventry, UK Centre for Applied Biological and Exercise SciencesCoventry University, Coventry, UK.
authors.forename: Martin O
authors.initials: MO
authors.lastname: Weickert
authors.affiliation: The Arden NET CoEWarwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism, University Hospitals of Coventry and Warwickshire NHS Trust, Coventry, UK Division of Translational and Experimental MedicineWarwick Medical School, University of Warwick, Coventry, UK Centre for Applied Biological and Exercise SciencesCoventry University, Coventry, UK.
authors.forename: Harpal S
authors.initials: HS
authors.lastname: Randeva
authors.affiliation: The Arden NET CoEWarwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism, University Hospitals of Coventry and Warwickshire NHS Trust, Coventry, UK Division of PathophysiologyNational and Kapodistrian University of Athens Medical School, Athens, Greece Oxford Center for DiabetesEndocrinology and Metabolism, University of Oxford, Oxford, UK.
authors.forename: Gregory
authors.initials: G
authors.lastname: Kaltsas
authors.affiliation: Oxford Center for DiabetesEndocrinology and Metabolism, University of Oxford, Oxford, UK.
authors.forename: Ashley
authors.initials: A
authors.lastname: Grossman
mesh_terms: D005770:Gastrointestinal Neoplasms / Q000188:drug therapy*; D006801:Humans; D018358:Neuroendocrine Tumors / Q000188:drug therapy*; D010190:Pancreatic Neoplasms / Q000188:drug therapy*; D009384:Paraneoplastic Endocrine Syndromes / Q000188:drug therapy*; D013004:Somatostatin / Q000031:analogs & derivatives / Q000627:therapeutic use
chemical_list: D013004:Somatostatin
keywords: VIPoma; carcinoid syndrome; ectopic hormonal secretion; glucagonoma; insulinoma; neuroendocrine tumours; secretory syndrome