Lucia Riccio1,2, Carmine Antonio Donofrio3, Giovanni Tomacelli4, Roberto De Blasi5, Alessandro Melatini6. 1. Department of Neurosurgery, Ospedale "Vito Fazzi", Piazza F. Muratore, 1, 73100, Lecce, Italy. dott.luciariccio@libero.it. 2. Vita-Salute San Raffaele University, Milan, Italy. dott.luciariccio@libero.it. 3. Department of Neurosurgery, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Manchester Academic Health Sciences Centre, University of Manchester, Manchester, UK. 4. ENT Department, Ospedale "Vito Fazzi", Lecce, Italy. 5. Department of Radiology, Azienda Ospedaliera "Cardinale Giovanni Panico", Tricase, Italy. 6. Department of Neurosurgery, Ospedale "Vito Fazzi", Piazza F. Muratore, 1, 73100, Lecce, Italy.
Abstract
PURPOSE: Acromegaly is usually due to growth hormone (GH)-secreting pituitary adenomas, but it may be exceptionally caused by GH-secreting ectopic pituitary adenomas (EPA). EPA are defined as extra-sellar pituitary tumours, extra- or intra-cranially sited, entirely separated from the pituitary stalk and gland. The aim of the study is to address the challenges in the management of clival GHEPA. METHODS: We reported a case of a 53-year-old acromegalic patient with a primary clival GHEPA and reviewed systematically the relevant English literature between 1975 and 2019, in keeping with the PRISMA guidelines. RESULTS: Four cases of primary clival GHEPA have been described in literature apart from ours. All patients presented with acromegalic features, elevated circulating GH and/or insulin-like growth factor-1 levels. Hyperprolactinemia and empty sella were described in two cases, respectively. These tumours show the typical imaging characteristics of pituitary adenomas, but their neuroradiological diagnosis may be challenging due to their sizes and the difficulty in defining the absence of connections with the pituitary fossa. CONCLUSION: Although primary clival GHEPA are exceedingly rare, even if likely under-reported in literature, they should be considered in the differential diagnosis of clival tumours because of their specific management. Surgery represents the first-line treatment option, while medical and radiation therapies can be adopted as neo-adjuvant, adjuvant or primary treatments according to tumour and patient characteristics.
PURPOSE:Acromegaly is usually due to growth hormone (GH)-secreting pituitary adenomas, but it may be exceptionally caused by GH-secreting ectopic pituitary adenomas (EPA). EPA are defined as extra-sellar pituitary tumours, extra- or intra-cranially sited, entirely separated from the pituitary stalk and gland. The aim of the study is to address the challenges in the management of clival GHEPA. METHODS: We reported a case of a 53-year-old acromegalicpatient with a primary clival GHEPA and reviewed systematically the relevant English literature between 1975 and 2019, in keeping with the PRISMA guidelines. RESULTS: Four cases of primary clival GHEPA have been described in literature apart from ours. All patients presented with acromegalic features, elevated circulating GH and/or insulin-like growth factor-1 levels. Hyperprolactinemia and empty sella were described in two cases, respectively. These tumours show the typical imaging characteristics of pituitary adenomas, but their neuroradiological diagnosis may be challenging due to their sizes and the difficulty in defining the absence of connections with the pituitary fossa. CONCLUSION: Although primary clival GHEPA are exceedingly rare, even if likely under-reported in literature, they should be considered in the differential diagnosis of clival tumours because of their specific management. Surgery represents the first-line treatment option, while medical and radiation therapies can be adopted as neo-adjuvant, adjuvant or primary treatments according to tumour and patient characteristics.
Authors: C Campana; F Nista; L Castelletti; M Caputo; E Lavezzi; P Marzullo; A Ferrero; G Gaggero; F R Canevari; D C Rossi; G Zona; A Lania; D Ferone; F Gatto Journal: J Endocrinol Invest Date: 2022-02-11 Impact factor: 5.467