{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,18]],"date-time":"2026-03-18T04:07:49Z","timestamp":1773806869660,"version":"3.50.1"},"reference-count":37,"publisher":"The Endocrine Society","issue":"6","license":[{"start":{"date-parts":[[2020,3,28]],"date-time":"2020-03-28T00:00:00Z","timestamp":1585353600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,6,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Context<\/jats:title>\n                  <jats:p>Up to 20% of children with neurofibromatosis type 1 (NF1) develop low-grade optic pathway gliomas (OPGs) that can result in endocrine dysfunction. Data on prevalence and type of endocrine disorders in NF1-related OPGs are scarce.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>The aim of the study was to determine the prevalence of endocrine dysfunctions in patients with NF1 and OPGs and to investigate predictive factors before oncological treatment.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Design<\/jats:title>\n                  <jats:p>Multicenter retrospective study.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Settings and patients<\/jats:title>\n                  <jats:p>Records were reviewed for 116 children (64 females, 52 males) with NF1 and OPGs followed at 4 Italian centers.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Main outcome measures<\/jats:title>\n                  <jats:p>We evaluated endocrine function and reviewed brain imaging at the time of OPG diagnosis before radio- and chemotherapy and\/or surgery. OPGs were classified according to the modified Dodge classification.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Thirty-two children (27.6%) with a median age of 7.8 years had endocrine dysfunctions including central precocious puberty in 23 (71.9%), growth hormone deficiency in 3 (9.4%), diencephalic syndrome in 4 (12.5%), and growth hormone hypersecretion in 2 (6.2%). In a multivariate cox regression analysis, hypothalamic involvement was the only independent predictor of endocrine dysfunctions (hazard ratio 5.02 [1.802-13.983]; P = .002).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>Endocrine disorders were found in approximately one-third of patients with Neurofibromatosis type 1 and OPGs before any oncological treatment, central precocious puberty being the most prevalent. Sign of diencephalic syndrome and growth hormone hypersecretion, although rare, could be predictive of optic pathway gliomas in NF1. Tumor location was the most important predictor of endocrine disorders, particularly hypothalamic involvement.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1210\/clinem\/dgaa138","type":"journal-article","created":{"date-parts":[[2020,4,15]],"date-time":"2020-04-15T17:27:24Z","timestamp":1586971644000},"page":"e2214-e2221","source":"Crossref","is-referenced-by-count":32,"title":["Pretreatment Endocrine Disorders Due to Optic Pathway Gliomas in Pediatric Neurofibromatosis Type 1: Multicenter Study"],"prefix":"10.1210","volume":"105","author":[{"given":"Claudia","family":"Santoro","sequence":"first","affiliation":[{"name":"Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica, Universit\u00e0 degli studi della Campania \u201cLuigi Vanvitelli\u201d, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Silverio","family":"Perrotta","sequence":"additional","affiliation":[{"name":"Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica, Universit\u00e0 degli studi della Campania \u201cLuigi Vanvitelli\u201d, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefania","family":"Picariello","sequence":"additional","affiliation":[{"name":"Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica, Universit\u00e0 degli studi della Campania \u201cLuigi Vanvitelli\u201d, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martina","family":"Scilipoti","sequence":"additional","affiliation":[{"name":"Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica, Universit\u00e0 degli studi della Campania \u201cLuigi Vanvitelli\u201d, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mario","family":"Cirillo","sequence":"additional","affiliation":[{"name":"Dipartimento di Scienze Mediche, Chirurgiche, Neurologiche, Metaboliche e dell\u2019Invecchiamento, Universit\u00e0 della Campania \u201cLuigi Vanvitelli\u201d, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lucia","family":"Quaglietta","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology, Santobono-Pausilipon Children\u2019s Hospital, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giuseppe","family":"Cinalli","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurosurgery Santobono-Pausilipon Children\u2019s Hospital, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniela","family":"Cioffi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Santobono-Pausilipon Children\u2019s Hospital, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Natascia","family":"Di Iorgi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, IRCCS Istituto Giannina Gaslini, Genova, Italy"},{"name":"Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health University of Genova, Genova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohamad","family":"Maghnie","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, IRCCS Istituto Giannina Gaslini, Genova, Italy"},{"name":"Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health University of Genova, Genova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annalisa","family":"Gallizia","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, IRCCS Istituto Giannina Gaslini, Genova, Italy"},{"name":"Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health University of Genova, Genova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria","family":"Parpagnoli","sequence":"additional","affiliation":[{"name":"\u2018Meyer\u2019 Children Hospital, Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federica","family":"Messa","sequence":"additional","affiliation":[{"name":"\u2018Meyer\u2019 Children Hospital, Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luisa","family":"De Sanctis","sequence":"additional","affiliation":[{"name":"Pediatric Endocrinology, Regina Margherita Children Hospital, University of Turin, Turin, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Silvia","family":"Vannelli","sequence":"additional","affiliation":[{"name":"Pediatric Endocrinology, Regina Margherita Children Hospital, University of Turin, Turin, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4682-0170","authenticated-orcid":false,"given":"Pierluigi","family":"Marzuillo","sequence":"additional","affiliation":[{"name":"Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica, Universit\u00e0 degli studi della Campania \u201cLuigi Vanvitelli\u201d, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emanuele","family":"Miraglia del Giudice","sequence":"additional","affiliation":[{"name":"Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica, Universit\u00e0 degli studi della Campania \u201cLuigi Vanvitelli\u201d, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6343-4768","authenticated-orcid":false,"given":"Anna","family":"Grandone","sequence":"additional","affiliation":[{"name":"Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica, Universit\u00e0 degli studi della Campania \u201cLuigi Vanvitelli\u201d, Naples, 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with neurofibromatosis-1: a real possibility","volume":"14","author":"Perilongo","year":"1999","journal-title":"J Child Neurol."},{"issue":"1","key":"2021122105023928100_CIT0007","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/S0022-3476(94)70122-9","article-title":"Natural history of optic pathway tumors in children with neurofibromatosis type 1: a longitudinal study","volume":"125","author":"Listernick","year":"1994","journal-title":"J Pediatr."},{"issue":"9","key":"2021122105023928100_CIT0008","first-page":"1691","article-title":"Spontaneous decrease of a pilocytic astrocytoma in neurofibromatosis type 1","volume":"17","author":"Leisti","year":"1996","journal-title":"AJNR Am J Neuroradiol."},{"issue":"3","key":"2021122105023928100_CIT0009","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1007\/s002340050734","article-title":"Spontaneous remission of chiasmatic\/hypothalamic masses in neurofibromatosis type 1: report of two 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children and adolescents of the German speaking society of pediatric oncology and hematology","volume":"14","author":"Gnekow","year":"2012","journal-title":"Neuro Oncol."},{"issue":"3","key":"2021122105023928100_CIT0013","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1097\/NEN.0b013e318165eb75","article-title":"Gliomas in neurofibromatosis type 1: a clinicopathologic study of 100 patients","volume":"67","author":"Rodriguez","year":"2008","journal-title":"J Neuropathol Exp Neurol."},{"issue":"4","key":"2021122105023928100_CIT0014","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1159\/000369802","article-title":"Endocrine implications of neurofibromatosis 1 in childhood","volume":"83","author":"Bizzarri","year":"2015","journal-title":"Horm Res Paediatr."},{"issue":"4","key":"2021122105023928100_CIT0015","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1542\/peds.100.4.667","article-title":"Endocrinologic disorders and optic pathway gliomas in children with 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National Institutes of Health Consensus Development Conference","volume":"45","year":"1988","journal-title":"Arch Neurol."},{"issue":"10","key":"2021122105023928100_CIT0019","doi-asserted-by":"crossref","first-page":"3787","DOI":"10.1210\/jc.2015-2028","article-title":"Neuroendocrine morbidity after pediatric optic gliomas: a longitudinal analysis of 166 children over 30 years","volume":"100","author":"Gan","year":"2015","journal-title":"J Clin Endocrinol Metab."},{"issue":"8","key":"2021122105023928100_CIT0020","first-page":"1499","article-title":"Diencephalic syndrome: clinical features and imaging findings","volume":"18","author":"Poussaint","year":"1997","journal-title":"AJNR Am J Neuroradiol."},{"issue":"11","key":"2021122105023928100_CIT0021","doi-asserted-by":"crossref","first-page":"3972","DOI":"10.1210\/jc.2014-1680","article-title":"Diencephalic syndrome in childhood craniopharyngioma\u2013results of German multicenter studies on 485 long-term survivors of childhood 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Pediatr."},{"issue":"2","key":"2021122105023928100_CIT0025","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1093\/neuonc\/noq178","article-title":"Survival and long-term health and cognitive outcomes after low-grade glioma","volume":"13","author":"Armstrong","year":"2011","journal-title":"Neuro Oncol."},{"issue":"22","key":"2021122105023928100_CIT0026","doi-asserted-by":"crossref","first-page":"3691","DOI":"10.1200\/JCO.2008.21.2738","article-title":"Late effects of conformal radiation therapy for pediatric patients with low-grade glioma: prospective evaluation of cognitive, endocrine, and hearing deficits","volume":"27","author":"Merchant","year":"2009","journal-title":"J Clin Oncol."},{"issue":"8","key":"2021122105023928100_CIT0027","doi-asserted-by":"crossref","first-page":"2761","DOI":"10.1210\/jc.2018-01175","article-title":"Hypothalamic-pituitary and growth disorders in survivors of childhood cancer: an endocrine society clinical practice 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