{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T02:17:21Z","timestamp":1792808241079,"version":"4.5.0"},"reference-count":28,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2017,12]]},"abstract":"<jats:sec><jats:title>OBJECTIVE<\/jats:title><jats:p>Isolated optic nerve gliomas (IONGs) constitute a rare subgroup of optic pathway gliomas (OPGs). Due to the rarity of this condition and the difficulty in differentiating IONGs from other types of OPGs in most clinical series, little is known about these tumors. Currently, due to lack of evidence, they are managed the same as any other OPG.<\/jats:p><\/jats:sec><jats:sec><jats:title>METHODS<\/jats:title><jats:p>The authors conducted a multicenter retrospective cohort study aimed at determining the natural history of IONGs. Included were patients with clear-cut glioma of the optic nerve without posterior (chiasmatic\/hypothalamic) involvement. At least 1 year of follow-up, 2 MRI studies, and 2 neuro-ophthalmological examinations were required for inclusion.<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS<\/jats:title><jats:p>Thirty-six patients with 39 tumors were included in this study. Age at diagnosis ranged between 6 months and 16 years (average 6 years). The mean follow-up time was 5.6 years. Twenty-five patients had neurofibromatosis Type 1. During the follow-up period, 59% of the tumors progressed, 23% remained stable, and 18% (all with neurofibromatosis Type 1) displayed some degree of spontaneous regression. Fifty-one percent of the patients presented with visual decline, of whom 90% experienced further deterioration. Nine patients were treated with chemotherapy, 5 of whom improved visually. Ten patients underwent operation, and no local or distal recurrence was noted.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSIONS<\/jats:title><jats:p>Isolated optic nerve gliomas are highly dynamic tumors. Radiological progression and visual deterioration occur in greater percentages than in the general population of patients with OPGs. Response to chemotherapy may be better in this group, and its use should be considered early in the course of the disease.<\/jats:p><\/jats:sec>","DOI":"10.3171\/2017.6.peds17107","type":"journal-article","created":{"date-parts":[[2017,10,6]],"date-time":"2017-10-06T13:57:20Z","timestamp":1507298240000},"page":"549-555","source":"Crossref","is-referenced-by-count":20,"title":["Isolated optic nerve gliomas: a multicenter historical cohort study"],"prefix":"10.3171","volume":"20","author":[{"given":"Ben","family":"Shofty","sequence":"first","affiliation":[{"name":"The Gilbert Israeli Neurofibromatosis Center, Dana Children\u2019s Hospital;"},{"name":"Division of Neurosurgery;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liat","family":"Ben-Sira","sequence":"additional","affiliation":[{"name":"The Gilbert Israeli Neurofibromatosis Center, Dana Children\u2019s Hospital;"},{"name":"Pediatric Radiology;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anat","family":"Kesler","sequence":"additional","affiliation":[{"name":"The Gilbert Israeli Neurofibromatosis Center, Dana Children\u2019s Hospital;"},{"name":"Division of Ophthalmology; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"George","family":"Jallo","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins School of Medicine and Hospital, Baltimore, Maryland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mari L.","family":"Groves","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins School of Medicine and Hospital, Baltimore, Maryland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rajiv R.","family":"Iyer","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins School of Medicine and Hospital, Baltimore, Maryland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alvaro","family":"Lassaletta","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uri","family":"Tabori","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Bouffet","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrich-Wilhelm","family":"Thomale","sequence":"additional","affiliation":[{"name":"Pediatric Neurosurgery, Charit\u00e9 Universit\u00e4tsmedizin, Campus Virchow Klinikum, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pablo","family":"Hern\u00e1iz Driever","sequence":"additional","affiliation":[{"name":"Pediatric Neurosurgery, Charit\u00e9 Universit\u00e4tsmedizin, Campus Virchow Klinikum, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shlomi","family":"Constantini","sequence":"additional","affiliation":[{"name":"The Gilbert Israeli Neurofibromatosis Center, Dana Children\u2019s Hospital;"},{"name":"Pediatric Neurosurgery, Dana Children\u2019s Hospital, Tel Aviv Medical Center and Tel Aviv University, Tel Aviv, Israel;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"_","family":"_","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1","series-title":"AJNR Am J Neuroradiol","first-page":"666","article-title":"Defining optic nerve tortuosity","volume":"28","author":"Armstrong GT","year":"2007","unstructured":"Armstrong GT, Localio AR, Feygin T, Bilaniuk L, Phillips PC, Fisher MJ, : Defining optic nerve tortuosity. 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