Shofty Ben, Ben-Sira Liat, Kesler Anat, Jallo George, Groves Mari L, Iyer Rajiv R, Lassaletta Alvaro, Tabori Uri, Bouffet Eric, Thomale Ulrich-Wilhelm, Hernáiz Driever Pablo, Constantini Shlomi
1The Gilbert Israeli Neurofibromatosis Center, Dana Children's Hospital.
2Division of Neurosurgery.
J Neurosurg Pediatr. 2017 Dec;20(6):549-555. doi: 10.3171/2017.6.PEDS17107. Epub 2017 Oct 6.
OBJECTIVE 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. METHODS 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. RESULTS 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. CONCLUSIONS 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.
目的 孤立性视神经胶质瘤（IONG）是视神经通路胶质瘤（OPG）中一个罕见的亚组。由于这种疾病罕见，且在大多数临床系列中难以将IONG与其他类型的OPG区分开来，因此对这些肿瘤了解甚少。目前，由于缺乏证据，它们的治疗方式与其他任何OPG相同。方法 作者进行了一项多中心回顾性队列研究，旨在确定IONG的自然病程。纳入的患者为明确诊断为视神经胶质瘤且无后部（视交叉/下丘脑）受累。纳入标准要求至少随访1年、进行2次MRI检查和2次神经眼科检查。结果 本研究纳入了36例患者的39个肿瘤。诊断时年龄在6个月至16岁之间（平均6岁）。平均随访时间为5.6年。25例患者患有1型神经纤维瘤病。在随访期间，59%的肿瘤进展，23%保持稳定，18%（均为1型神经纤维瘤病患者）出现一定程度的自发消退。51%的患者出现视力下降，其中90%视力进一步恶化。9例患者接受了化疗，其中5例视力改善。10例患者接受了手术，未发现局部或远处复发。结论 孤立性视神经胶质瘤是高度动态变化的肿瘤。与OPG患者总体相比，放射学进展和视力恶化的发生率更高。该组患者对化疗的反应可能更好，应在疾病过程中尽早考虑使用化疗。
