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AimsTo report visual acuity (VA) outcomes following chemotherapy for optic pathway glioma (OPG) in children with or without neurofibromatosis type-1 (NF1) and to analyse associated risk factors.MethodsA prospective, multicentre, cohort study involving 155 children treated between September 2004 and December 2012. Initial and final VA was used for per-eye and per-subject analysis. Correlation tests were performed to determine whether initial VA predicted final VA. Logistic regression was used to determine whether age and tumour location were associated risk factors.Results90 children had complete ophthalmological data. At initiation of chemotherapy, 26% and 49% of eyes with NF1-OPG and sporadic OPG, respectively, had VA of ≥0.7 log of the minimum angle of resolution (logMAR). At final visit, per eye, 49% had ≤0.2, 23% had 0.30–0.60 and 28% had VA≥0.70 logMAR in the NF1-OPG group. In the sporadic OPG group, per eye, 32% had ≤0.2, 11% had VA 0.30–0.60 and 57% had ≥0.70 logMAR. Children with sporadic OPG, per eye, were significantly less likely to have VA outcomes ≤0.60 logMAR compared with children with NF1-OPG (OR=0.30; 95% CI 0.16 to 0.56; P<0.0001). Per subject, VA improved in 24%, remained stable in 35% and worsened in 41% of children with NF1-OPG and improved in 18%, remained stable in 43% and worsened in 39% of children with sporadic OPG.ConclusionsChildren with and without NF1 demonstrated the same rate of VA improvement, stabilisation or worsening; however, children with sporadic OPG had a poorer VA outcome. Better initial VA, older age, absence of postchiasm tumour and presence of NF1 were associated with improved or stable VA outcomes.

Purpose To investigate the timing of enucleation, treatment course, and outcome for retinoblastoma (RB) with optic nerve (ON) invasion on imaging. Study design Retrospective clinical study. Methods Of the 160 patients with RB who presented to the National Center for Child Health and Development in Japan between 2005 and 2022, ON invasion on imaging at the initial presentation was seen in five patients. The clinical, computed tomography (CT), and magnetic resonance imaging (MRI) findings, and treatment courses were reviewed retrospectively. Results MRI showed ON invasion in all five patients (three with unilateral RB, 2 with bilateral RB); in two patients CT detected no invasion. Enucleation was performed in four patients, three of whom underwent neoadjuvant therapy and one had a positive ON resection margin following the enucleation as initial treatment. One patient did not undergo enucleation due to cerebrospinal fluid dissemination. All enucleated patients underwent adjuvant chemotherapy. Four patients underwent radiotherapy. During follow-up (mean, 89.4 months), four patients survived and one died. Conclusion MRI is recommended to evaluate ON invasion and determine the timing of enucleation for RB. The appropriate choice of neoadjuvant or adjuvant therapy would be helpful to avoid radiotherapy for RB with ON invasion on imaging.

Purpose To survey the real-world effectiveness and cost of optic nerve sheath meningioma (ONSM) treating with Gamma Knife Radiosurgery (GKRS), and compare with the external beam radiation therapy (EBRT). Methods Retrospective, comparative study that included patients with primary ONSM treated with either GKRS or EBRT in Samsung Medical Center, Korea. The treatment response, and treatment costs were compared between GKRS and EBRT groups. Results There were 34 adult patients with primary ONSM treated with either GKRS ( n  = 25) or EBRT ( n  = 9) (follow-up period: 6–207 months). The local tumor control rates (GKRS: 92%; EBRT: 100%; P  = 1) and vision preservation rates (GKRS: 64%; EBRT: 67%; P  = 1) were similar in both groups. The mean gross tumor volume (GTV) decreased by 21.4 ± 19.7% after GKRS and 26.4 ± 18.7% after EBRT ( P  = 0.4803). The complication rates did not differ between two modalities. Factors associated with better visual outcomes were pretreatment BCVA > 20/50 (odds ratio: 6.000, P  = 0.0234) and the absence of intracranial tumor extension (odds ratio: 30.00, P  = 0.0001). GKRS reduced the total costs of care by 43% under Korean National Health Insurance System (NHIS). Conclusion This study revealed that GKRS and EBRT had similar treatment efficacy and safety profile in treating ONSMs. Instant management is advantageous when the BCVA deteriorates to 20/50 and tumors involve intracranially. Under the framework of Korean NHIS, GKRS contributed to less indirect cost estimates and was preferred by patients under shared decision-making process.

Orbital tumours include a variety of orbital diseases of different origins. In the case of malignant orbital tumours, early detection is important so that treatment can be initiated promptly. Neuroradiological imaging, in particular magnetic resonance imaging (MRI), plays an important role in the diagnostic of orbital tumours. In adults, lymphoproliferative diseases, inflammations and secondary orbital tumours are most frequently found, whereas in children mostly dermoid cysts, optic gliomas and capillary haemangiomas are found. Optic glioma is a pilocytic astrocytoma and accounts for two thirds of all primary optic tumours. Optic nerve sheath meningiomas mostly affect middle-aged women. In childhood, retinoblastoma is the most common intraocular tumour. This is an aggressive malignant tumour which can occur unilaterally or bilaterally. Based on the imaging findings, differential diagnoses can usually be easily narrowed down using criteria such as age of manifestation, frequency, localisation and imaging characteristics.

Treatment of childhood low-grade gliomas is a challenging issue owing to their low incidence and the lack of consensus about \"optimal\" treatment approach. Reports in the literature spanning 60 years of radiation therapy, including orthovoltage, megavoltage and recently modern high-precision treatments, were reviewed with respect to visual function, survival, prognostic factors, dose prescriptions, target volumes, and treatment techniques. Based on these experiences, future strategies in the management of childhood low-grade glioma are presented. Evaluation of published reports is difficult because of inconsistencies in data presentation, relatively short follow-up in some series and failure to present findings and results in a comparable way. Even with the shortcomings of the reports available in the literature, primarily concerning indications, age at treatment, dose response, timing and use of \"optimal\" treatment fields, radiation therapy continues to play an important role in the management of these tumors achieving long-term survival rates up to 80% or more. Particularly in gliomas of the visual pathway, high local tumor control and improved or stable visual function is achieved in approximately 90% of cases. Data on dose-response relationships recommend dose prescriptions between 45 and 54 Gy with standard fractionation. There is consensus now to employ radiation therapy in older children in case of progressive disease only, regardless of tumor location and histologic subtype. In younger children, the role of radiotherapy is unclear. Recent advances in treatment techniques, such as 3-D treatment planning and various \"high-precision\" treatments achieved promising initial outcome, however with limited patient numbers and short follow-ups. Radiation therapy is an effective treatment modality in children with low-grade glioma regarding tumor control and improvement and/or preservation of neurologic function or vision, respectively. More prospective studies are needed to address the impact of modern radiation therapy technologies (including intensity-modulated radiotherapy) on outcome especially in the very young and to define the role of radiation therapy as a part of a comprehensive treatment approach. The forthcoming prospective trial SIOP/GPOH LGG RT 2003 is addressing this issue.

PurposeThe present study examined the efficacy and complications associated with intensity-modulated radiation therapy (IMRT) for optic nerve sheath meningioma (ONSM) in 15 cases and compared visual function before and after treatment.MethodsConsecutively diagnosed patients with ONSM treated with IMRT were evaluated from 2012 to 2017. We categorized ONSM with three growth patterns (diffuse, fusiform, or globular). Visual acuity, visual fields, and optic disc findings were assessed before and after IMRT. Ocular and systemic complications were evaluated during and after treatment.ResultsThe 15 patients selected for analysis ranged in age from 33 to 77 years. Post-treatment observation periods were 8 to 57 months. After IMRT, tumor enlargement was not detected in any eyes, and tumor reduction was seen in 2 eyes. At final post-treatment follow-up, eyes with fusiform and globular growth maintained better visual acuity compared with pre-treatment, whereas 2 of 5 eyes with diffuse growth showed reduced vision. Five eyes with no apparent optic disc abnormality maintained better visual acuity compared with pre-treatment, whereas 8 of 10 eyes with disc edema and atrophy remained stable or showed reduced vision. Improvements were seen in all 5 eyes with optic discs negative for pre-treatment abnormalities. Final post-treatment visual field abnormalities improved in 11 eyes. All adverse events identified during IMRT improved rapidly during the treatment period.ConclusionIMRT for the treatment of ONSM achieved improvement and preserved visual function. In particular, early treatment with IMRT before the appearance of optic disc abnormalities can be more effective for improving visual function.

Optic disc capillary hemangiomas, a rare capillary hamartoma that often invades optic nerve fibers and disc plaque bundles, have been a therapeutic challenge for ophthalmologists in recent years due to their unique location. A woman in her early 50s presented with blurred vision in the right eye for 2 days. On ocular examination, her best-corrected visual acuity was 20/340 in the right eye. Wide-field fundus imaging revealed a red mass on the temporal side of the optic disc. Optical coherence tomography revealed macular edema. The diagnosis was optic disc capillary hemangioma. Subsequently, 0.5 mg ranibizumab was intravitreally injected into the right eye once monthly, for a total of two injections. The patient was re-examined at 15 days, 50 days, and 21 months after the first operation. The patient’s postoperative best-corrected visual acuity improved to 20/60, and macular edema and exudation were reduced in the short term compared with those in the previous period; however, the tumor did not change significantly. The anti-vascular endothelial growth factor agent ranibizumab is effective in treating optic disc capillary hemangiomas.

To examine the incidence, presentation, and outcome of optic gliomas in children with neurofibromatosis type 1 (NF1) in Southern California Kaiser Permanente. The authors queried the Southern California Kaiser Permanente electronic medical record database to find patients diagnosed as having NF1. Genetics, ophthalmology, and imaging medical records of patients with optic glioma were reviewed. A total of 708 patients younger than 21 years had a diagnosis of NF1 in Southern California Kaiser Permanente and 30 (4.2%) had a diagnosis of optic glioma. The average age of diagnosis was 5 years, with a range of 18 months to 12 years. Half (15 of 30) of the patients diagnosed as having optic glioma presented with symptoms (eg, vision loss, proptosis, and precocious puberty). Eight of 15 of the symptomatic patients were treated with surgery and/or chemotherapy. Symptomatic children were diagnosed later than those diagnosed through routine screening (5.7 vs 3.9 years old). The oldest child presented with symptoms at age 12 years. One asymptomatic patient had prophylactic chemotherapy. Sixty-three percent (19 of 30) of the gliomas were bilateral, 23% (7 of 30) were right-sided, and 13% (4 of 30) were left-sided. Fifty-three percent (17 of 30) of the gliomas involved the optic chiasm. Screening practices for optic glioma are inconsistent. Most children with NF1 at risk for optic glioma do not have even one visit with an ophthalmologist. Children with NF1 can develop asymptomatic optic glioma as early as age 1 year. Annual ophthalmologic examination and screening for precocious puberty in children with NF1 is important for early diagnosis of optic gliomas and may reduce morbidity. [J Pediatr Ophthalmol Strabismus. 2016;53(6):334-338.].

Abstract BACKGROUND: Optic pathway gliomas (OPGs) are considered relatively benign pediatric tumors. Adult patients with OPG can be divided into 2 groups: adult patients with tumors diagnosed in childhood and adult patients diagnosed during adulthood. OBJECTIVE: To characterize the clinical course of adult patients with OPG. METHODS: We retrospectively collected clinical and imaging data of all adult OPG patients monitored in our medical center between 1990 and 2012. RESULTS: Twenty-two adult patients were included. Age at diagnosis varied widely (6 months-66 years), as did age at last follow-up (18-74 years). Ten patients were diagnosed at adulthood and 12 in childhood. Of the patients diagnosed at childhood, 6 had radiological progression during childhood, and 3 of those patients suffered visual impairment. From this group, 1 patient had further progression during adulthood accompanied by additional visual decline, and 2 patients had additional visual decline during adulthood despite no signs of progression. Of the 6 patients whose tumors were stable during childhood, all 6 remained stable during adulthood. Of 10 patients diagnosed at adulthood, 6 patients suffered visual deterioration; in 5 of them, a concomitant progression was noted. Two patients were diagnosed with high-grade gliomas. CONCLUSION: OPGs may be active during childhood or adulthood. Those patients who experienced anatomic activity during childhood are prone to continue experiencing active disease during adulthood. A significant percentage of patients diagnosed with low-grade OPG at adulthood may suffer progression, visual decline, or both.

[...]in recommending radiation therapy to a patient with an ONSM, one must consider the potential side effects that could damage the vision that one is attempting to preserve or improve, and any method that could prevent or reduce the frequency and severity of these side effects would be welcome.