Optic Nerve Sheath Meningioma Research Articles

Recovery of visual field in early treated optic nerve sheath meningioma: A Case Report

Recovery of visual field in early treated optic nerve sheath meningioma: A Case Report

Results of radiation therapy for optic nerve sheath meningioma

To assess the neuro-ophthalmic symptomatology of ONSM and how it changes in response to radiotherapy. In the period from 2004 to 2018, 112 patients with ONSM aged 17 to 76 years underwent stereotactic radiotherapy. In 91 patients, visual functions varied from light perception to 1.0; in 21 patients the affected eye was blind. The prospective follow-up of 103 patients lasted 6 months to 10 years (mean follow-up duration was 57 months). Studied patients underwent either conventional fractionated radiotherapy with Novalis (n=88) or hypofractionation radiotherapy with CyberKnife (n=24). According to ophthalmological examination, in 60.5% of cases an improvement of visual functions was observed, 39.5% of patients had visual functions remain stable at the initial level. No vision impairment during irradiation was detected. Vision deterioration in the long-term follow-up was observed in 2 patients: extended tumor growth in one case and radio-induced retinopathy in the other. The study showed that this method of treatment for ONSM is effective and safe, allowing preservation of visual functions and in some cases - its improvement, while tumor growth remains under control.

Radiotherapy for the treatment of optic nerve sheath meningioma: A systematic review and meta-analysis

ObjectivesThe management of Optic Nerve Sheath Meningiomas (ONSM) has suffered a significant shift due to new radiation techniques. However, there is no conclusive information on which approach presents better results in the literature. This meta-analysis aims to evaluate the outcomes of different radiotherapy (RT) modalities in the management of ONSM. MethodsA systematic review based on an electronic search was performed in MEDLINE, EMBASE, Cochrane, and Lilacs databases. Eligible studies included patients with ONSM treated with RT. Two reviewers independently assessed the eligibility of potential studies, extracted data, and performed the meta-analysis. Outcomes of interest were tumor control, visual acuity (VA), visual field, and complications. ResultsThirty-nine non-comparative studies involving 736 eyes with ONSM treated with RT were included. Six different techniques were studied: 2-dimensional RT (2DRT), 3D-conformal RT (3CRT), Stereotactic Fractionation RT (SFRT), stereotactic radiosurgery (SRS), intensity-modulated RT (IMRT), and proton beam therapy (PBT). With a mean follow-up period of 46 months, tumor control was 97,4% (95% CI: 98–100%). No difference was observed regarding the rate of final VA post-treatment among the modalities. The 3CRT showed substantially higher rates of complication than other techniques. ConclusionsRT produces remarkable tumor control. New radiation modalities such as SFRT, IMRT, SRS, and PBT resulted in better therapeutic results with fewer complications than 2DRT and 3CRT. Thus, they should be the preferential choices for treating ONSM regardless of initial clinical presentation.

Characteristics of peripapillary retinal nerve fiber layer atrophy in glaucoma, optic nerve sheath meningioma, and sphenoid wing meningioma

Background/objectivesThe correct classification of a slowly progressing optic atrophy can be challenging. The aim of this work was to find out if the characteristics of peripapillary retinal nerve fiber layer (RNFL) thickness loss differ between open angle glaucoma (POAG), optic nerve sheath meningioma (ONSM), and sphenoid wing meningioma (SWM).MethodsA total of 45 patients with POAG, ONSM, and SWM were included in the retrospective study. The peripapillary RNFL thickness measured by spectral-domain optical coherence tomography was analyzed using the Heidelberg Engineering glaucoma module©.ResultsEach group consisted of 15 patients. The temporal sector of the RNFL thickness showed a median decrease of − 17 µm in glaucoma patients (range + 6/–34 µm), − 43 µm in ONSM (range − 19/ − 52 µm), and − 44 µm in SWM patients (range − 25/ − 52 µm). The RNFL thickness of the temporal sector of glaucoma patients differed significantly from the other groups (p < 0.001). All other sectors showed no significant difference between the 3 groups.ConclusionThe peripapillary RNFL thickness of the temporal sector of patients with beginning to moderate POAG is usually inside normal limits or borderline. In contrast, patients with ONSM and SWM are much more likely to show a considerable reduction in RNFL thickness of the temporal sector. RNFL thickness of the temporal sector marked outside normal limits occurred exclusively in meningioma patients. Considering the presence of this condition as a predictor for meningioma, sensitivity and specificity were 0.8 and 1.0, respectively. In patients with significant reduction in RNFL thickness of the temporal sector, magnetic resonance imaging of the head should be considered to rule out compression of the optic nerves.

PO-1055 Fractionated stereotactic radiotherapy in optic nerve meningioma

PO-1055 Fractionated stereotactic radiotherapy in optic nerve meningioma

68Ga-DOTATOC-PET/MRI-A Secure One-Stop Shop Imaging Tool for Robotic Radiosurgery Treatment Planning in Patients with Optic Nerve Sheath Meningioma.

Simple SummaryThrough combined information about spatial spread in magnetic resonance imaging (MRI) and positron emission tomography (PET), gallium-68-labeled (DOTA0-Phe1-Tyr3) octreotide (Ga68-DOTATOC) PET/MRI before radiosurgery allows safe treatment planning for optic nerve sheath meningiomas (ONSM). Moreover, because of specific receptor stocking, diagnostic accuracy is improved without the need for biopsy. Thus, Ga68-DOTATOC-PET/MRI can be considered a safe “one-stop shop” image tool prior to radiosurgery for ONSM.Optic nerve sheath meningiomas (ONSM) are rare but can lead to irreversible blindness. Hybrid imaging may enhance tumor delineation and diagnostic accuracy via receptor binding. However, relevant clinical data for ONSM are lacking. We evaluated the feasibility of receptor-based hybrid imaging prior to robotic radiosurgery (RRS). We retrospectively analyzed all of our institution’s patients with suspected ONSM who underwent combined positron emission tomography and magnetic resonance imaging (PET/MRI) with gallium-68-labeled (DOTA0-Phe1-Tyr3) octreotide (Ga68-DOTATOC) before RRS between 2018 and 2019. Eight patients with ten suspected ONSM (female = 7; median age, 51.2 years; IQR, 43.0–66.0) were included. Nine out of ten ONSM were deemed PET-positive with a median standard uptake value (SUV) max of 5.6 (IQR, 2.6–7.8). For all nine ONSM that presented 68Ga-DOTATOC uptake, hybrid PET/MRI was used for target volume contouring prior to RSS. At a median follow-up of 11.7 months (IQR, 9.4–16.4), tumor control was achieved in all patients. Radiosurgery resulted in the improvement of visual acuity in two of eight patients, whereas six showed stable vision. Ga68-DOTATOC-PET/MRI can be used for target volume contouring prior to RRS for ONSM as it enables safe treatment planning and improves diagnostic accuracy.

Effectiveness and Safety of Robotic Radiosurgery for Optic Nerve Sheath Meningiomas: A Single Institution Series.

Simple SummaryOptic nerve sheath meningiomas (ONSM) are a rare subtype of meningioma. Only four retrospective studies with 3–21 patients have been published on the treatment of ONSM by radiosurgery. This study represents the largest published series on robotic radiosurgery to date, treating 25 patients with 27 ONSM lesions. Furthermore, hypofractionated radiosurgical treatment proves to be a safe alternative to surgery and fractionated stereotactic radiation with an overall local tumor control rate of 96.0% and stable or improved visual acuity in 90.0% and 10.0% of patients, respectively. We believe that our study makes a significant contribution to the literature, as our results indicate that robotic radiosurgery is a safe and effective treatment for the management of ONSM and offers a potential treatment option that would improve patient care and clinical outcomes.The role of robotic radiosurgery (RRS) in the treatment of optic nerve sheath meningiomas (ONSM) remains controversial and it is only performed in specialized institutions due to tight dose constraints. We evaluated the effectiveness and safety of RRS in the management of ONSM. Twenty-five patients with 27 ONSM lesions who underwent RRS using the Cyberknife (CK) system were retrospectively analyzed (median age, 47.9 years; 84.0% women). Multisession RRS was used with 4–5 fractions with a cumulative dose of 20.0–25.0 Gy in 84.0% of patients and a single fraction at a dose of 14.0–15.0 Gy in 16% of patients. Prior to RRS, seven (28%) patients experienced blindness on the lesion side. In those patients with preserved vision prior to radiosurgery, the visual acuity remained the same in 90.0% and improved in 10.0% of the patients. Overall local tumor control was 96.0% (mean follow-up period; 37.4 ± 27.2 months). Neither patient age, previous surgery, or the period from the initial diagnosis to RRS showed a dependency on visual acuity before or after radiosurgery. RRS is a safe and effective treatment for the management of ONSM. Hypofractionation of radiosurgery in patients with preserved vision before CK treatment results in stable or improved vision.

Anticancer activity of bergenin against cervical cancer cells involves apoptosis, cell cycle arrest, inhibition of cell migration and the STAT3 signalling pathway.

[This retracts the article DOI: 10.3892/etm.2019.7380.].

Primary and Secondary Optic Nerve Sheath Meningioma.

Objective This study was aimed to review issues relating to the recognition, radiographic diagnosis, monitoring, and management of primary and secondary optic nerve sheath meningioma (ONSM). Design This study is a review of peer-reviewed literature combined with illustrative case studies. Participants and Methods A literature search was conducted via the PubMed database using pertinent search terms. Selected articles were limited to those written or translated into English. Additional works cited within articles were also included. Individual cases were drawn from the experience of a tertiary academic neuroophthalmic and orbital practice. Tables summarize radiotherapeutic and surgical studies, excluding single case reports and studies focusing on meningioma of intracranial origin. Main Outcome Measurements Review of reported surgical and radiotherapeutic series is the primary measurement. Results The natural history of optic nerve sheath meningiomas is primarily characterized by progressive ipsilateral vision loss. Diagnosis is typically based on radiographic imaging findings, with biopsy remaining indicated in some patients. Management strategies may include observation, radiation, and/or surgical intervention, or a combination of these approaches. The role of surgery, especially with respect to primary ONSM (pONSM), remains controversial. Advancement of radiotherapy techniques has shifted modern treatment paradigms in pONSM toward radiation as primary treatment, as surgical outcomes are inferior in major studies. Although radiation remains the treatment of choice in many cases, selected patients may benefit from surgery, especially in the setting of secondary ONSM (sONSM). Conclusion A wide variety of radiotherapeutic and surgical treatment modalities for ONSM exist. The specific indications for each management strategy continue to be redefined.

Prognostic Factors for Functional Outcome of Patients with Optic Nerve Sheath Meningiomas Treated with Stereotactic Radiotherapy-Evaluation of Own and Meta-Analysis of Published Data.

Simple SummaryThis article highlights the fractionated stereotactic radiotherapy in the treatment of optic nerve sheath meningioma (ONSM). ONSM, a slow-growing tumor next to the optic nerve, causes over a long period partial or complete blindness. As visual impairments appear and progress slowly in the disease history, the need for treatment and treatment starting time are controversial. We restate in our article that fractionated stereotactic radiotherapy improves visual function. We further search for prognostic factors which might provide valuable additional and independent information on when to start treatment. We reveal that the visual acuity is a prognostic factor for the outcome of stereotactic therapy for ONSM and promote it as a biomarker for the decision for treatment initiation.Objective: To evaluate prognostic factors for a favorable outcome (improvement of the visual acuity or visual fields) after fractionated stereotactic radiotherapy (fSRT) of optic nerve sheath meningioma (ONSM). Methods: We performed a database search for ONSM treatments during the period from April 2008 to September 2019 in the prospective database for stereotactic radiosurgery/radiotherapy (SRS/SRT) of the Robert Janker Clinic Bonn (Department of Radiotherapy) and performed a literature review and meta-analysis of published data on ONSM between 2010 and 2019. Ophthalmic status before and after treatment was evaluated and the collective was dichotomized into two groups: functional improvement (FI; improvement of either visual acuity or visual fields) and non functional improvement (NFI; with stable or deteriorating visual acuity or visual fields). The two groups were compared regarding different variables: pretreatment visual acuity, age, gender, gross tumor volume (GTV), follow up (FU) time, tumor localization, and maximal retina dose. Results: Overall, 13 stereotactic radiotherapies were performed for ONSM (12 × fSRT, 1 × SRS). Mean follow up was 3 years (range: 1–5 years). The total dose was 50.4 Gy (5 × 1.8 Gy/week) in 12 patients treated with fSRT and 1 × 14 Gy in one SRS case. Mean GTV was 1.13 ccm (range: 0.44–2.20 ccm). During follow up, all tumors were stable or showed shrinkage of tumor volume (100% tumor control), no adverse events were observed, 53% of the patients achieved either better visual acuity or visual fields. Pretreatment visual acuity was significantly different between the FI and the NFI group (0.17 vs. 0.63, p = 0.03) in our series and in the meta analysis (p < 0.01). Moreover, shorter FU time and lower retinal dose were significantly linked (p < 0.05 and p < 0.01, respectively) with a better outcome in the meta-analysis but not in our patient cohort. Intracranial tumor localization, gender, and age were not significantly different between the two outcome groups. Conclusion: FSRT for ONSM achieves in over 50% of cases an improvement of the ophthalmic status with low morbidity and excellent tumor control in our series and the meta analysis. Patients with a favorable outcome had in all analysis a significantly higher visual acuity before treatment start. Therefore, we advocate using fSRT as early as possible before vision deterioration occurs.

Normative Orbital Measurement Based on Computed Tomography Imaging in Tertiary Care Hospital, Bangalore, Karnataka

Introduction: The orbit is an anatomically complex region. Clear idea of the normal orbital measurements is needed for the diagnosis and management of the various diseases that may affect the orbit like optic neuritis, optic nerve glioma, meningioma, proptosis and exophthalmos. Aim: To evaluate normative orbital measurement at tertiary care hospital in Bangalore, Karnataka in Indian population based on Non Enhanced Computed Tomography (NECT) imaging. Materials and Methods: A cross-sectional observational study of 100 patients referred to a tertiary care centre for NECT of the brain for diseases other than those of the orbits was conducted. Patients having any orbital disorder, surgery or trauma and having metallic implants were excluded from the study. The patients of all age groups and either sex were included. The horizontal orbital diameter, vertical orbital diameter, orbital index, optic nerve complex, globe position, interorbital diameter and interzygomatic line measurements were calculated for 100 patients, i.e., total of 200 orbits using both axial and direct coronal CT images. Mean measurements of these parameters were calculated. Scans were performed on Siemens Somatom Perspective 128 slice multi- detector CT scanner. All statistical analysis was performed using Statistical Package for The Social Sciences (SPSS) for windows software (SPSS Inc. Illinois, Chicago, USA). Results: The mean orbital index was 118.6 and 118.3 for right and left orbit, respectively. It was derived from mean horizontal orbital diameter of 32.5 mm and 31.7 mm of right and left orbit, respectively and mean vertical orbital diameter of 38.4 mm and 37.4 mm of right and left orbit, respectively. Mean optic nerve sheath complex diameter was 5.2 mm and 5.4 mm for right and left orbit, respectively, while mean globe position measurements 6.7 mm and 6.8 mm of right and left orbit, respectively. Average interorbital distance was 22.9 mm and average interzygomatic distance 97.1 mm. Conclusion: The present study has given normative mean measurements for various orbital structures that are essential to ophthalmologists and radiologists to differentiate normal from abnormal dimensions which is likely to be helpful in accurate assessment of various orbital pathologies.

Optic nerve sheath carcinomatosis masquerading as optic perineuritis: The first sign of gastric cancer relapse

Optic perineuritis (OPN) is a rare inflammatory disease involving the optic nerve sheath. Most cases are idiopathic, but associations with a variety of infectious and inflammatory conditions have been described. Patients with suspected OPN should also be investigated for neoplasms in the form of optic nerve sheath meningioma or meningeal carcinomatosis, given they share important clinical and radiological features. We present a case of suspected idiopathic bilateral OPN that was particularly challenging, eventually revealing a neoplastic etiology.

Unilateral Isolated Paucisymptomatic Optic Disc Edema.

Unilateral isolated optic disc edema (UIODE) represents a challenging clinical presentation that frequently precipitates an extensive diagnostic work-up. Patients without an apparent diagnosis despite appropriate investigations are often categorized as having "papillophlebitis," an entity that is poorly defined in the existing literature. Our aim was to describe the characteristics of a series of patients with paucisymptomatic UIODE, determine the optimal diagnostic approach to such cases, and clarify the clinical features of presumed papillophlebitis. We retrospectively identified 29 patients with UIODE who were seen by neuro-ophthalmologists at a single center between 2005 and 2019. Each patient presented with isolated, unilateral disc edema that was either entirely asymptomatic or associated with minimal visual symptoms. Patients underwent a comprehensive neuro-ophthalmic evaluation and several ophthalmic and systemic investigations. Data from the initial visit and all subsequent clinical visits were collected, including patient demographics, examination findings, and details of the diagnostic work-up. Our 29 patients with UIODE were found to have a variety of underlying diagnoses including unilateral papilledema due to idiopathic intracranial hypertension (10 patients), optic nerve sheath meningioma (5), incipient nonarteritic anterior ischemic neuropathy (4), vitreopapillary traction (3), orbital masses (2), a peripapillary choroidal neovascular membrane (1), and presumed papillophlebitis (4). The duration of disc edema varied considerably based on the etiology, but most patients had favorable visual outcomes. A systematic approach to the evaluation of UIODE, combined with long-term follow-up, led to a definite diagnosis in a majority of patients, with only 4 patients presumed to have papillophlebitis, a diagnosis the actual existence of which remains controversial.

Intracranial And Intraorbital Rosai Dorfman Disease

Rosai Dorfman disease is a rare histiocytic disorder. It is also known as Sinus Histiocytosis. It is with massive lymphadenopathy involves an overproduction of a type of white blood cell. The disease is rarely associated with intracranial and intraorbital involvement. Intracranial Rosai-Dorfman can mimic meningioma. Other pathologies also underline its pathologies. Here, we report a nine-year-old boy with a history of proptosis of the right eye and presenting with multiple skull lesions. Histopathological study revealed Sphenopetroclival lesion, which features that of Rosai Dorfman Disease. His MRI scan of the brain was taken, which showed evidence of right optic nerve meningioma with sella and suprasellar extension, causing severe proptosis. The child underwent right frontotemporal craniotomy with petrosectomy and Transylvanian, subtemporal approach to multicompartmental Rosai-Dorfmans lesion. After four months, the patient had a recurrence of the disease on which chemotherapy and steroids were started, which also did not show much response while taking an MRI scan. A corticosteroid is a useful option in the Central Nervous System Rosai Dorfman disease treatment. But this patient showed a negative outcome to the treatment.

Optic nerve sheath meningioma.

Optic nerve sheath meningiomas (ONSMs) are rare benign tumors of the anterior visual pathway which present with slowly progressive and painless vision loss and account for approximately 2% of all orbital tumors. This article provides an overview as well as an update on the ONSMs with regards to cause, epidemiology, clinical presentation, diagnosis, and management in adults and pediatric population. The clinical presentation and prognosis of ONSMs can vary and largely depend on the location of tumor as well as the histologic type. Overall, the diagnosis is based on clinical presentation, examination, and neuroimaging findings. Nevertheless, delays in diagnosis or misdiagnosis are not uncommon and can result in higher morbidity rates. Recent advances in diagnostic as well as more effective and less-invasive treatment options are discussed in this review. ONSMs are a rare cause of slowly progressive and inexorable visual loss. Although ONSM diagnosis depends on the characteristic clinical and radiologic findings, prompt diagnosis, and appropriate management is critical for favorable visual outcomes. Thus, current focus is optimizing diagnostic as well-treatment methods for patients with ONSMs.

The Role of Neuro-Ophthalmologists in the Care of Patients With Neurofibromatosis Type 2.

Neurofibromatosis type 2 (NF2) is a rare genetic disorder, affecting the central nervous system and leading to various degrees of disability. Its hallmark is bilateral vestibular schwannomas that invariably lead to progressive hearing loss. Specific ophthalmic abnormalities in patients with NF2 may help to establish an early diagnosis. These include juvenile cataract, epiretinal membrane, combined hamartoma of the retina and the retinal pigment epithelium, optic disc glioma, and optic nerve sheath meningioma. In addition, intracranial tumors may produce a variety of neuro-ophthalmic abnormalities that have the potential to impair visual function, such as postpapilledema optic atrophy, compression of the visual pathways, keratopathy, ocular motor cranial nerve palsies, and amblyopia. Care of NF2 patients is best provided by interdisciplinary medical teams including a neuro-ophthalmologist.

Noncoplanar volumetric modulated arc therapy for patient with optic nerve sheath meningioma: a case report and literature review

Noncoplanar volumetric modulated arc therapy for patient with optic nerve sheath meningioma: a case report and literature review

Large Orbital Tumor in a Patient with Neurofibromatosis Type 2

Neurofibromatosis type 2 (NF2) is a separate entity from Neurofibromatosis type 1 (NF1) or von Recklinghausen’s disease, and is much less frequent than NF1. Vestibular schwannomas are the hallmark lesion, affecting 95% of individuals and typically occur bilaterally. Schwannomas commonly occur on other nerves intracranially and in the spinal compartment, along with meningiomas, ependymomas, and gliomas. Ophthalmologic abnormalities are present in the majority of NF2 patients, including cataracts, retinal changes, optic nerve sheath meningiomas and other optic pathway tumors. These tumors are sometimes large and can induce significant proptosis. We report the case of a large orbital meningioma responsible for severe proptosis in a patient with NF2.

Optimal management in optic nerve sheath meningioma – A multicentre study and pooled data analysis

Optic nerve sheath meningiomas (ONSMs) are rare, benign neoplasms of the anterior visual pathway. The optimal modality of radiotherapy for treatment has not yet been established. This is the first study to show significant difference in visual outcomes between radiotherapy subtypes in the management of ONSM. We performed a retrospective analysis of visual outcomes and side effects in ONSM patients treated with radiotherapy at three centres in Sydney, Australia, between 2000 and 2016. 15 patients with ONSM were included, and visual outcomes (visual acuity, visual fields, colour vision, OCT retinal nerve fibre layer thickness and radiological tumour response) and treatment toxicities were assessed. Pooled data analysis of available studies was also performed. Statistical analysis was performed with binomial, two-tailed chi-squared tests and Fisher exact tests. In our cohort a significant majority experienced improved visual field (p = 0.046), stable or improved visual acuity (p = 0.0017) and colour vision (p = 0.015) after fractionated radiotherapy. Pooled analysis with strict inclusion criteria found 3D conformal radiotherapy to offer significantly poorer visual acuity compared to fractionated stereotactic radiotherapy (p = 0.008). When all published studies were included, stereotactic radiosurgery was superior to 3D conformal methods (p = 0.035), and equivalent to other fractionated methods. 3D conformal methods also had significantly higher rates of long-term side effects. These results support the use of fractionated radiotherapy and radiosurgery for ONSM treatment, however 3D conformal methods cannot be recommended.

Results of Neuroimaging in Patients with Atypical Normal-Tension Glaucoma.

Aim The aim of the study was to determine the frequency of pathologies which can mimic normal-tension glaucoma (NTG), observed in neuroimaging of NTG patients, and to evaluate the frequency of pathologies in determined additional indications for neuroimaging. Material and Methods. The studied group consisted of 126 NTG patients who met at least one of the following criteria: unilateral NTG, damage in the visual field (VF) inconsistent with optic disc appearance, fast VF progression, worsening of visual acuity, predominant optic disc pallor rather than optic disc excavation, diagnosis under the age of 50, and scotoma in VF restricted by a vertical line. The patients included in the research underwent MRI scans of the brain and both orbits. Results After neuroimaging, the results of 29 (23%) patients were qualified as positive; 18 (14.2%) of the identified pathologies were found to clinically affect the visual pathway. The most frequent brain pathology was intracranial meningiomas, observed in 4 patients (3.1%), followed by optic nerve sheath meningiomas diagnosed in 3 cases (2.4%), and brain glioma in 1 patient (0.8%). Pituitary gland adenomas were described in 6 patients (4.5%); 3 of the tumours were in contact with the optic chiasm. 53 (40%) patients had minimal ischemic changes in different regions of the brain. In the case of worsening BCVA or fast VF progression, the frequency of positive results was the highest (50% and 40%), whereas in the case of diagnosis at a young age and unilateral involvement, neuropathology was the rarest (0% and 6.9%). Conclusions In the case of NTG, the decision to perform neuroimaging should be made after a detailed assessment of clinical status, rather in the event of finding the signs of possible compressive optic neuropathy than as an obligatory procedure for every patient.