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Purpose: The optic nerve sheath meningioma (ONSM) is one of the most challenging tumors in orbital surgery. From the perspective of mental health and patient needs, we analyzed the necessity and importance of the endoscopic transnasal approach (ETA) combined with optic nerve transection (ONT) in gross-total resection (GTR) in ONSM patients with residual vision and aim to broaden the use of ONT for specific people., Methods: The authors included patients with ONSMs who were treated between 2014 and 2022. We divided those cases into two groups named ETA group and lateral orbitotomy approach (LOA) group. We present the application of ETA and analyze the preoperative indication of the ONT and compared the advantages and disadvantages between ETA and LOA. The degree of tumor resection was based on imaging and surgical evaluation., Results: A total of 23 patients with ONSM were included. Sixteen patients underwent ETA, and seven underwent LOA. Among ETA cases, GTR was achieved in 14 patients with ONT and most patients maintained normal eye movement function (75%) and morphology (93.75%). In the ETA group, 14 patients experienced vision loss, while two other patients saw improvements in vision. And proptosis was alleviated (5.20 ± 2.34 vs 0.27 ± 0.46, p < 0.0001). Six patients with blindness and proptosis of the LOA group resulted in GTR with ONT and ophthalmectomy. Although intracranial extension and recurrence included no cases in the two groups, a significant psychological gap was presented due to cosmetic problems., Conclusions: Under the premise of reducing damage and improving aesthetics, the selection of ETA combined with ONT to gross-total resect ONSMs successfully provides a minimally invasive access with acceptable complications. As an important adjunct to GTR in the surgical treatment of ONSM, the scope of ONT application should be expanded to relieve the patient's psychological burden., (© 2024. The Author(s).)

Background: Management of patients with optic nerve sheath meningiomas (ONSMs) is controversial and the treatment strategy in this patient group is still up for discussion. Transnasal endoscopic orbital and optic nerve decompression aims to reduce the pressure in the orbit and on the optic nerve and thereby prevent vision loss. This article presents material from 7 cases of transnasal endoscopic orbital decompression., Methods: The study design is a retrospective cohort study. The aim was to include all patients with a meningioma residing along the nerve sheath and who were operated using endoscopic transnasal decompression of the orbit and if needed the optic canal at Odense University Hospital. Data from the medical records were collected and pre- and postoperative eye examinations were compared. In addition, it was recorded whether there were complications to the procedure and whether additional treatments were given., Results: In total, 4 women and 3 men were included in the study. Four out of 7 patients experienced improvement in vision after the operation. One patient experienced unchanged vision and 2 patients experienced deterioration of vision after surgery., Conclusions: The current report of 7 patients with ONSM shows promising results for this surgical procedure as 4 out of 7 patients experienced improvement in their vision at follow-up examinations. The 2 patients who experienced deterioration of vision already had severely reduced vision preoperatively, which indicates that surgery should be considered before the vision becomes significantly reduced., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Meningiomas arising from or adjacent to the optic nerve sheath meningioma (ONSM) are management challenges because of their risk of visual loss. Stereotactic radiosurgery (SRS) is a minimally invasive modality that can be added as adjuvant treatment for patients whose tumor has progressed or recurred after initial resection., Methods: The authors retrospectively reviewed 2,030 meningioma patients who underwent SRS between 1987 and 2022. In total, 7 patients (4 females; median age = 49) were found with tumors originating from the optic nerve sheath. None of the patients had tumors that engulfed the optic nerve as such tumors typically undergo fractionated radiation therapy (FRT) to preserve vision. The clinical history, visual function, and radiographic and neurological findings were characterized. Outcome measures included visual status, tumor control, and the need for additional management., Results: All patients underwent either initial gross total (n = 1) or partial surgical resection (n = 6) before SRS. Two patients with progressive tumor growth also had SRS after failing additional fractionated radiation after surgery (54 Gy, 30 fractions for both patients). The median time between the date of surgery and the SRS procedure date was 38 months. The Leksell Gamma Knife was used to deliver a margin dose of 12 Gy (range: 8-14 Gy) to a median cumulative tumor volume of 3.3 cc (range: 1.2-18 cc). The median maximal optic nerve radiation dose was 6.5 Gy (range: 1.9-8.1 Gy). After SRS, the median follow-up time was 130 months (range: 26-169 months). Two patients showed local tumor progression at 20 and 55 months after SRS. Four had stable visual function, 2 experienced improved visual acuity, and 1 patient had visual deterioration., Conclusions: Meningiomas arising from (but not engulfing the optic nerve) represent management quandaries after failed initial surgical removal. In this experience, salvage SRS was associated with tumor control and vision preservation in 5 of 7 patients. Additional experience with this strategy may further define the role of SRS both as a salvage and primary option., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Purpose: To report a case of a globular primary optic nerve sheath meningioma managed surgically with improvement in vision and review the literature for outcomes of purely intraorbital exophytic-globular primary optic nerve sheath meningiomas managed surgically., Methods: A literature review was conducted using Google Scholar and PubMed with the search terms "primary optic nerve sheath meningioma," "surgery," "exophytic," and "globular." Articles were included if they were available in English. Individual cases from the reviewed articles were included if the tumor was purely intraorbital with a globular or exophytic morphology, was managed with total or subtotal surgical excision, and visual outcomes were reported. Cases were excluded if the tumor extended intracanalicularly or intracranially, tumor morphology was unknown, or surgical management consisted of biopsy, optic nerve sheath decompression, or optic canal decompression rather than tumor debulking., Results: A total of 28 patients with intraorbital globular-exophytic primary optic nerve sheath meningiomas managed surgically have been reported in the literature. Vision improved in 29% (n = 8/28) and remained stable in 43% (n = 12/28) of patients. Furthermore, patients with good (Snellen notation ≥ 0.5) vision (n = 10) typically retained good vision postoperatively and at follow-up, with 1 patient experiencing a decline to poor (Snellen ≤0.1) vision at the last follow-up (92 months postoperatively). Similarly, patients with fair (Snellen notation >0.1 and <0.5) vision (n = 5) often improved to good vision (n = 3) or stayed at fair vision (n = 1), with 1 declining to poor vision at postoperative hospital discharge., Conclusions: Surgical management of exophytic or globular optic nerve meningiomas does not universally lead to vision loss and may be appropriate in select patients., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To assess the long-term effects of fractionated stereotactic radiotherapy (fSRT) for the treatment of (presumed) benign intraorbital tumours on visual acuity, visual fields, globe and eyelid position, and complications including radiation retinopathy and deviations of retinal nerve fibre layer (RNFL) on OCT., Methods: Multi-centre retrospective follow-up study of a consecutive series of 25 patients treated in the Rotterdam Orbital Center (collaboration between Erasmus Medical Center and Rotterdam Eye Hospital) between 2002 and 2018. Data on the dose of fSRT, visual acuity, Humphrey field analyser (HFA) perimetry, globe and eyelid position were obtained from the medical records., Results: In this retrospective consecutive series of 25 patients with a median follow-up of 104 months (range 48-215 months), 80.0% of the patients had presumed optic nerve sheath meningioma and 20.0 % presumed cavernous haemangioma with signs suggestive of recent growth. In most patients, a better visual acuity and RNFL thickness were observed after stereotactic radiotherapy. Improvement of the visual field defects was observed after treatment, with a mean deviation of -14.98 dB (12.9 SD) before treatment versus -4.56 dB (10.8 SD) after treatment, respectively. Significant, but small changes in exophthalmometry values were observed with a mean of 14.92 mm (7.9 SD) versus 13.79 mm (7.3 SD) after treatment, respectively. Only 3 patients (15.0%) developed radiation retinopathy after stereotactic radiotherapy. All patients with radiation retinopathy had presumed optic nerve sheath meningioma., Conclusions: Based on our results, fSRT is an effective treatment modality for this subset of orbital tumours with few complications and good long-term visual and cosmetic outcomes. FSRT is non-invasive and safer than surgery for lesions in the posterior orbit or around the optic nerve., (© 2022. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Primary optic nerve sheath meningioma (pONSM) is one of the most challenging tumors to manage. Although surgical excision may be considered a treatment option to maintain visual function, the safety of surgery remains debatable due to the non-negligible risk of optic nerve injury. pONSM often grows concentrically around the optic nerve; however, it can also demonstrate an exophytic growth from the optic nerve. The risk of surgical excision of pONSM may vary based on the tumor's growth pattern and area of contact with the optic nerve; however, there has been no detailed report on risk stratification to date. The authors present an illustrative case of an exophytic pONSM which was removed surgically without complications, suggesting that tumor morphology may also influence the surgical risk. In this report, the imaging and intraoperative features of exophytic pONSM are presented in detail, and the risk factors for complications are discussed., (Copyright © 2023 by Mutaz B. Habal, MD.)

Background: Optic nerve sheath meningiomas (ONMs) are often managed with radiotherapy (RT) with the goal of achieving radiographic local control (LC) and preventing deterioration of visual acuity (VA). We aimed to perform a systematic review and meta-analysis of outcomes for patients with ONM treated with RT., Methods: The PICOS/PRISMA/MOOSE selection criteria were used to identify studies. Primary outcomes were stable or improved VA and radiographic LC at last follow-up. The secondary outcomes were incidences of radiation-induced retinopathy and xerophthalmia and stable or improved visual fields (VFs). Weighted random-effects meta-analyses using the DerSimonian and Laird methods were conducted to characterize effect sizes. Mixed-effects regression models were used to examine potential correlations between gross tumor volume (GTV) and outcomes., Results: In total, 444 patients with ONM across 20 published studies were included. The estimated LC rate was 99.8% (95% confidence interval [CI], 98.3%-100%), and the estimated proportion of patients with stable or improved VA or VF was 89.7% (95% CI, 86.2%-92.4%) and 93.3% (95% CI, 89.5%-95.8%), respectively. Estimated incidences of radiation-induced retinopathy and xerophthalmia were 7.2% and 10.1%, respectively. GTV was significantly associated with VA (P = 0.014) with estimated VA rates of 96.4%, 91.4%, and 80.5% for GTVs of 2.0, 3.0, and 4.0 cm 3 , respectively., Conclusions: RT was well tolerated, with excellent LC achieved. Nearly 90% of patients noted either stability or improvement in VA and VF. Larger ONMs were associated with poorer VA., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Even though intracranial meningiomas commonly invade the optic canals, true optic nerve sheath meningiomas are extremely rare. They are insidious lesions that frequently grow in 4 stages, leading to progressive visual loss.1-4 Frequently, management includes observation for asymptomatic patients, and fractioned stereotactic radiotherapy with progressive visual loss.1,3 However, surgery is avoided due to the risk of perioperative visual loss.2-4 However, many of these cases present in the early stage of tumor growth, where an arachnoidal plane is still present, and vision can be saved by microsurgical tumor resection.2 Here we present the case of a 33-yr-old female presenting with a 3-wk history of blurred vision. Neuroimaging depicted an enhancing lesion along both optic nerves consistent with bilateral optic nerve sheath meningioma. Neuroophthalmological examination showed inferior cut of visual field bilaterally, more extensive on the left eye. Acuity was 20/20 on both eyes. Microsurgical resection of the tumor was performed through left supraorbital craniotomy, with the goal of eliminating intracranial extension and stabilizing visual function.5 The optic canal was unroofed with diamond bit drill under copious irrigation. A remarkable improvement of her visual field was observed with maintenance of acuity at 20/20 on follow-up. As the patient is attempting pregnancy, she is closely monitored without radiation. Surgery can be offered as a primary treatment of optic nerve sheath meningiomas, especially in early stages, with likely preservation and, in some cases, improvement of visual function.6 The patient consented to the procedure and the use of image. Images at 1:46 and 3:48 reprinted from Al-Mefty O, Operative Atlas of Meningiomas. 1998: Raven Press; Philadelphia, PA, with permission from LWW. Image at 2:57 reprinted from Rassi et al2; © Anil Can, 2018, used with permission. Image at 9:27 in public domain/age., (© Congress of Neurological Surgeons 2021.)

Purpose: The purpose of this paper is to provide novel insight into the rare pediatric meningiomas., Methods: We retrospectively analyzed pediatric surgical cases of meningioma during 2002 to 2017 in our institution. The preoperative, intraoperative, and the postoperative status were collected to find any unique features that has not reported in the past., Results: Nine surgeries out of 5 patients were identified. The mean age was 7 years old (range 1-14 years old). Four patients were females. The mean tumor diameter was 52 mm (range 23-81 mm). The tumor locations were optic nerve sheath, Sylvian fissure, parasagittal, trigone of the lateral ventricle, and cerebellopontine angle. The Sylvian fissure meningioma without dural attachment (MWODA) was found in a 15-month-old female. A relapsed parasagittal meningioma showed regression in histological grade and residual tumor demonstrated spontaneous regression. In the initial surgeries, Simpson grade 1 resection was achieved in 2 cases. The pathological diagnoses were 1 meningothelial, 1 metaplastic, 2 atypical, and 1 clear cell meningiomas. The mean postoperative follow-up period was 71 months. Three patients experienced recurrence of the tumor. At the latest follow-up, all patients were free of radiological tumor recurrence or regrowth with a mean follow-up of 4 years (range 1-6.9 years). All patients were in the modified Rankin scale of 0-1., Conclusions: MWODA is not considered to be rare in pediatric meningioma and should be included in the differential diagnosis. We presented a histologically regressed relapsed meningioma, which spontaneously regressed after subtotal resection. In the case of recurrent meningioma, surgical resection and adjuvant radiation therapy could be effective for long-term control of the tumor.

We analysed the long-term outcomes of patients with primary optic nerve sheath meningioma (ONSM) treated with stereotactic radiotherapy (SRT). 26 patients with primary ONSM were treated with SRT between 2004 and 2013 at a single institution. SRT was delivered with image guidance to a median dose of 50.4 Gy in 28 fractions. 4 patients had prior surgical debulking. At a median radiological follow-up of 68 months, the MRI based tumour control was 100%. Visual acuity improved in 10 (38.4%), remained stable in 10 (38.4%) and was reduced in 6 (23.1%) patients following treatment. Stable or improved vision post-treatment was seen in 92.3% of patients with good pre-treatment vision (best corrected visual acuity 6/18 or better), compared to only 61.5% of patients with poor pre-treatment vision (best corrected visual acuity 6/24 or worse). Overall, the treatment was well tolerated with no Grade 2 or greater acute toxicity. Minimal other ophthalmic complications were seen with only one patient developing late onset Grade 3 radiation retinopathy., (Copyright © 2019. Published by Elsevier Ltd.)

Objective: Although meningiomas frequently involve the optic nerve, primary optic nerve sheath meningiomas (ONSMs) are rare, accounting for only 1% of all meningiomas. Given the high risk of vision loss with these tumors, surgical intervention is seldom considered, and radiation or observation is commonly applied. Here, the authors describe the visual outcomes for a series of patients who were treated with surgery aiming at maximal tumor resection and highlight their prognostic factors., Methods: The authors retrospectively analyzed the data for 8 patients with intracanalicular ONSMs who had been surgically treated by the senior author (O.A.) between 1998 and 2016. Meningiomas extending into the optic canal from the intracranial cavity (i.e., clinoid, sphenoid wing, tuberculum sellae, diaphragma sellae) were excluded. Diagnosis was based on ophthalmological, radiological, and intraoperative findings, which were confirmed by the typical histological findings. Preoperative, postoperative, and follow-up visual assessments were performed by neuro-ophthalmologists in all cases., Results: The patients included 7 females and 1 male. The mean age at diagnosis was 45.1 years (range 25.0-70.0 years). Mean duration of follow-up was 38.9 months (range 3.0-88.0 months). All patients reported visual complaints, and all had objective evidence of optic nerve dysfunction. Their evaluation included visual field, visual acuity, funduscopy, and retinal fiber thickness. Total resection was obtained in 4 cases. Comparing preoperative and postoperative visual function revealed that 4 patients had improvement at the last follow-up, 1 patient had stable vision, and 3 patients had decreased function but none had total vision loss. All patients with good preoperative visual acuity maintained this status following surgical treatment. There was no surgical mortality or infection. Operative complications included binocular diplopia in 4 patients, which remitted spontaneously., Conclusions: Surgery can play a beneficial role in the primary treatment of ONSM, especially lesions located in the posterior third of the nerve. Total removal can be achieved with vision preservation or improvement, without major surgical complications, especially at early stages of the disease. Patients with good preoperative vision and CSF flow in the optic sheath have better chances of a favorable outcome than those with poor vision.

Optic nerve sheath meningiomas (ONSM) and intra-conal orbital lymphomas are common entities on the differential of a retrobulbar optic nerve involving space-occupying lesion. In this study, we compare the pre-surgical diagnosis, based on clinical presentation and neuroimaging, to the surgical pathology results of intra-conal orbital lymphomas and ONSM. This is an IRB approved retrospective chart review of orbital lymphomas and optic nerve sheath meningiomas biopsied by a single surgeon over a 4-year period at a single institution. Pre-surgical diagnosis and surgical pathology were compared. Fifteen cases of orbital lymphoma were identified. Fourteen were excluded based on extra-conal location. The single histologically confirmed intra-conal orbital lymphoma had a pre-surgical diagnosis of ONSM. Four cases of optic nerve sheath meningioma were identified. Three of the 4 cases of histologically confirmed ONSM had a pre-surgical diagnosis of ONSM. One of the 4 had a pre-surgical diagnosis of lymphoma. Diagnosis based on surgical pathology differed from the pre-surgical diagnosis in 2 out of 5 cases showing that clinical diagnosis does not always correlate with histologic diagnosis. Although both diseases are typically managed with radiation therapy, the treatment dosage and systemic disease implications are very different. These findings emphasis the importance of biopsy in the diagnosis of orbital lesions surrounding the optic nerve.

Optic nerve sheath meningioma (ONSM) is typically diagnosed based on clinical suspicion and imaging characteristics and is most often treated with radiation. Historically, biopsy, optic nerve sheath decompression, and debulking surgeries have been avoided for fear of optic nerve vascular disruption and tumor spread into the orbit. This is a case of a 48-year-old man who presented with unilateral optic disc edema, declining visual acuity, and a visual field defect. Despite an initial improvement with acetazolamide, his vision subsequently worsened. With an elevated lumbar puncture opening pressure and imaging showing right optic nerve sheath enhancement, the differential diagnosis included ONSM, perineuritis and idiopathic intracranial hypertension (IIH). Optic nerve sheath decompression (ONSD) with biopsy was performed, simultaneously decompressing the nerve and yielding a sample for pathologic analysis. A pathologic diagnosis of ONSM was made and treatment with radiation was subsequently initiated, but vision began to improve after the surgical decompression alone.

The coexistence of aneurysms and meningiomas is rare, and encasement of an aneurysm within a meningioma even more so. We report a case of an optic nerve sheath meningioma encasing an aneurysm of the internal carotid artery and present a brief review of the literature., (Georg Thieme Verlag KG Stuttgart · New York.)

Objectives: With respect to its characteristic pattern of growth from the orbit into the intracranial space toward the chiasm, patients with optic nerve sheath meningiomas (ONSM) are threatened to loose function of both optic nerves. Fortunately, in less than 5% both optic nerves are involved initially. Hence, prevention of vision of the contralateral eye is the foremost aim of any therapy. Performing pre-chiasmatic transection might offer a further treatment option to avoid further tumor growth toward the chiasm., Patients and Methods: In this retrospective study 12 patients with ONSM and blindness of the affected eye were included. The surgical approach was performed either from pterional intradural or as a combined approach from pterional extra- and intradural., Results: Without any exceptions, vision of the contralateral eye could be preserved and did not show any deterioration after surgery or during the follow-up time of 50.6 months. Furthermore in 58.3% of patients gross total tumor resection could be achieved. During follow up observation in 67% of patients no further tumor progress or recurrences could be observed. 4 patients, however, showed delayed tumor progress or recurrences that were treated by radiotherapy., Conclusion: Pre-chiasmatic transection of the optic nerve might offer a surgical treatment option to control tumor growth and to preserve vision of the contralateral eye., (Copyright © 2013 Elsevier B.V. All rights reserved.)

Meningiomas rarely cause CSF dissemination, and CSF seeding to the optic nerve (ON) is extremely rare. This is the first report of 2 cases of atypical meningioma with subacute visual loss due to ON seeding. The authors present the genetic characteristics of these atypical meningiomas with CSF dissemination. The patient in Case 1 was a 36-year-old woman with a 1.5-cm mass within the left ON, and the patient in Case 2 was a 70-year-old woman with a 0.9-cm mass around the right ON. Both individuals had undergone multiple surgeries for primary lesions and local recurrent lesions. They presented with subacute visual loss, and both tumors were completely resected. The pathological diagnosis was atypical meningioma with high MIB-1 indices and p53-positive cell ratios in each case. Comparative genomic hybridization showed significant chromosomal copy number alterations similar to the results of previous surgeries, confirming that the tumors were disseminated lesions. The present findings suggest that genetic characteristics, such as 1p and 10qcen-23 losses and 17q and 20 gains, shared by the 2 cases might be associated with CSF dissemination of meningiomas.

A 36-year-old woman presented with progressive loss of vision in the left eye for 3 years, and rapid progression and painful protrusion of the eye for one month. Clinical evaluation revealed no light perception, severe proptosis and hypoglobus, optic atrophy, and optociliary shunt vessels. Orbital imaging showed a well-defined heterogeneous intraconal mass partially encasing the optic nerve. A clinical diagnosis of optic nerve sheath meningioma was made, and the tumor was completely excised along with enucleation, followed by postoperative adjuvant external beam radiotherapy. There was no local recurrence at 15 month follow-up. Histopathologically, the tumor was found to be arising from the optic nerve meninges with classical "stag-horn" pattern and abundant cellularity. Immunohistochemistry supported the histopathological diagnosis of hemangiopericytoma. Optic nerve meningeal hemangiopericytoma is extremely rare-only two such cases have been reported in the literature., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Meningiomas are the most common form of primary intracranial tumors. If the anterior visual pathways are affected clinical visual compromise, visual field defects, oculomotor nerve disturbances or propotosis are predominant. Meningiomas of the anterior visual pathway remain therapeutically challenging due to the direct anatomical relationship to the circulus arteriosus cerebri, the cavernous sinus and cranial nerves. The therapy of choice is microsurgical resection. In many cases a curative approach can be achieved with modern surgical techniques and simultaneously maintaining patient quality of life. Problematic are cases in which resection would be associated with the risk of clinical impairment and in these cases radiotherapy is an important therapeutic option. In cases involving complex invasiveness an interdisciplinary treatment to control local clinical symptoms is favored.

Introduction: Early diagnosis of tumors which compress the anterior optic apparatus is important in order to prevent visual functional damage. Pre-operative visual function is correlated with post operative visuaL results, therefore, delay in diagnosis leading to deterioration in vision may cause irreversible damage., Objectives: Reviewing our experience with 100 patients with meningiomas involving the optic apparatus and establishing guidelines for early imaging in patients with visual impairment to prevent delays in diagnosis., Methods: Data was retrospectively collected for 100 patients with a meningioma involving the anterior optic apparatus, who underwent surgery between the years 2003-2011. Data was collected from hospital inpatient and outpatient files, as well as eye exams from the outset of the patient's symptoms and other exams that were carried out in the community., Results: In 14 patients there was a delay of one year or more in the diagnosis of meningioma. Of these, 3 were initially diagnosed with glaucoma, 3 with cataracts, 2 with migraines, in 5 patients no further investigation was conducted in spite of their ongoing complaints and in 1 patient, brain imaging was reported as normal., Conclusions: In a distinct number of patients there was a delay in diagnosis of over a year. A patient with complaints of deterioration in visual acuity which is not corrected by glasses, with examination findings of an afferent pupil and visual field defects, should undergo neuro-imaging in order to rule out a space-occupying lesion. This should be the case even if the patient has findings of a cataract which may partially explain deterioration in visual acuity.

Aims: To assess visual outcome, tumour control and treatment-related morbidity in patients with optic nerve sheath meningiomas (ONSMs) treated with fractionated stereotactic radiotherapy (FSRT)., Patients and Methods: A retrospective analysis of 45 patients (13 men and 32 women, median age 46 years) with ONSMs (51 optic nerves involved) treated in a single institution between 1997 and 2010 was carried out. FSRT was delivered to a dose of 50 Gy in 30 or 33 fractions as primary treatment in 39 patients and after surgery in six patients., Results: At a median follow-up of 30 months (range 1-13 years), the tumour control in 41 evaluable patients (four were lost to follow-up) was 100% at 5 years with no subsequent local or distant recurrence. Of the 46 evaluable optic nerves treated, 41 had residual vision (38 with impaired vision) before radiotherapy and five were blind in one eye. There was no recovery of vision in any of the blind eyes. Of 41 optic nerves with residual vision, 13 had improvement, 24 remained stable and four deteriorated; two patients (4%) developed radiation retinopathy. One patient developed a central retinal artery occlusion in the untreated eye 10 years after treatment., Conclusion: FSRT is highly effective at controlling the growth of ONSMs with improvement or stabilisation of visual deficit in 89% of the optic nerves retaining some vision, albeit with a small risk of radiation-induced retinopathy. The results support the use of FSRT as an effective approach in the management of ONSM. The lack of functional benefit in patients with severe visual impairment would argue for earlier institution of treatment before complete visual loss is established., (Copyright © 2012 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Optic nerve sheath meningiomas (ONSM) are rare tumors of the meninges that surround the optic nerve as it enters the orbit. If left untreated, these benign tumors lead to progressive compression of the optic nerve and vascular compromise resulting in visual loss. Surgical resection of ONSM is associated with a high morbidity due to injury to the vascular supply of the optic nerve, with up to 94% of patients reporting worsened vision post-operatively. Fractionated radiotherapy is a non-invasive alternative to surgery for the treatment of ONSM that has demonstrated improved outcomes. The reported long-term tumor control rates approach 100%, with greater than 80% vision preservation or improvement after treatment. Recently, improved technology for delivery of radiotherapy, including stereotactic and three-dimensional conformal radiotherapy has emerged. The literature suggests that the modality of radiotherapy does not affect the outcomes as long as conformal targeting with a total dose of 50 Gy to 54 Gy and a fractional dose of less than 2.0 Gy is used. Radiosurgery is not generally used for ONSM due to the high toxicity to the optic nerve when high-dose single fraction radiation is given. Therefore, conformal fractionated radiotherapy appears to be the most effective treatment for ONSM, and should be used as a primary therapy unless there is a specific indication for surgical intervention., (Copyright © 2012. Published by Elsevier Ltd.)

Meningiomas are the most common non-malignant tumours of the brain. Gross-total resection remains the preferred treatment, if achievable without morbidity. Radiation therapy is advocated for inoperable, incompletely resected, or recurrent grade 1 tumours, if there is a progressive, symptomatic lesion, or in case of functional impairment. Postoperative radiation therapy is recommended for grade 2 or 3 lesions. Fractionated stereotactic radiotherapy and stereotactic radiosurgery are high precision techniques, allowing good sparing of surrounding tissues. Fractionated stereotactic radiotherapy and stereotactic radiosurgery give comparable results, with excellent 5-year tumour control rates of more than 90% for benign meningiomas. Toxicity is low and seems equivalent, despite a biased use of fractionated stereotactic radiotherapy for larger meningiomas, close to critical structures. Fractionated stereotactic radiotherapy seems to be of special interest in the treatment of cavernous sinus or optic pathways meningiomas. The different therapeutic modalities should be discussed by a multidisciplinary team., (Copyright © 2012 Société française de radiothérapie oncologique (SFRO). Published by Elsevier SAS. All rights reserved.)

Purpose: To evaluate the effectiveness of fractionated stereotactic radiotherapy (SFRT) in the treatment of optic nerve sheath meningioma (ONSM)., Methods and Materials: Between 1993 and 2005, 109 patients (113 eyes) with primary (n = 37) or secondary (n = 76) ONSM were treated according to a prospective protocol with SFRT to a median dose of 54 Gy. All patients underwent radiographic, ophthalmologic, and endocrine analysis before and after SFRT. Radiographic response, visual control, and late side effects were endpoints of the analysis., Results: Median time to last clinical, radiographic, and ophthalmologic follow up was 30.2 months (n = 113), 42.7 months (n = 108), and 53.7 months (n = 91), respectively. Regression of the tumor was observed in 5 eyes and progression in 4 eyes, whereas 104 remained stable. Visual acuity improved in 12, deteriorated in 11, and remained stable in 68 eyes. Mean visual field defects reduced from 33.6% (n = 90) to 17.8% (n = 56) in ipsilateral and from 10% (n = 94) to 6.7% (n = 62) in contralateral eyes. Ocular motility improved in 23, remained stable in 65, and deteriorated in 3 eyes. Radiographic tumor control was 100% at 3 years and 98% at 5 years. Visual acuity was preserved in 94.8% after 3 years and in 90.9% after 5 years. Endocrine function was normal in 90.8% after 3 years and in 81.3% after 5 years., Conclusions: SFRT represents a highly effective treatment for ONSM. Interdisciplinary counseling of the patients is recommended. Because of the high rate of preservation of visual acuity we consider SFRT the standard approach for the treatment of ONSM. Prolonged observation is warranted to more accurately assess late visual impairment. Moderate de-escalation of the radiation dose might improve the preservation of visual acuity and pituitary gland function., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: Traditional treatment options for optic nerve sheath meningiomas (ONSMs) include observation, surgery, and radiotherapy, but to date none of these has become the clear treatment of choice., Objective: To evaluate the effectiveness and safety of multisession radiosurgery for ONSMs., Methods: From May 2004 to June 2008, 21 patients with ONSMs were treated by radiosurgery using the frameless CyberKnife system. Patient age ranged from 36 to 73 years (mean, 54 years). All patients were treated using multisession radiosurgery, with 5 fractions of 5 Gy each to a total dose of 25 Gy prescribed to the 75% to 85% isodose line. Patients were evaluated for tumor growth control and visual function., Results: The median pretreatment tumor volume was 2.8 mL (range, 0.3-23 mL). The mean follow-up was 30 months (range, 11-68 months). All patients tolerated treatment well, with only 1 patient in whom a mild optic neuropathy developed (which remitted after systemic steroid therapy). No other acute or late radiation-induced toxicities were observed. No patients showed ONSM progression on follow-up magnetic resonance imaging. Two patients (10%) had a partial response. No patients had worsening of visual function; visual function was stable in 65% and improved in 35% of patients., Conclusion: Multisession radiosurgery for ONSMs was found to be safe and effective. The preliminary results from this study, in terms of growth control, visual function improvement, and toxicity, are quite promising. Further investigations are warranted.

Purpose: We analyzed our long-term experience with fractionated stereotactic radiotherapy (FSRT) in patients with meningioma of the optic nerve sheath (ONSM)., Patients and Methods: Between January 1991 and January 2010, 40 patients with ONSM were treated using FSRT. Of these, 19 patients received radiotherapy as primary treatment, and 21 patients were treated after surgical resection. The median target volume was 9.2 ml, median total dose was 54 Gy in median single fractions of 1,8 Gy., Results: Local progression-free survival was 100%. Median survival after FSRT was 60 months (range 4-228 months). In all patients overall toleration of FSRT was very good. Acute toxicity was mild. Prior to RT, 29 patients complained about any kind of visual impairment including visual field deficits, diplopia or amaurosis. Prior surgical resection was identified as a negative prognostic factor for visual outcome, whereas patients with larger tumor volumes demonstrated a higher number of patients with improvement of pre-existing visual deficits., Conclusion: Long-term outcome after FSRT for ONSM shows improved vision in patients not treated surgically prior to RT; moreover, the best improvement of visual deficits are observed in patients with larger target volumes. The absence of tumor recurrences supports that FSRT is a strong alternative to surgical resection especially in small tumors without extensive compression of normal tissue structures.

Objective: Optic nerve sheath meningiomas (ONSMs) represent the most challenging lesions involving the optic pathways: Microsurgery is not indicated and classical single-stage radiosurgery appears to be too risky due to the expected destruction of the common blood supply with consequent loss of vision. Staged radiosurgery might be one treatment option because it exploits the ability of normal tissues to repair sub-lethal radiation-induced damage, offering a chance to control tumor growth while sparing function. Staged robotic radiosurgery was offered to 5 patients harboring ONSMs with the aim of sparing vision while achieving local growth control., Patients and Methods: Five patients with ONSM presenting with visual field deficits and loss of visual acuity were treated with staged CyberKnife radiosurgery, receiving 20 Gy in 4 stages (5 Gy per stage). Treatment planning was based on contrast-enhanced thin-slice CT (1.25 mm thickness for the first three cases, 0.5 mm for the last two) and volumetric MR imaging (1.5 T for the first three cases, 3 T for the last two). An interval of 24 hours was strictly observed between stages. Visual acuity and visual fields were assessed in all patients immediately prior to treatment and at intervals of 6 months thereafter. Follow-up MRIs were performed every 6 months for 2 years, then once per year., Results: The entire procedure, inclusive of imaging, treatment planning and treatment delivery, was performed in 5 days. Irradiation required approximately 45 min per stage. Mean tumor volume was 2.94 cc (range: 0.8-6.4 cc). Treatment was well tolerated in all patients. Follow-up ranged from 36 to 74 months. Local growth control was achieved in all patients. Restoration of normal vision was experienced by 4 patients 6 to 12 months after the treatment. One patient, who was also affected by diabetic retinopathy, showed a modest improvement after 6 months, remaining stable thereafter., Conclusion: Staged CyberKnife radiosurgery provides a fast and well-tolerated non-invasive treatment with excellent visual outcomes. If these preliminary results are confirmed by larger series, staged radiosurgery could be proposed as a first-line treatment for ONSM.

Object: The goal of this study was to assess the long-term results of Gamma Knife surgery (GKS) in patients harboring an optic nerve sheath meningioma (ONSM)., Methods: Thirty patients harboring an ONSM were treated with GKS between 1998 and 2003. Gamma Knife surgery was performed as the sole treatment option in 21 of these patients and resection had been performed previously in 9 patients. The mean volume of the tumor at the time of GKS was 3.6 cm(3) (range 1.4-9.7 cm(3)), and the mean prescription peripheral dose was 13.3 Gy (range 10-17 Gy). The mean number of isocenters used to treat these lesions was 8 (range 5-14 isocenters)., Results: At a median follow-up of 56 months, visual acuity improved in 11 patients, remained stable in 13 patients (including 4 patients who were completely blind before GKS), and deteriorated in 6 patients. Follow-up images were available in all patients and showed tumor regression in 20 patients and stable tumor in 8 patients. Persistent imaging evidence of progression was only present in 2 patients. With the exception of reversible conjunctival edema in 4 cases, no other serious acute side effect was observed., Conclusions: Gamma Knife surgery provides long-term tumor control for ONSM. The results of this study add substantial evidence that GKS may definitely become a standard treatment approach in selected cases of ONSM.

Objective: The management of optic nerve sheath meningiomas (ONSM) remains controversial, but includes surgery, radiotherapy and plain observation. We present a follow-up study and treatment modalities based on our classification system., Patients and Methods: A retrospective analysis was performed of 90 patients with optic nerve sheath meningiomas who were treated between 1991 and 2008 (n=65 surgery only, n=5 radiation only, n=18 surgery and postoperative radiation, n=2 observation). Follow-up data was available, ranging from 6 to 220 months with a median of 45.8 months., Results: Our classification system differentiates between intraorbital (type 1), intracanalicular or intrafissural (type 2), and intraorbital and intracranial (type 3) ONSMs. Thirty-seven tumors demonstrated extension through the optic canal (type 2a). 41 further tumors reached the chiasm (type 3a) or contralateral side (type 3b). Visual acuity was not significantly influenced by surgery but did become worse with a longer duration of preoperative symptoms and a longer follow-up period. Radiotherapy improved vision in 4 and preserved vision in 16 out of 23 cases., Conclusions: Loss of vision in optic nerve sheath meningiomas is a question of time. Radiotherapy should be offered for intraorbital ONSM. Surgery with decompression of the optic canal and intracranial tumor resection is still favored for tumors with intracanalicular and intracranial extension., ((c) Georg Thieme Verlag KG Stuttgart-New York.)

Optic nerve sheath meningiomas account for one-third of optic nerve tumors, 1-2 % of all meningiomas, and 2-3.5 % of all orbital tumors. Loss of vision is the main clinical sign. Diagnosis is easily made after the neuroradiological evaluation. Fractionated stereotactic radiotherapy (RSF) is now the gold standard treatment for controlling tumor volume and preserving visual acuity., (Copyright 2010 Elsevier Masson SAS. All rights reserved.)

Meningioma, though benign, may invade adjacent structures such as bone, soft tissues, dural sinuses and arteries. However brain infarctions secondary to meningioma involving the cavernous sinus and encasing and narrowing the intracranial carotid artery are rare. We report the case of a young man with recurrent left carotid artery infarctions due to a left sphenoid meningioma infiltrating the posterior optic nerve sheath through the optic canal and circumscribing the intracranial carotid artery. The patient had a gradually progressive occlusion of the middle cerebral artery, the distal internal carotid artery and finally the anterior cerebral artery ipsilateral to the sphenoid meningioma.

Aims: Optic nerve sheath meningiomas typically present with unilateral visual deterioration. Here, a single centre's experience with radiotherapy aimed at local control and visual stabilisation is presented., Materials and Methods: The meningioma database within the Radiation Oncology Department, Prince of Wales Hospital was audited for patients whose meningiomas took origin from the optic nerve sheath. Excluded from this evaluation was any patient whose meningioma secondarily involved the optic nerve. Where vision was not a consideration, treatment was given by stereotactic radiosurgery for patients with retained vision. The remaining patients were treated by fractionated radiotherapy, predominately via a stereotactic approach. The main end points were: lack of radiological progression of the tumour and maintenance of preradiotherapy vision., Results: There were 15 eligible patients, one patient with neurofibromatosis had bilateral optic nerve involvement; thus, 16 optic nerves were treated. Women (10) outnumbered men (five) and the age range was 7-74 years. One patient progressed outside the volume treated (for a geographical failure) with no infield progression. This patient became blind, was re-treated by stereotactic radiosurgery, had tumour control and vision improved. Thus, for 17 optic nerves (or part thereof) treated, all patients ultimately had local control (100%) with worsening of vision only occurring in one patient. No other late morbidity was present for any patient., Conclusion: Optic nerve sheath meningiomas have high local control rates and preservation of vision with radiotherapy.

Intraoperative visual system monitoring of lesions with a close relationship to the optic apparatus by using light flashes reportedly is difficult to perform, and the results are too unreliable to interpret. The authors used direct epidural electrical stimulation of the optic nerve (ON) during surgery instead of light flashes. Four patients were included in this feasibility study. In 3 patients-1 each harboring a planum sphenoidale meningioma, a tuberculum sellae meningioma, and an intraorbital ON sheath meningioma-2 stimulating needle electrodes were placed on each side of the ON just anterior to the optic canal, before unroofing the optic canal and an extradural anterior clinoidectomy. In the fourth patient, who harbored a frontotemporal astrocytoma, stimulation was applied at the exit of the ON from the canal. The electrically induced visual evoked potentials (eVEPs) were recorded from the scalp before, during, and after tumor removal. A typical eVEP consisted of N20 and N40 waves. The amplitude of the N40 wave varied up to 25% prior to tumor removal. In the patient with a symptomatic tuberculum sellae meningioma, the decompressive effect of opening the optic canal and the impact of manipulation during piecemeal tumor removal were detected by the eVEPs. In the patient with an ON sheath meningioma and light sensation, only the N20 wave was observed. Epidural electrical stimulation of the ON is a safe means of providing a stable signal and real-time information on nerve conduction during surgery. It may be a useful adjunct in improving visual outcomes postoperatively. Further clinical studies are necessary.

Case Report: A 56-year-old woman with a right optic nerve sheath meningioma and visual loss, was treated with fractionated stereotactic radiotherapy. This resulted in an almost complete recovery of her visual acuity., Discussion: Currently there are many different alternatives available for the management of an optic nerve sheath meningioma: observation, surgery, conventional radiotherapy and fractionated stereotactic radiotherapy. The last of these treatments has been demonstrated to preserve or improve vision in 2 out of 3 treated patients, with fewer side effects, and has not been associated with recurrent disease or tumor progression.

Primary optic nerve meningiomas occur at lower ages than meningiomas arising from the coverings of the brain and spinal cord. Here we report the case of a 20-year-old female with an aggressive orbital meningioma referred to the Ophthalmology Department of the Farabi Hospital in Tehran. The patient had a history of orbital meningioma from 10 years ago and several surgical resections due to tumor recurrence during these 10 years. On admission, the patient had a large orbital mass and severe proptosis. MRI images revealed a large left orbital mass with optic nerve involvement and extension to the left maxillary sinus, pterygoid fossa and the dura in the floor of the anterior fossa. Fine-needle aspiration cytology of the mass confirmed tumor recurrence. The patient first received radiotherapy due to the inoperable mass, and the tumor was resected 1.5 month later. Microscopic study showed meningotheliomatous meningioma with extensive involvement of the optic nerve and invasion of the optic disc, sclera and choroid. The interesting aspect of this case was the aggressive behavior of the tumor with intraocular invasion, despite its benign histopathological features, which led to wide exenteration of the eye together with resection of the upper and lower lids.

Unlabelled: Optic nerve sheath meningioma (ONSM) accounts for one-third of primary optic nerve tumors, and 2% of all meningiomas. ONSM must be distinguished from other meningiomas, in particular from cavernous meningiomas because of the different prognosis and treatment. The most frequent clinical sign is a progressive or sudden unilateral visual loss. Treatment of ONSM is still subject to discussion. This report covers a series of eight ONSM patients treated with fractionated stereotactic radiotherapy., Material and Methods: Between 2000 and 2006, we managed eight patients with ONSM. The average patient age was 47 years. There were five women and three men. The most frequent clinical signs were visual loss (100%), proptosis (35%), diplopia (25%). One patient was initially treated with surgery. All patient have been treated by fractionated stereotactic radiotherapy. 45 Gy in 25 fractions were delivered on the meningioma area at a rate of 5 fractions of 1.8 Gy per week. We used a Brainlab framework associated with a thermo-formed mask. A computed tomography then magnetic resonance imaging was obtained for each patient. The data was merged and planning took place on a Brainlab dosimetric console. The treatment was performed with a head-only Varion linear accelerator, with a Brainlab multi-blade collimator., Results: The average follow-up was 27 months. Each patient had a complete radiological and ophthalmologic exam every 3 months during the first year, then every 6 months thereafter. Tumor control rate was 100%. Vision was re-established in five patients and three patients had improvement, including one patient during treatment. 100% of proptosis and diplopias regressed. No side effect was reported., Conclusion: This is still a preliminary study, but the results suggest that fractionated stereotactic radiotherapy may emerge as a primary treatment for ONSM, delaying surgery, which has proven functionally disappointing.

A 28-year-old man with a biopsy-proven benign intraorbital optic nerve sheath meningioma developed recurrent clinical manifestations of ipsilateral retrobulbar inflammation 9 years after undergoing postoperative radiation therapy. Debulking of the tumor 11 years after the original surgery again revealed no pathologic signs of inflammation. Whether growth of tumor, surgery, radiation, or edema triggered the inflammatory manifestations is unclear. Our case affirms that primary optic nerve meningiomas may rarely cause episodic manifestations resembling those of idiopathic orbital inflammation that resolve with corticosteroid treatment.

Optic nerve sheath meningiomas (ONSMs) are benign lesions originating from the dural sheath of the optic nerve. Progressive growth can lead to gradual loss of vision and exophthalmos. Loss of vision following microsurgical resection is not uncommon, and although stereotactic fractionated radiotherapy can be a safe alternative to control tumor growth and preserve vision, it may also lead to complications. Frame-based stereotactic radiosurgery has only been rarely used because single-fraction high-dose treatments of intrinsic optic nerve lesions may induce unacceptably high toxicity. New frameless radiosurgery devices such as the robotic CyberKnife, an image-guided radiosurgery system, can provide the extremely tight conformality and submillimetric accuracy of frame-based systems combined with the possibility of delivering radiation in several sessions. In the present report the authors review the clinical presentation and management of ONSMs and describe their preliminary experience using multisession radiosurgery to treat these lesions.

Objective: To evaluate results of stereotactic radiotherapy for the treatment of optic nerve sheath meningioma (OM) at Ramathibodi Hospital., Material and Method: Twelve patients with primary OM were treated with stereotactic radiation between 1998 and 2005. Five patients underwent surgery and had no light perception before radiation. All patients except one were treated with fractionated stereotactic radiotherapy (FSRT). Mean average dose of FSRT was 55. 7 Gy; 180 cGy/fraction. One patient was treated with 15-Gy stereotactic radiosurgery., Results: With a median follow-up of 34 months, there was no visual improvement in the five patients who were completely blind before radiation. Visual acuity improved in four patients and remained stable in two patients. Four of six patients had improved visual field, and five of six decreased in proptosis. Follow-up images were available in six patients, showing minimal tumor regression in five and stable in one. No serious acute side effect was observed. Vision became worse in one patient, who developed vitreous hemorrhage two years after FSRT., Conclusion: Stereotactic radiotherapy is an effective treatment for primary OM. It provides tumor control and visual preservation with low risk of complications. However more patients and further follow-up are needed for long-term outcomes.

Purpose: To describe a role for optic nerve decompression as adjuvant surgical therapy in the management of optic nerve sheath meningioma in patients with severe, progressive visual loss and optic disc edema before or after radiation therapy., Methods: Interventional case report., Results: Two patients with unilateral optic nerve sheath meningioma had progressive visual loss (20/200 and no light perception) and disc edema. The first had previously undergone fractionated stereotactic radiotherapy and the second subsequently was treated with fractionated stereotactic radiotherapy after decompression surgery. After excision of a dural window and biopsy of the tumor from the nerve sheath, visual acuity improved to 20/25 and 20/200, respectively, both coinciding with resolution of disc edema., Conclusions: In patients with optic nerve sheath meningioma with severe disc edema and rapid vision loss, surgery may serve an important but restricted, adjuvant role to radiation therapy under special conditions.

A 58-year-old woman with a 35-year history of left proptosis underwent neuroimaging that revealed a large cystic lesion. Surgery revealed an optic nerve sheath meningioma associated with cyst formation. The cyst was part of the tumor, a phenomenon that is well described in intracranial meningiomas but not in optic nerve sheath meningiomas.

The authors describe the case of bilateral optic nerve sheath meningioma /ONSM/ in 14 years old girl. When being hospitalized at the Neurology Clinic with paresis n.VII, the patient was examined by ophthalmologist. The diagnosis was made on the basis of clinical and characteristic changes revealed by USG, CT and MRI. The very first ophthalmologic examination confirmed a visual acuity 5/5 OD and hand movements OS, associated with papilloedema OD and edematous optic atrophy OS. The use of USG, CT and MRI revealed bilateral tubular thickening of the optic nerves (8mm), with calcification. After the period of one year, clinical progression was observed. The intracranial tumor extended. It was shown in diagnostic imaging. The patient was operated on by neurosurgeons with histopathology verification. In the later stage she underwent radiotherapy. The case has been described because of its diagnostic and therapeutic difficulties.

Objective: To investigate the clinical effect of early stage operation for optic nerve sheath meningioma., Methods: Retrospective study The clinical data of 21 cases of optic nerve sheath meningioma, 6 males and 25 females, aged 39 (12-60), with the chief complaints of loss of vision in 13 eyes (including 6 blind eyes) and proptosis in 8 eyes who underwent resection of the tumors via transcranio-orbital approach were analyzed retrospectively. Radiology showed 15 tumors growing along the optic nerve and enclosing it (flat type), 5 of which showed intracranial invasion; and other 6 of which had the tumors located on one side of optic nerve with spherical shape (nodular type) without intracranial invasion. The patients were followed up for 2-6 years., Results: Total resection was accomplished in 15 cases, and partial removal in 6 cases. Function of oculomotor nerve was preserved in 17 cases, and function of abducent nerve was preserved in 18 cases, visual function was successfully preserved in the 2 cases of small tumor. Tumor recurred in two cases., Conclusion: Resection of optic nerve sheath meningioma via transcranio-orbital approach has the advantage of totally removing the tumor and preserving the function of the oculomotor and abducent nerves, preventing tumor from intracranial invasion. Early stage operation is recommended. Visual function may be preserved in the cases of small tumors.

The management of optic nerve sheath meningeoma has been controversial and difficult. The conservative strategy was "to wait and see" until the patient became blind on the affected eye. Surgical excision had poor outcome and, with the exception of a few anecdotal case stories, led to blindness or postoperative eye motility disturbances. It has been long known that radiation therapy can prevent tumour progression, but the end result was very often visual deterioration and blindness. A few recent well documented studies describe how stereotactic fractionated conformal radiotherapy can provide stabilisation or improvement in the visual function in optic nerve sheath meningeoma. This article gives an overview, illustrated by a case story.