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Background: Progressive isolated optic nerve glioma (ONG) in children is a rare disease, treated with various modalities. A global treatment consensus is not available., Methods: We conducted a national retrospective multicenter cohort study (1995-2020) to investigate how different treatment strategies impact outcome for ONG in children, by assessing treatment responses to systemic anticancer therapy (SAT), surgery, and radiotherapy for ONG. The primary endpoints included changes in best-corrected visual acuity (BCVA) and tumor volume (TV) on MRI, both evaluated at the start and end of therapy and at long-term follow up., Results: A total of 21 ONGs (20 patients) received SAT (n = 14 (66.7%)), surgery (n = 4 (19.0%)), and radiotherapy (n = 3 (14.3%)). After SAT BCVA stabilized or improved in 66.6% (n = 4) and the TV decreased by a median of 45.1% (range: -88.6% to +31.5%) (n = 13). Before resection two eyes were already blind. After resection BCVA decreased to blindness in one eye. In total all four eyes were blind after resection. After first-line RT BCVA decreased in 66.7% of ONG to counting fingers or less, TV increased <3 months after RT by a median of 47.3% (range: -42.8% to +245.1%) (n = 3), followed by a long-term decrease of 94.4 and 13.8% (n = 2), respectively., Conclusion: SAT appears to be the preferred modality for progressive ONG in case of potential rescue of visual functions. Complete resection of ONG appears effective to reduce proptosis in case of preexisting blindness. The use of radiotherapy requires careful consideration due to the risk of severe visual impairment and secondary disease., (© 2024 The Author(s). Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Tumors of the anterior visual pathways are challenging to operate because of the crucial balance between extent of resection and sight preservation. Sodium fluoresceine (SF) has already been reported in maximizing brain tumor resection but not when the optic-chiasmatic region is involved. The role of Visual Evoked Potentials (VEPs) in optic-chiasmatic tumors is still debated. The simultaneous use of both technique in this setting has not been reported so far. The aim of our work is to analyse the intraoperative combination of the use of SF and VEPs in the anterior visual pathway tumor surgery. Clinical and intraoperative data from six surgical procedures on four patients affected by optic-chiasmatic tumors with preoperative visual deficits were retrospectively analyzed. In five procedures VEPs remained intraoperatively stable and no postoperative worsening of visual function was reported. In 1 case an intraoperative VEPs deterioration was predictive of a postoperative visual worsening. In all cases, tumor was fluorescent, and SF was intraoperatively helpful in guiding resection. The pattern of fluorescence was different according to the lesion histology. Based on our preliminary experience, the simultaneous combination of SF and VEPs in surgery of the optic pathway tumors seems helpful to maximize tumor resection and predict postoperative functional outcome. Since we presented a small series, larger studies are needed to confirm our data.

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).)

Optic nerve schwannoma (ONS) is an exception in that it does not possess the typical Schwann cells. Instead, it is believed to possibly originate from ectopic neural crest Schwann cells and perivascular Schwann cells. There are very few cases of Optic Nerve Schwannoma reported in literature. The patient is a 68-year-old male who presented with progressive left eye loss of vision. The patient's treatment encompassed Trans Nasal Endoscopic Excision of the mass with simultaneous Orbital Decompression. Laryngoscope, 134:3102-3104, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

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.)

Background: Cavernous malformations (CMs) of the optic nerve and chiasm are extremely rare, accounting for less than 1% of all intracranial CMs. Acute, subacute, or progressive visual loss from CM may occur with or without hemorrhage. Prompt surgical excision of the CM offers the best hope to improve or stabilize vision. Given its rarity, optic nerve and chiasm CMs may not be readily suspected. We provide 3 cases of optic nerve and chiasm CM, highlighting key neuroimaging features and the importance of expedited intervention., Methods: Case records of the neuro-ophthalmology clinics of the Bascom Palmer Eye Institute and the University of Colorado, and literature review of reported cases of optic CM., Results: A 49-year-old woman reported acute progressive painless vision loss in the right eye. MRI showed a suprasellar mass with heterogeneity in signal involving the right prechiasmatic optic nerve. Surgical excision of the CM 5 days after onset of visual loss improved vision from 20/300 to 20/30. A 29-year-old woman with acute painless blurred vision in the right eye had anterior chiasmal junctional visual field defects corresponding to a heterogeneously minimally enhancing mass with blood products enlarging the optic chiasm and proximal right optic nerve. Surgical excision of the CM 8 weeks after onset of visual loss improved vision from 20/40 to 20/15 with improved visual fields. A 33-year-old woman with a history of familial multiple CMs, diagnosed at age 18, reported new-onset severe headache followed by blurred vision. MRI showed a hemorrhagic lesion of the optic chiasm and right optic tract. She was 20/20 in each eye with a reported left superior homonymous hemianopia. No intervention was recommended. Vision of the right eye worsened to 20/400 2 months later. The patient was followed over 13 years, and the MRI and visual function remained unchanged. Literature review yielded 87 optic CM cases occurring across gender and nearly all ages with visual loss and headache as the most common presenting symptoms. Optic chiasm is the most common site of involvement (79%). Nearly 95% of reported CM cases were treated with surgery with 81% with improved vision and 1% with worsened vision., Conclusion: MRI features are critical to the diagnosis of optic nerve and chiasm CM and may mimic other lesions. A high index of suspicion by the neuro-ophthalmologist and neuroradiologist leads to early recognition and intervention. Given optic CM displaces and does not infiltrate neural tissue, expedited surgical resection by a neurosurgeon after consideration of other diagnostic possibilities improves visual function in most cases., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by North American Neuro-Ophthalmology Society.)

Background: Optic nerve astrocytomas (ONAs) are neurological neoplasms in the central nervous system (CNS), and they have the highest incidence rate among all the tumor types in the visual pathway. In this study, we conducted a Surveillance, Epidemiology, and End Results (SEER) -based research to explore the demographic, survival, and prognostic factors of patients diagnosed with ONAs., Methods: Utilizing the SEER database, we retrospectively evaluated data of patients diagnosed with ONAs of all ages from 1984 to 2016. We used the Student's t distribution to test variables of patients and various characteristics, and Kaplan-Meier curve to illustrate overall survival (OS) with 95.0% confidence intervals (CIs). We also performed univariate and multivariate analyses to evaluate various variables' validity on overall survival., Results: A total of 1004 cases were analyzed, and revealed that age (P<0.001, hazard ratio (HR) = 8.830, 95% CI: 4.088-19.073), tumor grade (P<0.001, HR = 1.927, 95% CI: 1.516-2.450), diagnostic confirmation (P<0.001, HR = 2.444, 95% CI: 1.632-3.660), and histology type (P = 0.046, HR = 1.563, 95% CI: 1.008-2.424) of the tumor were associated with decreased survival., Conclusions: From this large, comparative study of ONAs, we found that younger age may be considered as a protective indicator, while high-grade astrocytic tumors have a worse prognosis. We also found that diagnostic confirmation and tumor grade were independent prognostic factors in this patient population., (© 2021. The Author(s).)

Optic chiasm cavernous malformations (CMs) are rare. Patients with these CMs typically present with progressive vision loss and are treated via microsurgical resection. We present a middle-aged man with a CM of the optic chiasm and right optic nerve treated via microsurgical resection. We used a minipterional-transsylvian approach. The CM capsule was incised at the lateral surface of the optic chiasm, and the lesion was entered. The malformation was debulked and resected in a piecemeal fashion. A plane was developed circumferentially around the CM borders to separate it from the surrounding neural tissue until removal was complete. At 6-week follow-up, the patient reported stabilized vision in his right eye, and a transient postoperative deficit in his left eye had fully resolved. Postoperative magnetic resonance imaging showed that gross total resection was achieved. Optic apparatus CMs are approached using technical principles similar to brainstem CM resections., (Copyright © 2021. Published by Elsevier Inc.)

Object: Optic pathway tumors (OPT) represent a challenge for pediatric neurosurgeons. Role of surgery is debated due to the high risk of iatrogenic damage, and in lasts decades it lost its importance in favor of chemotherapy. However, in some cases surgery is necessary to make biomolecular and histological diagnosis, to manage intracranial hypertension (IH) and to cooperate with medical therapies in controlling tumor relapse. With the aim to standardize selection of surgical OPT cases, we propose a simple, practical and reproducible classification., Methods: We retrospectively analyzed data of 38 patients with OPT treated at our institution (1990-2018). After careful analysis of MRI images, we describe a new classification system. Group 1: lesion limited to one or both optic nerve(s). Group 2: chiasmatic lesions extending minimally to hypothalamus. Group 3: hypothalamo-chiasmatic exophitic lesions invading the third ventricle; they can be further divided on the base of concomitant hydrocephalus. Group 4: hypothalamo-chiasmatic lesions extending widely in lateral direction, toward the temporal or the frontal lobes. Patients' data and adopted treatment are reported and analyzed, also depending on this classification., Results: Twenty children were operated on for treatment of OPT during the study period. Permanent clinical impairment was noted in 5 (25%) of operated patients, while visual improvement was noted in 1 patient. OS rate was 100% at 5 years, with a median follow up of 9 years (ranging from 2 to 23). Prevalence of intracranial hypertension and proportion of first-line surgical treatment decision were significantly higher in groups 3-4 compared to groups 1-2 (P<0.001 for both tests)., Conclusion: Surgery can offer a valuable therapeutic complement for OPT without major risk of iatrogenic damage. Surgery is indispensable in cases presenting with IH, as in groups 3 and 4 lesions. Eligibility of patients to surgery can be based on this new classification system., (Copyright © 2020 Elsevier Masson SAS. 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.)

Objective: Opto-chiasmatic (OC) cavernous malformations are sporadic lesions that are often misdiagnosed clinically and radiologically. Presenting symptoms range from incidental findings to the more frequent and dramatic "chiasmal apoplexy." The present study aims to evaluate the potential role of arachnoidal membranes of the basal cisterns in the onset of OC apoplexy. A possible mechanism resembling a compartment syndrome is discussed through the description of two cases of bleeding cavernomas., Methods: We describe clinical, radiological, intraoperative findings in two cases of young patients presenting with OC apoplexy from bleeding cavernoma. The first was a 38-year-old man diagnosed with optic neuritis at the first episode of visual acuity deterioration. The second patient was a 22 -year-old woman who suffered two OC apoplexy episodes from a recurrence, which also presented with bleeding., Results: Both patients were operated on via pterional craniotomy and presented a postoperative improvement of visual symptoms. The second patient experienced deterioration 30 months after surgical resection due to rebleeding from a recurrence and required a second operation. Follow-up revealed a good recovery of visual disturbances; MRI at 6 and 3 years showed in both patients an apparent complete removal of the cavernous malformations., Conclusion: The cisternal environment where OC cavernous malformations develop and the paradigm of a compartment syndrome could explain the clinical presentation variability. This very rare subset of cavernomas would benefit from a classification system using ad hoc neuroimaging protocols and consistent indications., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Optic pathway cavernous malformations represent less than 1% of all central nervous system cavernomas. They can lead to visual loss with indeterminate speed, and therefore, the timing of intervention is controversial. We present a patient with an optic nerve cavernoma, which was discovered incidentally 3 years before the onset of visual symptoms. The evolution of her symptoms, visual function and radiographic findings are reported in detail. The cavernoma was eventually removed via a transciliary orbitocranial keyhole approach with the goal to protect the optic chiasm from progressive involvement. The function in the affected optic nerve was not salvageable. This is the second reported case of a cavernoma selectively involving the intracranial portion of the optic nerve. The debate on the timing of intervention is highlighted with reference to the natural history of these rare lesions., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Rationale: Optic Schwannoma is rarely observed clinically as optic nerve had anatomically impossibility for the location of Schwannoma. However, several reports described the established cases of optic Schwannoma, of which the locations were in orbit or within optic canal. The occurrence of optic Schwannoma intracranially has been not reported., Patient Concerns: A 60-year-old female complained of visual impairment in the right eye and the frequent headache and the dizziness over 2 years with unknown reasons. The result of magnetic resonance imaging (MRI) showed a round, well-circumscribed, heterogeneously enhancing signal with cystic change displayed on the right suprasellar cistern., Diagnoses: Intracranial optic Schwannomas., Interventions: The patient accepted microneurosurgery assisted by endoscopy. We observed a gray and yellow lesion located near the right anterior clinoid process with a mid-sized cyst. And there was a vague boundary between the tumor and the right optic nerve which was compressed by the tumor. Optic chiasm and left optic nerve were also compressed. Meanwhile, the tumor had also adhesion to the right anterior cerebral artery (ACA)., Outcomes: After the tumor was totally resected, the patient had satisfactory recovery., Lessons: We reported an intracranial optic Schwannoma removal with the lateral supraorbital keyhole approach assisted by neuroendoscopy. Intracranial optic Schwannoma was rarely seen clinically. Neuroendoscopy imaging suggested the close relationship between the tumor and ACA, supporting vasculature-origin hypothesis for the optic Schwannoma.

Introduction: Aim of the present study is to present an historical overview of the surgical techniques introduced for the treatment of the cancer of the optic nerve and orbit. Methods: From the late 19th and early twentieth century there were introduced many novel surgical techniques for the treatment of the cancer of the optic nerve and orbit. The majority of these techniques concerned for the cure of orbital malignance, while optic nerve cancer many times was treated by enucleation of the eyeball. Results: Except of the extraction of the malignant tumors the surgeons of the time tried to present the best aesthetic result, fact which points the progress of the surgery those years. Conclusions: Many of the techniques introduced those years are still in use.

Purpose: Optic nerve invasion is an important cause of mortality in retinoblastoma. We aimed correlate preoperative imaging and surgical histopathology findings in enucleated eyes with retinoblastoma to determine the efficacy of preoperative imaging in predicting optic nerve invasion in retinoblastoma., Methods: A retrospective review of records of all patients undergoing primary enucleation for retinoblastoma at a tertiary eyecare system between March 2013 and December 2017 with all patients having undergone preoperative imaging, either CT scan or MRI. Data was analyzed statistically to determine the correlation between preoperative CT scan/MRI and histopathology., Results: Totally, 97 eyes of 97 patients were included in the study who underwent primary enucleation for unilateral retinoblastoma. The average age at presentation was 27.8 months with the chief complaint being leukocoria in all the cases. 14 patients (14.43%) had evidence of optic nerve involvement in preoperative imaging. 30 patients had optic nerve invasion on histopathology (laminar and retrolaminar). Spearman's rank correlation test revealed a significant correlation between MRI findings and HP and an insignficant correlation between CT findings and HP. The CT scan had a sensitivity of 20%, specificity of 88.89%, 50% positive predictive value and 66.67% negative predictive value. MRI had a sensitivity of 40%, specificity of 93.55%, positive predictive value of 66.67% and a negative predictive value of 82.86%., Conclusion: MRI showed significant moderate correlation with surgical histopathology for predicting optic nerve invasion in retinoblastoma whereas CT shows insignificant correlation with HPE. Therefore, we recommend MRI for predicting optic nerve invasion in cases of retinoblastoma., Competing Interests: None

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.)

Aims : To report clinicopathological characteristics of two patients with optic nerve sheath schwannoma (ONSS) and review the literature. Method : The first patient (22-year-old man) presented with left eye proptosis and decreased vision in 2012 whose orbital imaging showed a large cystic lesion around the optic nerve. The second patient (52-year-old man) presented with decreased vision in the left eye (without proptosis) in the 2006. His imaging showed a small orbital apex lesion between the medial rectus and optic nerve. Both lesions were histopathologically consistent with ONSS. ONSS has previously been reported in 12 patients. Results : Orbital biopsy and subsequently external beam radiotherapy were performed for the first patient who showed a temporary improvement of vision. However, his proptosis progressed and vision decreased to light perception (LP) a few months after radiotherapy when the lesion was resected in April 2013. Second patient declined any procedure until his vision gradually decreased to LP in October 2007. It was then removed through a combined medial and lateral orbitotomy procedure. Both patients ended up with visual acuity of no LP and no recurrence in their last follow up visits in April 2018. No histopathological evidence was found to show that optic nerve sheath could be the origin for presenting and previously reported cases in the literature. Conclusion : Presenting cases and literature review imply that ONSS is a misnomer and all the lesions are different types of orbital schwannomas with optic nerve sheath attachment.

Background: Hemangioblastomas of the optic nerve are very rare tumors. They occur in association with Von Hippel-Lindau (VHL) syndrome; however, sporadic occurrences have been reported. We describe here a case of optic nerve hemangioblastoma in the absence of VHL and review the pertinent literature., Case Description: A 33-year-old woman presented with gradually progressive vision loss in the right eye. On examination, the visual acuity on the right was hand movement close to face in all quadrants. Color discrimination was impaired. Fundoscopy revealed optic atrophy and no other retinal pathology. There was relative afferent pupillary defect in the right eye. No neurocutaneous markers were found. Imaging revealed lesion isointense on T1, hyperintense on T2/fluid-attenuated inversion recovery, and showing relatively homogenous enhancement on postcontrast study. Multiple flow voids were seen in the intracranial part of the lesion. The proximal part of the intraorbital right optic nerve was enlarged and tortuous with distended optic nerve sheath. A right single-piece fronto-orbital craniotomy was done. A reddish lesion seen involving the right optic nerve just proximal to the chiasm with multiple vessels and a distinct feeding vessel was seen supplying the tumor. The lesion was excised and the optic nerve was sacrificed approximately 1 cm proximal to the chiasm. The postoperative course was uneventful., Conclusions: Conclusions: Optic nerve hemangioblastoma is a rare occurrence and a high level of suspicion is required preoperatively in the absence of VHL syndrome., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Optic pathway gliomas (OPGs) are relatively rare, and their presentation after the first decade of life is even less common. Although many treatment options exist, surgery is typically reserved for tumors significantly compressing surrounding structures. Pregnancy can complicate the management of these tumors, as fetal developmental considerations limit the ways in which they are imaged and treated., Case Description: In this report we detail the case of a 27-year-old pregnant woman who was found to have an OPG during her third trimester. After a decline in this patient's vision and clinical status, a decision was made to induce labor at 31 weeks so that her disease could be more thoroughly addressed., Conclusions: While OPGs are typically benign tumors, pregnancy complicates their management significantly. Contrast media and anesthesia pose significant risks to the fetus, while pregnancy may contribute to increased rates of tumor growth and clinical deterioration. Managing OPGs in pregnant patients thus requires balancing the risks to the fetus and patient., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Although orbital tumors involving the optic nerve are rare, it is well-known that they are very likely to cause serious visual impairment in a patient. Unfortunately, at present, there are no effective interventions that can reliably preserve visual function while controlling tumor growth into intracranial spaces. To ensure visual function of the non-affected side, transection of the optic nerve together with the tumors involved is necessary in some cases. For this procedure large craniotomy and orbital unroofing are commonly utilized. As an alternative, we propose a novel surgical intervention for transection of the optic nerve having optic nerve tumors, which utilizes a lateral orbitotomy approach. To evaluate the invasiveness of different surgical approaches, we compared the days of hospitalization after surgery across patients who underwent the transcranial, lateral, and anterior approaches, respectively. We successfully removed 2 optic nerve tumors using the lateral approach, which required significantly shorter hospitalization than the transcranial approach. The transection of the optic nerve together with tumor removal by the lateral approach may be one of the novel surgical interventions for optic nerve tumors as this method is considerably less invasive than the transcranial removal method., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

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.

Mesenchymal chondrosarcoma (MCS) is a rare tumor in the orbit. Although optic nerve displacement is a common finding in intraorbital MCS, optic nerve tissue involvement in tumor has rarely been reported in huge tumors associated with intracranial extension. Herein the authors report a patient with MCS involving optic nerve tissue without intracranial extension. A 59-year-old woman with a 2-month history of progressive proptosis and normal vision presented to us. Computed tomography revealed a clearly outlined heterogeneous mass with calcified foci in its center, which was attached to the optic nerve, magnetic resonance imaging showed the mass to be isointense to gray matter on T1- and T2-weighted images. She underwent lateral orbitotomy and partial tumor excision. Histopathologic study confirmed MCS. She refused exenteration till 1 year but the tumor recurred and her vision decreased to no light perception. Then exenteration was performed with obtaining free margin and she is now free of tumor after 6 months without radiotherapy or chemotherapy. Mesenchymal chondrosarcoma must be differentiated from more common calcified tumors attached to optic nerve like meningioma.

Background: Tumors extending into the optic canal can cause progressive visual impairment because of optic nerve compression. Prompt surgical resection is often necessary. When the tumor is located medially in the optic canal, endoscopic transnasal surgery provides a safer, less invasive alternative to a transcranial approach., Case Description: We recently encountered a case of small solitary fibrous tumor in the optic canal causing rapid visual deterioration. The radiographic findings of preoperative imaging studies were compatible with those of meningioma; however, unlike meningioma, bleeding from the tumor was profuse during the operation. The endoscopic transnasal approach was effective for handling the highly vascularized tumor in this delicate region, and gross total removal was achieved with postoperative gradual improvement in his visual function. Nevertheless, the tumor recurred after 6 months, and re-resection was performed using the same surgical corridor, followed by adjuvant radiotherapy., Conclusions: Endoscopic transnasal surgery is a valuable option for aggressive lesions in the optic canal. Although the efficacy of radiotherapy for solitary fibrous tumor remains controversial, it should be considered when the tumor shows progressive features., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Optic pathway hypothalamic gliomas are intrinsic low-grade gliomas involving the optic nerve, chiasm, optic tract, and hypothalamus. The rarity of these tumors and their unpredictable course make assessment and standardization of treatment modalities difficult. Tumor debulking via various transcranial approaches was considered to be effective at controlling tumor growth, but with high rates of severe surgery-related complications. In the present case, endoscopic transsphenoidal surgery was initiated to debulk the exophytic chiasmatic/hypothalamic glioma with good preservation of hypothalamic and endocrine functions. The authors suggest transsphenoidal surgery with tumor debulking could be an effective and safe treatment for patients with chiasmatic/hypothalamic gliomas.

Optic nerve Schwannoma is a very rare tumour described in literature. The rarity of this tumour is due to the fact that the optic nerve is myelinated by oligodendrocytes. We present a case of an ancient optic nerve schwannoma in a 16 year old girl who presented to the clinic with right sided proptosis and bilateral loss of vision. She underwent complete excision of the tumour via a craniotomy and histopathology was confirmatory. The various theories explaining the origin of this tumour are discussed along with surgical nuances of removing this tumour. The importance of taking every precaution to preserve vision and avoiding imaging confusion in patients with von Recklinghausen syndrome is also discussed. Only 6 cases of optic nerve schwannomas are described in literature while none have been described in a patient with NF 1.

The objective of this systematic literature review is to assess the role of retinal optical coherence tomography (OCT) in the evaluation of patients with tumors of the visual pathway. We performed a PubMed database search according to the guidelines of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses. The search was restricted to articles published in English between 2000 and 2016, with at least 10 human adult participants enrolled. Twenty-seven articles met the eligibility criteria. All studies investigated tumors of the anterior visual pathway. Both time-domain and spectral-domain OCT technologies were used and the role of OCT as diagnostic and/or prognostic tool was studied. Retinal OCT provides structural information about ganglion cell axon integrity and is complementary to visual function examination. OCT is a prognostic factor for post-operative visual outcome.

PurposeThe lateral compartment of the orbit can readily be accessed through a horizontal lateral canthotomy without the need to swing the lid or remove bone. In this paper the technique, accessible orbital territory, and duration of surgery are presented.Patients and methodsRetrospective, non-interventional descriptive case series for patients who underwent a lateral canthotomy to access pathology within the lateral orbit.ResultsA series of 18 patients are included, all presenting with pathology lateral to, or within, the optic nerve. Pathologies included amyloidosis (1), lymphoma (4), metastatic adenocarcinoma within the optic nerve (1), idiopathic lateral rectus muscle mysositis (4), meningothelial meningioma of the optic nerve (1), intraconal orbital meningioma (1), reactive lymphoid hyperplasia (1), optic nerve glioma (3), optic nerve meningioma (1), and cavernous haemangioma (1). The median surgical time was 36 min (range 23-75 min). No patient required detachment of the lower lid, the technique leaving both upper and lower 'arms' of the lateral canthal tendon attached to Whitnall's tubercle.ConclusionsThe lateral canthotomy approach orbitotomy is a rapid, safe, and minimally disruptive approach for accessing pathology in the lateral orbit and optic nerve. The lateral canthal tendon is split along the horizontal raphe without detachment of either limb from Whitnall's tubercle, no bone is removed, and the post-operative recovery is rapid with minimal associated inflammation or chemosis. This approach is also flexible, permitting the clinician to increase exposure to the orbit peroperatively by swinging the lower lid if required.

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.

This paper reports the case of a patient with optic nerve schwannoma and the first use of neurophysiological intraoperative monitoring of visual evoked potentials during the removal of such tumor with no postoperative visual damage. Schwannomas are benign neoplasms of the peripheral nervous system arising from the neural crest-derived Schwann cells, these tumors are rarely located in the optic nerve and the treatment consists on surgical removal leading to high risk of damage to the visual pathway. Case report of a thirty-year-old woman with an optic nerve schwannoma. The patient underwent surgery for tumor removal on the left optic nerve through a left orbitozygomatic approach with intraoperative monitoring of left II and III cranial nerves. We used Nicolet Endeavour CR IOM (Carefusion, Middleton WI, USA) to performed visual evoked potentials stimulating binocularly with LED flash goggles with the patient´s eyes closed and direct epidural optic nerve stimulation delivering rostral to the tumor a rectangular current pulse. At follow up examinations 7 months later, the left eye visual acuity was 20/60; Ishihara score was 8/8 in both eyes; the right eye photomotor reflex was normal and left eye was mydriatic and arreflectic; optokinetic reflex and ocular conjugate movements were normal. In this case, the epidural direct electrical stimulation of optic nerve provided stable waveforms during optic nerve schwannoma resection without visual loss.

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.

Optic pathway glioma (OPG) encompasses a spectrum of findings ranging from lesions confined to the optic nerve only, lesions affecting the optic chiasm and hypothalamus, and lesions with diffuse involvement of a large part of the optic pathway and neighboring structures. The majority of pediatric low-grade astrocytomas in the optic/chiasmatic region are typical pilocytic astrocytoma. The rest of them (10 %) may be other gliomas such as fibrillary pilomyxoid astrocytoma (grade 2 WHO). The postsurgical local recurrence rate of 55 to 76 % has been reported in some histological subtypes such as pilomyxoid astrocytoma (grade 2). Performing a prechiasmatic transection might offer a new surgical option to avoid further tumor growth toward the chiasm in the optic nerve glioma with predominantly orbital manifestations. In this retrospective study, four patients (three children, two without neurofibromatosis type 1 (NF1), and one with NF1 and one adult without NF1) with optic nerve glioma without involvement of the chiasm but blindness, disfiguring proptosis, and pain of the affected eye were included. The surgical approach was performed as a combined approach from pterional extradural and intradural. 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 between 17 and 106 months. Furthermore, in all patients, gross total tumor resection could be achieved. During follow-up observation in all patients, no further tumor progress or recurrences could be observed. None of the patients were treated postoperatively by radiotherapy or chemotherapy. Prechiasmatic transection of the optic nerve in optic nerve glioma without affecting the chiasm might offer a surgical treatment option to control tumor growth and to preserve vision of the contralateral eye.

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.)

Purpose: To evaluate the impact of surgery on the optic pathway of patients with intra- or parasellar mass lesions, as evidenced by readout-segmented DTI., Materials and Methods: Twenty-four patients with intra- or parasellar mass lesions were included in the study. Readout-segmented DTI and T2WI were obtained before and after surgery. The ROIs were set on the optic chiasm as well as the anterior and posterior optic tracts. For each ROI, axial diffusivity (AD), radial diffusivity (RD), fractional anisotropy (FA), and ADC values were calculated. DTI parameters in preoperative studies of all patients were compared and related to the presence of tumor compression. In patients who underwent surgery, pre- and postoperative DTI parameters were compared. The correlation between DTI parameters and visual function was determined., Results: In the preoperative studies, the optic chiasm of patients with tumor compression showed significant lower AD and RD values. The optic chiasm of patients with visual field disorder showed significantly lower AD and RD values compared to patients without the disorder. There was a negative correlation with a trend toward significance between FA values and visual field disorder scores. The comparative analysis of patients in pre- and postoperative studies showed that the optic chiasm of patients with tumor compression presented a significant lower FA (0.41 versus 0.30, p=0.0068) and higher RD values after surgery., Conclusions: DTI is a useful tool to assess the impact of surgery on the optic chiasm and nerve., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: To retrospectively assess the clinicopathological characteristics of orbital granular cell tumours (GCTs)., Methods: A non-comparative review of the clinical characteristics, imaging, histopathological features, management, and prognosis of five cases of benign GCT and one case of malignant GCT (MGCT) was conducted, along with a review of the English language literature., Results: Among the six cases, four tumours were adherent to the extraocular muscle (EOM), and three tumours to the optic nerve (ON). Morphologic examinations revealed polygonal cells containing periodic-acid-Schiff-positive eosinophilic granules. All tumours (100%) were positive for VIM and NSE, five (83.3%) tumours were positive for S-100, and three (50%) tumours were positive for CD68. The follow-up examination of the MGCT witnessed recurrence and brain metastasis despite several thorough resections, but the patient remained alive; the follow-up examination of the four benign GCTs that had received incomplete excision revealed recurrence in one patient and dramatic shrinkage of the residual tumour in another; there was no recurrence in the other two patients., Conclusions: GCT should be considered in the differential diagnosis of orbital tumours, which may affect EOMs and ON. The natural course of GCT can include tumour progression, stability, or spontaneous regression. To avoid recurrence, complete resection is recommended for orbital GCT. To the best of our knowledge, primary orbital MGCT is reported for the first time.

Traumatic or amputation neuromas are neoformations developing after damage to a peripheral nerve. They are not proper tumors but rather a reactive process or a frustrated attempt of nerve regeneration. Traumatic neuromas are potentially found in every sensory peripheral nerve and often at the site of past surgical intervention, including orbital surgery. A 29-year-old Northern African migrant presented progressive exophthalmos and progressive loss of acuity in left eye, which had started about 6 months before after a cranio-facial trauma caused by a violent assault. MRI of the orbits showed a massive intra-orbital, intra-conical lesion, clearly compressing and dislocating the optic nerve and extending posteriorly to the orbital apex. Surgery was performed through lateral approach of Kroenlein and led to complete excision of the lesion. Histology revealed fibrotic, adipose and striated muscle tissues, a disordered, non-neoplastic overgrowth of small and large fascicles of nerves, inflammatory infiltrates, and fibrosis with sparse calcifications were diffusely observed in a background of fat, scar and striated muscle tissued. Patient was discharged on the fifth day in good health condition, without deficit of eye motion but without recovery of visual acuity. In conclusion, this case demonstrates that traumatic neuromas may arise in the orbit in patients with minor direct trauma to nerves and without previous surgical treatment.