An Overview of Optic Pathway Glioma With Neurofibromatosis Type 1: Pathogenesis, Risk Factors, and Therapeutic Strategies.

Optic pathway gliomas (OPGs) are most predominant pilocytic astrocytomas, which are typically diagnosed within the first decade of life. The majority of affected children with OPGs also present with neurofibromatosis type 1 (NF1), the most common tumor predisposition syndrome. OPGs in individuals with NF1 primarily affect the optic pathway and lead to visual disturbance. However, it is challenging to assess risk in asymptomatic patients without valid biomarkers. On the other hand, for symptomatic patients, there is still no effective treatment to prevent or recover vision loss. Therefore, this review summarizes current knowledge regarding the pathogenesis of NF1-associated OPGs (NF1-OPGs) from preclinical studies to seek potential prognostic markers and therapeutic targets. First, the loss of the NF1 gene activates 3 distinct Ras effector pathways, including the PI3K/AKT/mTOR pathway, the MEK/ERK pathway, and the cAMP pathway, which mediate glioma tumorigenesis. Meanwhile, non-neoplastic cells from the tumor microenvironment (microglia, T cells, neurons, etc.) also contribute to gliomagenesis via various soluble factors. Subsequently, we investigated potential genetic risk factors, molecularly targeted therapies, and neuroprotective strategies for tumor prevention and vision recovery. Last, potential directions and promising preclinical models of NF1-OPGs are presented for further research. On the whole, NF1-OPGs develop as a result of the interaction between glioma cells and the tumor microenvironment. Developing effective treatments require a better understanding of tumor molecular characteristics, as well as multistage interventions targeting both neoplastic cells and non-neoplastic cells.

Treatment of Meningiomas Involving the Optic Canal with Endoscopic Transnasal Decompression of the Optic Nerve.

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.

Gross-total resection in optic nerve sheath meningiomas: minimally invasive and cosmetic pleasing.

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.

Endoscopic Excision of Optic Nerve Schwannoma.

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.

[Orbital tumors]. / Tumoren der Orbita.

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

The treatment efficacy of radiotherapy for optic nerve sheath meningioma.

OBJECTIVES: Optic nerve sheath meningioma (ONSM) is a rare benign tumour that accounts for approximately 2% of all orbital tumours. Radiotherapy has gradually become an important treatment for ONSM because of its good effect in preserving or improving vision. We aimed to explore the effect of radiotherapy on tumour control and vision preservation/improvement in patients with ONSM. METHODS: Forty-three patients with primary ONSM treated in our institution from 2015 to 2021 were enrolled. The irradiation dose was from 50.4 to 54 Gy with 28-30 fractions. We evaluated the tumour volume on MRI or CT, and visual acuity before and after the radiotherapy. RESULTS: Thirty-four patients (79%) experienced a vision decrease at diagnosis. The mean duration of follow-up was 54.1 months (ranges: 18-93, median: 56). Among 25 patients who had tumour evaluation using MRI, 16 patients (37.2%) showed stable tumours, 7 patients (16.3%) had tumour shrinkage, but 2 patients (4.7%) experienced tumour progression. Among the 39 patients performing vision acuity evaluation, 16 patients (37.2%) had vision improvement or recovery. 16 of the 23 patients without vision improvement demonstrated severe visual loss at diagnosis. Two patients had evidence of tumour progression during the follow-up. Additionally, 4 (10.2%) patients had dry eyes, 7 (17.9%) patients experienced watery eyes, and 3 (7.7%) patients had eye swelling. Patients with vision loss for more than 12 months had a lower possibility of vision recovery than those with vision loss for less than 12 months. CONCLUSIONS: Radiotherapy such as IMRT, VMAT, and 3D-CRT plays an important role in the treatment of ONSM. The probability of vision recovery is lower in patients with severe vision loss at diagnosis or the duration of vision loss is more than 12 months.

Visual Outcomes Following Surgical Debulking in Exophytic Optic Nerve Sheath Meningiomas.

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.

Baseline and Post-NACT Imaging in Retinoblastoma With Optic Nerve Involvement: Can MRI Predict Prognosis?

PURPOSE: To describe a staging system for optic nerve invasion using magnetic resonance imaging (MRI) and report any correlation with survival outcome. METHODS: This was a ambispective study. Twenty-one patients with retinoblastoma who had optic nerve involvement on MRI were staged at baseline based on contrast enhancement and/or thickening and length of involvement. Response to neoadjuvant chemotherapy (NACT) was noted according to proposed response evaluation criteria and results were correlated with survival outcome. RESULTS: Baseline MRI staging was able to predict event-free survival (EFS) (P = .0015) using the log-rank test for trends. Patients with optic nerve enhancement alone showed 100% survival prognosis. Optic nerve thickening cases with complete or partial response to NACT showed better EFS (P > .90) than those with stable disease according to response evaluation criteria. CONCLUSIONS: The modified staging system for optic nerve invasion used in the current study significantly predicted EFS. The study also showed that response to NACT may be affected by baseline staging. The authors recommend that cases with optic nerve enhancement only, irrespective of the length of involvement (stage 0), may be treated with upfront enucleation. Cases with optic nerve thickening may be staged to evaluate the correlation with survival outcome in a larger cohort in future studies. [J Pediatr Ophthalmol Strabismus. 2024;61(2):98-105.].

Pediatric Optic Pathway Gliomas: A Report From Northern Greece.

Optic pathway gliomas (OPGs) are the most common pediatric optic nerve tumors. Their behavior ranges between rapid growth, stability, or spontaneous regression. Τhey are characterized by low mortality albeit with significant morbidity. We present the characteristics, management, and outcome of 23 OPG patients (16 females, median age: 4.8 y) managed in a Pediatric Oncology Department in Northern Greece over a 25-year period. Overall, 57% had a background of neurofibromatosis type 1. Diagnosis was based on imaging (10 patients) or biopsy (13 patients). Presenting symptoms were mostly visual impairment/squint (52%). Proptosis/exophthalmos, raised intracranial pressure, and headache were also noted. In 2 occasions, it was detected with surveillance magnetic resonance imaging in the context of neurofibromatosis type 1. Eight patients had unilateral and 2 bilateral optic nerve tumors (Modified Dodge Classification, stage 1a/1b), 3 had chiasmatic (stage 2a/b), and 10 had multiple tumors (stage 3/4). Predominant histology was pilocytic astrocytoma (77%). Management included: observation (4), chemotherapy only (9), surgery only (3), or various combinations (7). Chemotherapy regimens included vincristine and carboplatin, vinblastine, or bevacizumab with irinotecan. Most patients demonstrated a slow disease course with complete response/partial response to chemotherapy and/or surgery, whereas 39% presented ≥1 recurrences. After a median follow-up of 8.5 years (range to 19 y), 20 patients (87%) are still alive with stable disease, in partial/complete remission, or on treatment.

An unusual presentation of bilateral optic pathway glioma in Crouzon Syndrome.

Crouzon Syndrome is a genetic craniosynostosis disorder associated with a high risk of ophthalmologic sequelae secondary to structural causes. However, ophthalmologic disorders due to intrinsic nerve aberrations in Crouzon Syndrome have not been described. Optic pathway gliomas (OPGs) are low grade gliomas that are intrinsic to the visual pathway, frequently associated with Neurofibromatosis type 1 (NF-1). OPGs involving both optic nerves without affecting the optic chiasm are rarely seen outside of NF-1. We report an unusual case of bilateral optic nerve glioma without chiasmatic involvement in a 17-month-old male patient with Crouzon Syndrome without any clinical or genetic findings of NF-1. This case suggests that close ophthalmologic follow up and orbital MRIs may benefit patients with Crouzon Syndrome.

[Clinical and imaging characteristics of optic nerve tumors as the differencial diagnosis of optic neuritis].

Objective: To investigate the clinical and imaging features of optic nerve tumors that require differential diagnosis from optic neuritis. Methods: A retrospective case series study was conducted. Clinical data of patients diagnosed with optic nerve tumors from January 2017 to December 2021 at the Second Affiliated Hospital of Zhejiang University School of Medicine were collected. A total of 29 patients (39 eyes) with clinical and magnetic resonance imaging (MRI) findings similar to optic neuritis or optic neuropathy were included. There were 15 cases of optic nerve sheath meningioma (ONSM) (17 eyes), 4 cases of optic nerve glioma (ONG) (5 eyes), and 10 cases of infiltrative optic nerve lesions (ION) (17 eyes). All patients underwent best-corrected visual acuity (BCVA), anterior and posterior segment examinations, visual field examination, and orbital or cranial MRI examination. Patient data were observed and analyzed, treatment and follow-up information were recorded, and clinical and imaging features were summarized and compared with those of optic neuritis or optic neuropathy. Results: Among the 29 patients with optic nerve tumors, 10 were male and 19 were female, with an average age of (43.3±13.8) years and a range of 11 to 72 years. The follow-up time was 6.8 (2.0, 11.0) months, with a range of 1 to 33 months. Sixteen patients (21 eyes) with optic nerve tumors were initially misdiagnosed as having acute optic neuritis and showed poor response to steroid treatment. Of these, 9 cases (11 eyes) were ONSM, 4 cases (6 eyes) were ION, and 3 cases (4 eyes) were ONG. The diagnostic delay time was 7.1 (1.5, 12.0) months, with a range of 1 to 24 months. The main clinical symptoms of all affected eyes were acute vision loss in 23 eyes, slow vision loss in 5 eyes, transient blackouts in 4 eyes, and no obvious visual symptoms in 7 eyes. The median BCVA of all affected eyes was 0.1, ranging from light perception to 1.0. Fundus examination results showed optic disc edema in 34 eyes and normal optic disc in 5 eyes among the 39 eyes with optic nerve tumors. A total of 27 patients (33 eyes) completed visual field examinations, which revealed an enlarged physiological blind spot in 11 eyes, a concentric or tubular visual field contraction in 8 eyes, a diffuse decrease in light sensitivity in 7 eyes, an arcuate scotoma in 4 eyes, and a normal visual field in 3 eyes. All affected eyes completed orbital or cranial MRI examinations, which showed mild optic nerve thickening in 22 eyes, significant thickening in 6 eyes with distortion, and no significant thickening in 6 eyes. Contrast-enhanced T1-weighted imaging (T1WI) MRI showed optic nerve parenchymal thickening in 5 eyes, all of which were ONG, and 2 of them had optic nerve parenchymal enhancement. Optic nerve sheath thickening and enhancement without optic nerve parenchymal thickening or enhancement were observed in 28 eyes, including 17 eyes of ION and 11 eyes of ONSM. There were 6 eyes with no obvious optic nerve thickening, which were all ONSM, showing mild or significant thickening and enhancement of the optic nerve sheath without optic nerve parenchymal thickening or enhancement. Conclusions: Optic nerve tumors can present with ophthalmic clinical features similar to optic neuritis, such as optic disc edema, and demonstrate MRI findings that resemble those of optic neuritis. Therefore, differentiation between the two is crucial, based on differences in their natural course and response to steroid therapy.

A Patient With Exophytic Primary Optic Nerve Sheath Meningioma: Stratified Risk of Surgery Based on the Morphology.

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.

Medición de las velocidades de flujo sanguíneo cerebral, desviación de la línea media y diámetro de la vaina del nervio óptico mediante ecografía en pacientes sometidos a craneotomía electiva: Estudio observacional prospectivo / Assessment of cerebral blood flow velocities, brain midline shift and optic nerve sheath diameter by ultrasound in patients undergoing elective craniotomy: A prospective observational feasibility study

Introducción: La ecografía cerebral permite valorar las velocidades del flujo sanguíneo cerebral (VFSC), la desviación de la línea media (DLM) y el diámetro de la vaina del nervio óptico (DVNO). La literatura es escasa en determinar la viabilidad de realizar dichas medidas, de forma conjunta en el perioperatorio, en pacientes programados para craneotomía electiva. Métodos: Evaluamos las VFSC de forma bilateral con sus índices compuestos, la DLM y el DVNO por medio de ultrasonido cerebral en pacientes programados para craneotomía electiva antes de la inducción anestésica, en la extubación inmediata, a las seis y 24 horas posoperatorias. El objetivo fue evaluar la viabilidad del uso de la ecografía cerebral en pacientes sometidos a craneotomía electiva y describir los cambios de estas mediciones en diferentes momentos con respecto a los valores basales. Resultados: Fueron incluidos 16 pacientes en el estudio, de los cuales dos se excluyeron del análisis debido a una mala ventana ecográfica. No hubo cambios a lo largo del estudio con respecto a las VFSC, tampoco en la DLM o en el DVNO. Todos los parámetros se mantuvieron dentro de los rangos fisiológicos sin variaciones significativas durante el procedimiento. No hubo complicaciones perioperatorias. Conclusiones: Los resultados de nuestro trabajo muestran la factibilidad de realizar una valoración perioperatoria de las VFSC, DLM y DVNO de forma conjunta y exitosa para obtener información de la hemodinámica cerebral basal en pacientes programados para craneotomía electiva y valorar sus cambios durante las primeras 24 horas del posoperatorio. Son necesarios estudios con mayor número de pacientes para evaluar la eficacia del ultrasonido cerebral como herramienta de monitorización neurológica perioperatoria.(AU)

Background: Brain ultrasound allows measuring the cerebral flow velocity, brain midline shift and optic nerve sheath diameter. Literature is scarce in determining the feasibility to perioperatively perform these measurements altogether and the cerebrovascular behavior in patients scheduled for elective craniotomy. Methods: We assessed bilateral cerebral flow velocities, composite index, brain midline shift and optic nerve sheath diameter by cerebral ultrasound in patients scheduled for elective craniotomy before anesthetic induction, at extubation, and at 6 and 24 hours after. The aim was to assess the feasibility of brain ultrasound in patients for elective craniotomy and to describe the changes in cerebral flow velocities, brain midline shift and optic nerve sheath diameter from baseline values at different times in the postoperative period. Results: Sixteen patients were included, of these two were excluded from analysis due to an inadequate sonographic window. There were no changes throughout the study regarding cerebral flow velocity, brain midline shift nor optic nerve sheath diameter assessments. All parameters were maintained in the physiological range without significant variations during the procedure. No perioperative complications were detected. Conclusions: The results of our study show the feasibility to perform a perioperative assessment of cerebral flow velocity, brain midline shift or optic nerve sheath diameter jointly and successfully to obtain additional information of baseline cerebral hemodynamics in patients scheduled for elective craniotomy and their postoperative changes during the first 24 hours. Future studies with lager samples are needed to address the efficacy of cerebral ultrasound as a monitoring tool.(AU)

Radiation optic neuropathy and retinopathy in patients with presumed benign intraorbital tumours treated with fractionated stereotactic radiotherapy.

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.

Precocious and accelerated puberty in children with neurofibromatosis type 1: results from a close follow-up of a cohort of 45 patients.

PURPOSE: Central precocious puberty (CPP) in neurofibromatosis type 1 (NF1) occurs mainly in association with optic pathway glioma (OPG), but it can also develop in the absence of OPG. The aim of this study was to analyze the prevalence of puberty disorders in children with NF1 and its association with OPG and its location. METHODS: A retrospective study of 45 children with NF1 (68.9% boys) followed at our center between 2008 and 2020 was conducted. A cerebral MRI scan was performed in all children. We analyzed auxological, laboratory, and imaging data of children with CPP or accelerated puberty (AP). Treatments used for CPP/AP and their effect on height were also evaluated. RESULTS: The prevalence of puberty disorders in our cohort was 17.8% (male to female ratio of 7:1). CPP and AP were diagnosed in 8/45 (17.8%) NF1 children. Among children with puberty disorders, 5/8 (62.5%) had an OPG with chiasm involvement, 1/8 (12.5%) had an isolated optic nerve tumor, and 2/8 (25%) did not have any evidence of OPG on MRI. Fisher's exact test showed an association between CPP/AP and chiasm OPG (p = 0.025). Treatment with triptorrelin was initiated in 5/8 children, of whom four attained final predicted height. CONCLUSION: Our study confirms the higher prevalence of CPP/AP in NF1 patients, as well as an association between chiasm OPG and puberty disorders. However, CPP/AP also occurred in the absence of OPG with an incidence of 9.1%. Comprehensive evaluation of every child with NF1 regardless of the presence of OPG is therefore essential.

[Results of radiation therapy for optic nerve sheath meningioma]. / Rezul'taty luchevogo lecheniya meningiomy obolochki zritel'nogo nerva.

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

Primary Optic Disc Tumors - Case Series and Literature Overview. / Primäre Papillentumoren ­ Fallserie und Literaturüberblick.

PURPOSE: Primary optic disc tumors are often a challenge for ophthalmologists. They have very different appearances, and many primary optic disc tumors are associated with syndromic diseases (especially phakomatoses). Because of the rarity of primary optic disc tumors, classification and assessment are often difficult. MATERIAL AND METHODS: A systematic search in the electronic patient files (period 01.01.2015 - 01.06.2022) of the Department of Ophthalmology of the University of Münster Medical Center for patients with primary optic disc tumors was performed. For each tumor entity, exemplary cases were selected, which are presented here in detail. The criteria for the exemplary case selection were a clear diagnosis, the presence of suitable image material and follow-up examinations in our clinic. RESULTS: The search yielded seven cases with three different primary tumor entities in the optic disc region (capillary hemangioblastoma, astrocytic hamartoma and melanocytoma). Four patients were selected as examples and are presented here: two cases for capillary hemangioblastoma (one isolated and the other in the context of Von-Hippel-Lindau syndrome) and one case each for astrocytic hamartoma and melanocytoma). We outline the further diagnosis and the course of the disease and we give an overview of the essential features of the underlying tumors in each case. CONCLUSION: The knowledge of the different primary tumors of the optic disc is necessary for a correct diagnosis and for the differentiation from malignant processes and optic disc anomalies. In many cases, further interdisciplinary diagnostics are necessary. Multimodal imaging is helpful and a referral to a center for ocular tumors is worth considering.