[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.

[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.

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.

Evaluación multimodal de un «micromelanocitoma» del disco óptico: un reporte de caso / Multimodal imaging assessment of a “micro” optic disk melanocytoma: A case report

Un melanocitoma del disco óptico es una lesión neoplásica pigmentada y benigna que en promedio mide 2,00mm de diámetro. Melanocitomas del disco óptico más pequeños son raros y no han sido estudiados con múltiples técnicas de imagen. Un caso de un «micromelanocitoma» del disco óptico con un diámetro de 0,71mm fue estudiado con múltiples estudios de imagen, incluyendo autofluorescencia de fondo de ojo, ultrasonido ocular, campos visuales, tomografía de coherencia óptica (OCT) y angiografía por OCT. Mientras que la exploración con biomicroscopia y la autoflourescencia de fondo de ojo revelan cambios compatibles con los casos de tamaño promedio previamente publicados, la OCT, angiografía por OCT y el ultrasonido revelan cambios que difieren de los artículos previos y que podrían ser útiles para el seguimiento (AU)

An optic disk melanocytoma is a benign pigmented neoplasm of the optic disk measuring 2.00mm of diameter in average. Smaller optic disk melanocytomas are rare and have not been studied with multiple imaging studies. A case of a “micro” optic disk melanocytoma measuring 0.71mm in diameter was studied by multiple imaging modalities, including fundus autofluorescence, ultrasound, visual fields, optical coherence tomography (OCT) and OCT angiography. While clinical appreciation and fundus autofluorescence reveal changes compatible with previously reported cases, OCT, OCT angiography and ultrasound reveal specific changes that could be useful for follow up (AU)

Multimodal imaging assessment of a "micro" optic disk melanocytoma: A case report.

An optic disk melanocytoma is a benign pigmented neoplasm of the optic disk measuring 2.00 mm of diameter in average. Smaller optic disk melanocytomas are rare and have not been studied with multiple imaging studies. A case of a "micro" optic disk melanocytoma measuring 0.71 mm in diameter was studied by multiple imaging modalities, including fundus autofluorescence, ultrasound, visual fields, optical coherence tomography (OCT) and OCT angiography. While clinical appreciation and fundus autofluorescence reveal changes compatible with previously reported cases, OCT, OCT angiography and ultrasound reveal specific changes that could be useful for follow up.

Afectación metastásica del nervio óptico en paciente con neoplasia de mama y pulmón / Metastatic involvement of optic nerve in patient with breast and lung neoplasia

Las metástasis son los tumores intraoculares más frecuentes del adulto. Sin embargo, aquellas localizadas en el nervio óptico son muy infrecuentes y suelen asociarse a diseminación en otras localizaciones como el sistema nervioso central, lo que ensombrece el pronóstico.Se presenta un caso de una mujer de 67 años que refiere pérdida de visión progresiva en el ojo derecho de 15 días de evolución. En la exploración oftalmológica se observa un defecto pupilar aferente relativo en dicho ojo y un pseudoedema de papila con hemorragias retinianas en el fondo de ojo. Los antecedentes personales y las características del nervio óptico apuntan al diagnóstico de infiltración metastásica. (AU)

Metastases are the most common adult intraocular tumors. However, those located in the optic nerve are very uncommon and are usually associated with spread to other locations such as the central nervous system, which darkens the prognosis.There is a case of a 67-year-old woman who reports progressive vision loss in the right eye of 15 days of evolution. The ophthalmological examination shows a relative afferent pupil defect in this eye and a pseudoedema of the papilla with retinal hemorrhages in the fundus. Personal history and characteristics of the optic nerve suggest the diagnosis of metastatic infiltration (AU)

Surgical Treatment of Bilateral Optic Nerve Sheath Meningioma: 2-Dimensional Operative Video.

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.

Accuracy of Computed Tomography and Magnetic Resonance Imaging for Detection of Pathologic Risk Factors in Patients Diagnosed with Retinoblastoma.

Aim: To assess and compare the diagnostic accuracy of magnetic resonance imaging (MRI) and computed tomography (CT) for detecting the extent of tumor invasion in eyes with advanced retinoblastoma prior to enucleation using histopathologic analysis as a reference.Methods: A total of 56 patients (68 eyes) enucleated for retinoblastoma were reviewed. Eyes with complete data (CT 28, MRI 16) were included for analysis. Imaging data were reviewed by a pediatric radiologist, blinded to histopathology results. Four high-risk factors which included scleral, choroidal, anterior eye segment invasion, and postlaminar optic nerve invasion were assessed.Results: For CT, the diagnostic odds ratio (DOR), sensitivity, and specificity for detecting postlaminar optic nerve invasion were 1.7%, 25%, and 83.3%. Choroidal invasion was correctly identified in only 2 of 10 eyes (DOR 0.5, sensitivity 20%, specificity 66.7%). For scleral invasion, CT showed a diagnostic ratio of 1.5%, sensitivity 40%, specificity 69.6% whereas for anterior segment invasion DOR was 9.2%, sensitivity 100%, and specificity 65.4%. MRI showed a higher DOR for detecting postlaminar optic nerve invasion of 39%, sensitivity 77.8%, and specificity 100%. Choroidal and scleral invasion on MRI had a DOR of 6.5% (sensitivity 25%, specificity 100%) and 6% (sensitivity 33.3%, specificity 92.3%) respectively while DOR for anterior segment invasion was 1.3% (sensitivity 50%, specificity 57.1%).Conclusion: CT showed a poor diagnostic accuracy for all four high-risk factors in advanced retinoblastoma and therefore may not be a useful tool for assessment of tumor extension. For MRI, detection of postlaminar optic nerve invasion was moderately accurate although less accurate for detection of other risk factors. Decision-making for each child should not be based on any single parameter but rather on consideration of clinical factors in combination with radiologic findings.

Optic pathway tumor in children: Toward a new classification for neurosurgical use.

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.

The role of OCT angiography in a rare case of malignant trasformation of an optic disc melanocytoma.

The melanocytoma is a benign, highly pigmented tumor, localized on or adjacent to the optic disc. Its malignant trasformation represents a rare event. We describe a case of a 43-years old woman returned to our observation 29 years later with a malignant melanoma from an optic disc melanocytoma evaluated by Optical Coherence Tomography Angiography (OCTA). OCTA is a valid, non-invasive, dyeless and reliable method that could improve the diagnosis of this rare case of malignant trasformation of an optic disc melanocytoma.

Microneurosurgical treatment under the guidance of neuroendoscopy for an intracranial optic nerve Schwannoma: A case report.

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.

Management of advanced uni- or bilateral retinoblastoma with macroscopic optic nerve invasion.

BACKGROUND: Retinoblastoma with macroscopic optic nerve (ON) invasion depicted by imaging at diagnosis remains a major problem and carries a poor prognosis. We sought to describe the treatment and outcome of these high-risk patients. METHODS: Retrospective mono-institutional clinical, radiological, and histological review of patients with uni- or bilateral retinoblastoma with obvious ON invasion, defined by radiological optic nerve enlargement (RONE) depicted by computed tomography scan or magnetic resonance imaging (MRI), was performed. RESULTS: Between 1997 and 2014, among the 936 patients with retinoblastoma treated at Institut Curie, 11 had detectable RONE. Retinoblastoma was unilateral in 10 and bilateral in one. Median age at diagnosis was 28 months (range, 11-96). ON enlargement extended to the orbital portion in three patients, to the optic canal in five, to the prechiasmatic portion in two, and to the optic chiasm in one. Nine patients received neoadjuvant chemotherapy and partial response was obtained in all. Enucleation was performed in 10/11 patients-by an anterior approach in three and by anterior and subfrontal approaches in seven. Three patients had a positive ON resection margin (2/3 after primary enucleation). All enucleated patients received adjuvant treatment (conventional chemotherapy: 10, high-dose chemotherapy: seven, radiotherapy: five). Leptomeningeal progression occurred in four patients. Seven are in first complete remission (median follow up: 8 years [3.5-19.4]). CONCLUSION: Neoadjuvant chemotherapy and microscopic complete resection have a pivotal role in the management of retinoblastoma with RONE. MRI is recommended for initial and pre-operative accurate staging. Surgery should be performed by neurosurgeons in case of posterior nerve invasion. Radiotherapy is required in case of incomplete resection.

Progressive sixth nerve palsy secondary to intracavernous arachnoid cyst and complicated by contralateral optic nerve sheath meningioma.

INTRODUCTION: Optic nerve sheath meningiomas and intracavernous arachnoid cysts are both fairly rare conditions, and to the best of our knowledge, have not been previously reported to co-occur in the same patient. Both can cause diplopia, but only ONSMs have been documented to demonstrate progressive worsening of ocular motility. CASE REPORT: A 67-year-old woman with blur and diplopia demonstrated a right optic neuropathy and limited ductions bilaterally. Neuroimaging revealed a right optic nerve sheath meningioma and left intracavernous arachnoid cyst. She was conservatively managed with neurosurgical surveillance for 1.5 years, until her diplopia worsened. Ocular motility re-evaluation demonstrated a worsening left abduction deficit, suggesting interval change of the intracavernous cyst, rather than the meningioma. CONCLUSION: There are only a few reported cases of cranial nerve VI palsy secondary to a cavernous sinus arachnoid cyst. However, this is the first reported case in a patient with a concurrent optic nerve sheath meningioma, and the first case demonstrating progressive worsening of a sixth cranial nerve palsy from an intracavernous arachnoid cyst. Determining which comorbidity caused worsening of symptoms played a critical role in the management of this patient.