Optic Nerve Tumors Research Articles

Choroidal Melanoma With Optic Nerve Infiltration Presenting as Orbital Pseudocellulitis

LEGEND A 60-year-old male presented with (Fig. A) right orbital cellulitis with no perception of light, shallow anterior chamber, mid-dilated and fixed pupil, cataract and intraocular pressure of 62 mm Hg. B-scan ultrasonography (Fig. B) revealed mushroom shaped intraocular mass with choroidal excavation. A final diagnosis of choroidal melanoma with orbital pseudocellulitis was made. The right globe was enucleated after the inflammation was controlled with intravenous corticosteroids. Systemic work-up revealed no evidence of metastasis. Cut section of the enucleated globe (Fig. C) revealed a peripapillary pigmented choroidal tumor. On histopathology, necrotic variant of choroidal melanoma with epithelioid cells was confirmed (Fig. D). The tumor was infiltrating the optic nerve 1 mm beyond the lamina cribrosa. Tumor emboli were noted focally in the scleral blood vessels. Resected end of optic nerve was free of tumor. In this case of choroidal melanoma, presenting feature of orbital pseudocellulitis and optic nerve tumor infiltration were the two unusual features. Orbital pseudocellulitis is a rare presenting feature of choroidal melanoma and can occur in cases with or without extraocular tumor extension. Orbital pseudocellulitis in choroidal melanoma could be related to extensive tumor necrosis or secondary glaucoma, which causes release of inflammatory mediators causing orbital inflammation that responds favorably to systemic corticosteroids. In this case, orbital pseudocellulitis subsided after 48 hours of intravenous corticosteroids. Optic nerve invasion is rare in uveal melanoma and is associated with poor prognosis. The risk factors for optic nerve invasion of choroidal melanoma are elevated intraocular pressure, nonspindle cell type, and juxtapapillary tumor location. All these features were noted in this case.

Blood Flow in Monocular Retinoblastoma Assessed by Color Doppler and Correlations With High-Risk Pathologic Features.

To use color Doppler to analyze blood flow in the retrobulbar central retinal artery (CRA) and central retinal vein (CRV) in monocular retinoblastoma. This prospective study included patients with group D and E retinoblastomas managed with only enucleation. Peak blood velocities were assessed in the CRA and CRV of tumor-containing eyes (CRAv and CRVv, respectively). The resistivity index in the CRA (RIa) and pulse index in the CRV (PIv) were calculated and related to optic nerve invasion (ONi), choroid invasion (mCHi), and tumor volume. RIa and PIv were also calculated for healthy eyes. In total, 25 patients with a mean age of 30.8-months old were included. The means (SD) for CRAv, CRVv, RIa, and PIv were 26.94 (12.32) cm/s, 16.2 (9.56) cm/s, 0.88 (0.12) and 0.79 (0.29), respectively. Tumor volume was significantly correlated with CRAv (P = 0.025) and RIa (P = 0.032). ONi was present in 19 eyes and correlated with a smaller PIv (P < 0.001). A PIv less than 0.935 had a sensitivity of 89.5% and specificity of 83.3% for predicting ONi. mCHi was not correlated with flow values. Healthy eyes had a significantly lower RIa (P < 0.001) and lower PIv than eyes with (P = 0.009) and without (P < 0.001) ONi. In advanced-stage monocular retinoblastoma, tumor volume was directly correlated with CRAv and RIa, and lower PIv was correlated with optic nerve invasion when a predictive cut-off value of less than 0.935 was applied. Comparisons with healthy eyes showed that tumor-containing eyes were associated with higher RIa and PIv values.

A novel surgical approach for intraorbital optic nerve tumors

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.

Choroidal melanoma, simulating an optic nerve tumor: a clinical case

Choroidal melanoma is a primary malignant intraocular tumor with unfavorable vital prognosis. Factors predisposing to invasion of choroidal melanoma into the optic nerve include its juxtapapillary localization, the large tumor size, its diffuse growth, the presence of necrosis in the tumor, increased intraocular pressure and the epithelioid cell type of the tumor. The authors describe the clinical case of a 31-year-old patient with a primary diagnosis of an optic nerve neoplasm. Peripapillary edema and indistinct optic disc margins were found by ophthalmoscopy. There was a slightly protruding gray-slate lesion with indistinct boundaries near the optic nerve disc. The retina was edematous with multiple merging hemorrhages. The data obtained by echography (the focus protruding both inside the eye and along the optic nerve trunk), color Doppler mapping (single own vessels in the projection of the orbital part of the mass) and computed tomography allowed for the suspicion of the primary choroidal melanoma. To clarify the nature of the process, a trans-conjunctival orbitotomy with a revision of the orbit and the optic nerve was performed. The expansion of the proximal part of the optic nerve with partial breakthrough of the optic nerve shells was revealed intraoperatively. The patient underwent enucleation with morphological and molecular genetic verification of the primary choroidal melanoma with invasion of the optic nerve. The data obtained (absence of chromosome 3 monosomy, deletion of the short arm of chromosome 1, methylation of promoter regions of the RASSF1A gene, mutations in the 4 and 5 exons of the GNAQ gene, CT genotype of the polymorphic marker C3435T of the ABCB1 gene) indicate a relatively favorable vital prognosis, despite the extrascleral growth of the tumor and its mixed cell type. The patient underwent a course of anti-relapse proton irradiation of the orbit with a total dose of 58.8 Gy at 80–90% isodose. Currently, 7 years after the treatment, the patient continues to be followed up by ophthalmologists and oncologists, without any signs of local recurrence and distant metastases. This clinical example demonstrates the possibility of extraocular extension of a small intraocular choroidal melanoma simulating an optic nerve tumor, and confirms the need for a thorough clinical and instrumental examination for a correct diagnosis and adequate management of the patient.

Expression of multidrug-resistance associated proteins in human retinoblastoma treated by primary enucleation.

To reveal the expression of multidrug-resistance associated proteins: glutathione-S-transferase π (GSTπ), P-glycoprotein (P-gp) and vault protein lung resistance protein (LRP) in retinoblastoma (RB) without any conservative treatment before primary enucleation and to correlate this expression with histopathological tumor features. A total of 42 specimens of RB undergone primary enucleation were selected for the research. Sections from the formalin-fixed, paraffin-embedded specimens were stained with HE and immunohistochemistry to detect the expression of GSTπ, P-gp and LRP. GSTπ was expressed in 39/42 (92.86%) RBs and in 9/9 (100%) well-differentiated RBs. P-gp/GSTπ was found in 30 (71.42%) of 42 RBs. Totally 9 (21.43%) tumors were well differentiated and 33 (78.57%) were poorly differentiated. Totally 15 (35.71%) eyes had optic nerve (ON) tumor invasion, 36 (85.71%) had choroidal tumor invasion, and 14 (33.33%) had simultaneous choroidal and ON invasion. There was no statistically significant relationship between P-gp, GSTπ, LRP positivity and the degree of ocular layer tumor invasion and ON tumor invasion (P>0.05). RB intrinsically expresses GSTπ, P-gp and LRP. GSTπ expression is positive in 100% well-differentiation ones, so in which way it is correlated with differentiation. But the other two proteins expressions are not related to tumor differentiation and to the degree of tumor invasion. GSTπ may be a new target of chemotherapy in RB.

Long-term Outcomes After Proton Beam Irradiation in Patients With Large Choroidal Melanomas

Although radiotherapy has been used more frequently in past decades for the management of large melanomas, long-term efficacy of proton beam irradiation (PBI) of large choroidal melanomas has not been reported. To evaluate long-term outcomes in patients who underwent PBI for the treatment of large choroidal melanomas. Data were obtained at a single Boston, Massachusetts, academic tertiary referral practice for this retrospective cohort study. In total, 336 patients with large tumors treated over a 13-year period from January 1, 1985, to December 31, 1997, and followed up until the end points were reached or until December 31, 2008, were included. Data analyses were initially completed in February 2017 and finalized in July 2017. Large tumors were those with a height 10 mm or greater or a longest linear diameter greater than 16 mm or a height greater than 8 mm when the optic nerve was involved. Proton beam irradiation (total 70 Gy) delivered in 5 equal fractions. The primary outcomes of rates of visual acuity retention, eye retention, tumor recurrence, and melanoma-related mortality were calculated using Kaplan-Meier estimates, and Cox proportional hazards regression analyses were completed to evaluate risk factors for tumor recurrence and melanoma-related mortality. In this cohort of 336 patients with large tumors, 150 were women and 329 were white; mean (SD) age was 60.0 (14.0) years. Of 178 patients without optic nerve involvement (tumor >1 disc diameter from optic nerve), the mean (SD) largest basal diameter was 18.1 (1.9) mm and mean height was 8.2 (2.7) mm. Optic nerve involvement and tumors greater than 8 mm were observed in 109 patients (32.4% of the cohort). Baseline visual acuity of 20/200 or better was observed in 244 patients (72.6%), and worse than 20/800 in 52 (15.5%). Ten-year rates of visual acuity retention were 8.7% (95% CI, 4.1%-15.6%) for at least 20/200 and 22.4% (95% CI, 15.4%-30.4%) for at least counting fingers. Ten years after PBI therapy, the eye was retained (70.4%; 95% CI, 61.5%-77.6%) and tumor controlled (87.5%; 95% CI, 76.8%-93.5%) in most patients. The 10-year all-cause mortality rate was 60.7% (95% CI, 55.5%-65.9%). Approximately half of the patients died of metastatic uveal melanoma (10-year rate, 48.5%; 95% CI, 43.0%-54.4%). This study demonstrates that eye conservation is possible in most cases, with ambulatory vision retained in a small proportion of patients 10 years after PBI. Tumor recurrence rates were low and mortality rates were comparable to those observed after enucleation.

Unilateral malignant optic glioma following glioblastoma multiforme in the young: a case report and literature review

BackgroundMalignant optic gliomas are rare, but they rapidly become lethal visual pathway tumors. We present the clinical course, treatment, and prognosis of a case of unilateral malignant optic glioma in a young man with a history of brain glioblastoma multiforme (GBM).Case presentationA 21-year-old man, who had GBM 7 years ago complained of a transient shadow in his vision and presented with normal visual acuity but optic disc edema and an enlarged blind spot in the right eye (oculus dexter, OD). Magnetic resonance imaging (MRI) showed a right intraorbital optic nerve tumor without a brain lesion. Chiasm involvement and severe vision deterioration occurred 3 months later. A biopsy of the right optic nerve revealed glioblastoma. Concurrent chemoradiotherapy (CCRT) prevented involvement of the fellow eye 1 year after symptom onset.ConclusionThis report demonstrated that a regular ocular exam should be recommended for several years after GBM. In young healthy patients who are able to undergo chemotherapy and radiotherapy, visual function in the fellow eye can be preserved.

A neurosurgical perspective to approaches to the orbit: A cadaveric study.

Neurosurgeons are getting increasingly involved in surgery for orbital tumours. The various approaches to the orbit can be challenging. The authors have demonstrated on cadavers the various approaches to the orbit which are required to be mastered by the neurosurgeon. The superior approaches which include the transcranial, supraorbital and the hybrid approaches are the most commonly utilized surgical approaches by the neurosurgeon for excision of orbital pathologies. The lateral orbitotomy is an elegant approach for excision of the tumours lateral to the optic Nerve and lacrimal gland tumours. The authors also demonstrate the medial approach through a medial orbitotomy which is a relatively unfamiliar approach to neurosurgeons. The importance of the various landmarks in each approach is emphasised along with the methods to minimize complications. It is imperative that neurosurgeons are intimately familiar with the microsurgical anatomy of the orbit and the various approaches to it.

Optic Nerve Movement May Need to be Considered When Treating with Stereotactic Radiosurgery

Introduction: Fractionated stereotactic radiosurgery (fSRS) is an effective definitive treatment for optic nerve (ON) tumors. It is controversial whether treatment to the ON requires attention to the position of the eye as some consider the movement to be negligible. We investigated in four patients to establish whether there is clinically significant displacement of the ON as the globe moves and the effect this may have on target coverage in fSRS treatments. Method: Four patients receiving radiotherapy treatment to the skull and requiring cone beam computed tomography (CBCT) matching were asked to look in different directions as routine CBCT were obtained. An fSRS plan was created, treating the ON with the patient looking straight ahead. The displacement of the ON from straight was measured, with the patient looking fully left or right, the DVH of the optic nerve in the three locations was compared. Results: The data collected showed that there could be a significant difference in optic nerve position as the patient’s gaze changed. The DVH showed that the PTV coverage was affected as the patient moved their gaze either left or right by up to 9% (95%CI is from -15% to -3%, p=0.012). Conclusion: As a result of our study we recommend cooperative patients receive some training to ensure that optic nerve positioning is maintained throughout simulation and treatment.

OPTIC NERVE INFILTRATION BY RETINOBLASTOMA: Predictive Clinical Features and Outcome.

To identify the clinical features predictive of any optic nerve infiltration and postlaminar optic nerve infiltration by retinoblastoma on histopathology and to report the outcome (metastasis and death) in these patients. Retrospective study. Of the 403 patients who underwent primary enucleation for retinoblastoma, 196 patients had optic nerve tumor infiltration (Group 1) and 207 patients had no evidence of optic nerve tumor infiltration (Group 2). Group 1 included patients with prelaminar (n = 47; 24%), laminar (n = 74; 38%), and postlaminar tumor infiltration with or without involving optic nerve transection (n = 74; 38%). Comparing Group 1 and Group 2, the patients in Group 1 had prolonged duration of symptoms (>6 months) (16% vs. 8%; P = 0.02) and were associated with no vision at presentation (23% vs. 10%; P = 0.01), higher rates of secondary glaucoma (42% vs. 12%; P < 0.0001), iris neovascularization (39% vs. 23%; P < 0.001), and larger tumors (mean tumor thickness, 12.8 mm vs. 12 mm; P = 0.0001). There was a higher prevalence of metastasis in Group 1 than in Group 2 (4% vs. 0%; P = 0.006). On multivariate analysis, clinical features predictive of any optic nerve tumor infiltration secondary glaucoma (hazard ratio = 5.38; P < 0.001) and those predictive of postlaminar optic nerve tumor infiltration included iris neovascularization (hazard ratio = 2.66; P = 0.001) and secondary glaucoma (hazard ratio = 3.13; P < 0.001). In this study, clinical features predictive of any optic nerve tumor infiltration included secondary glaucoma and those predictive of postlaminar optic nerve tumor infiltration included iris neovascularization and secondary glaucoma. Despite adjuvant treatment in those with postlaminar optic nerve tumor infiltration, metastasis occurred in 8% of patients.

Treatment and Outcome in 65 Children with Optic Pathway Gliomas

Optic pathway gliomas (OPGs) are rare neoplasms in children with an unpredictable clinical course. There is significant controversy regarding the optimal management and outcome of these patients. Charts of all patients with OPG diagnosed and treated at Children's Cancer Hospital Egypt between July 2007 and July 2014 were retrospectively reviewed. We evaluated the roles of surgical, ophthalmologic, endocrinologic, neurologic, and treatment aspects of care. Sixty-five patients were included in this study, with a mean age of 5.3 years. OPGs were chiasmatic (n= 25), optic nerve (n= 18), hypothalamic (n= 7), and chiasmatic/hypothalamic (n= 7). Extensive involvement of the optic pathway was seen in an additional 8 patients. Twenty cases had neurofibromatosis type 1. Four cases underwent surgical debulking, and 28 were biopsied (16 open, 11 stereotactic, and 1 endoscopic). Nine of the 18 optic nerve tumors were managed by total excision. Twenty-four patients did not undergo any surgical intervention. Forty-five patients received chemotherapy. Histopathology revealed pilocytic (n= 20), pilomyxoid (n= 15), fibrillary astrocytoma (n= 4), and grade I papillary-glioneuronal tumor (n= 1). Nonrepresentative sample (n= 1). The 4-year overall survival rate was 86.3% with mean follow-up period of 32.2 months. The initial role of surgery in newly developed OPG is biopsy for tissue diagnosis and relief of the hydrocephalus, if present, followed by chemotherapy. Chemotherapy decreases or stabilizes the tumor size in most cases, leading to preservation of both visual and endocrinal functions. The most significant prognostic factor confirmed in this study was the age of the patient.

Feasibility of Proton Beam Therapy for Ocular Melanoma Using a Novel 3D Treatment Planning Technique

We evaluated sparing of normal structures using 3-dimensional (3D) treatment planning for proton therapy of ocular melanomas. We evaluated 26 consecutive patients with choroidal melanomas on a prospective registry. Ophthalmologic work-up included fundoscopic photographs, fluorescein angiography, ultrasonographic evaluation of tumor dimensions, and magnetic resonance imaging of orbits. Three tantalum clips were placed as fiducial markers to confirm eye position for treatment. Macula, fovea, optic disc, optic nerve, ciliary body, lacrimal gland, lens, and gross tumor volume were contoured on treatment planning compute tomography scans. 3D treatment planning was performed using noncoplanar field arrangements. Patients were typically treated with 3 fields, with at least 95% of planning target volume receiving 50 GyRBE in 5 fractions. Tumor stage was T1a in 10 patients, T2a in 10 patients, T2b in 1 patient, T3a in 2 patients, T3b in 1 patient, and T4a in 2 patients. Acute toxicity was mild. All patients completed treatment as planned. Mean optic nerve dose was 10.1 Gy relative biological effectiveness (RBE). Ciliary body doses were higher for nasal (mean: 11.4 GyRBE) than temporal tumors (5.8 GyRBE). Median follow-up was 31 months (range: 18-40 months). Six patients developed changes which required intraocular bevacizumab or corticosteroid therapy, but only 1 patient developed neovascular glaucoma. Five patients have since died: 1 from metastatic disease and 4 from other causes. Two patients have since required enucleation: 1 due to tumor and 1 due to neovascular glaucoma. 3D treatment planning can be used to obtain appropriate coverage of choroidal melanomas. This technique is feasible with relatively low doses to anterior structures, and appears to have acceptable rates of local control with low risk of enucleation. Further evaluation and follow-up is needed to determine optimal dose-volume relationships for organs at risk to decrease complications rates.

Quantification of Intraorbital Organ Motion Supports Fixed Gaze Treatment for Optic Nerve Tumors

Quantification of Intraorbital Organ Motion Supports Fixed Gaze Treatment for Optic Nerve Tumors

Blindness in Tuberous Sclerosis: A Case Report

Tuberous sclerosis (TS) is inherited as an autosomal dominant trait with variable penetrance characterised by glial cell tumor which arises from the cerebral and the retina. Blindness in association with Tuberous sclerosis (TS) is rare. When visual loss occurs it may be associated with hamartomas from retinal or optic nerve involvement or from intracranial (brain) tumours that affect either the part of the brain that processes visual information or from optic nerve damage following raised intracranial pressure. Very few cases of TS with blindness have been reported globally. Deterioration in academic performance might be the first pointer to the visual impairment. We report a case of a 13 year old girl who presented with increasing number of facial rash over an 11 years period, recurrent headache and deteriorating academic performance of 1 year and loss of vision of 6 months with a recent episode of convulsion. Similar skin rashes without other associated symptoms were noticed on the mother and one of the younger siblings. She was a Tanner stage one in development. She had facial angio fibromas, shagreen patches over the left hypochondria, back regions and face. Ophthalmic evaluation showed a visual acuity of being able to count fingers at not more than one meter from the face and only perception of light in the right and left eye respectively, both eyes had brisk pupillary activities, good mydriasis and clear media. The retinal and optic nerve head appeared normal in the right eye whereas in the left eye was a huge tuberous hamartoma of the optic disc and macular as well as generalised vascular occlusion and subretinal fluid. The Computerized tomography (CT) scan showed an Intraventricular tumour, with calcification within the tumours and subependymal. There was associated obstructive hydrocephalus. Patient was managed by a multi disciplinary team of ophthalmologists, neurosurgeons and radiologists, coordinated by a paediatrician. Conclusion: The diagnosis of tuberous sclerosis complex (TSC) was based on the lesions found on clinical examination, imaging, and pathologic studies. The blindness was multi-factorial in cause including intracranial, retinal and optic nerve tumours. Comprehensive medical history, detailed physical examinations and neuroimaging study are essential in making a diagnosis of TSC. Our patient was mis-diagnosed at various health facilities for many years. This delay in making appropriate diagnosis and instituting treatment could have contributed to the eventual outcome. Keywords: Tuberous Sclerosis, Blindness, Deteriorating Academic Performance

Optic pathway glioma in children: does visual deficit correlate with radiology in focal exophytic lesions?

Unlike pilocytic astrocytomas in other parts of the brain, optic pathway gliomas (OPG) are usually diffuse lesions involving the anterior optic pathways and hypothalamus. Their infiltrative nature often precludes complete surgical resection. We sought to determine whether careful magnetic resonance (MR) analysis, correlated with visual deficits, could be sufficient to identify those focal lesions that may be amenable to more aggressive surgical resection at presentation. We retrospectively reviewed the medical records of patients from two sites: children under 20 years of age treated for OPG between 1985 and 2009 at St Jude's Children's Research Hospital and children under 16 years of age treated at Great Ormond Street Hospital, London, UK, between 1984 and 2011. Patients with isolated optic nerve tumors were excluded. Visual acuity and visual field data at presentation were reviewed and correlated with MR characteristics, including extent of optic pathway involvement, symmetry, and lateral extension. Two hundred and one children were treated for OPG between 1984 and 2011 in the two institutions; 74 had neurofibromatosis 1 (NF1). At presentation, visual loss was symmetrical in 132 patients and asymmetrical in 69. Potential correlation between pattern of visual loss and tumor characteristics on routine MRI was found in only 13 patients with asymmetrical vision. There was no difference between patients with and without NF1. The decision for aggressive surgical resection for optic pathway gliomas should be based on clinical criteria, particularly in children with good vision in one eye and poor vision in the other, as current MRI results do not reliably predict visual field deficits.

SU‐E‐J‐187: Management of Optic Organ Motion in Fractionated Stereotactic Radiotherapy

Purpose:Fractionated stereotactic radiotherapy (FSRT) for optic nerve tumors can potentially use planning target volume (PTV) expansions as small as 1–5 mm. However, the motion of the intraorbital segment of the optic nerve has not been studied.Methods:A subject with a right optic nerve sheath meningioma underwent CT simulation in three fixed gaze positions: right, left, and fixed forward at a marker. The gross tumor volume (GTV) and the organs‐at‐risk (OAR) were contoured on all three scans. An IMRT plan using 10 static non‐coplanar fields to 50.4 Gy in 28 fractions was designed to treat the fixed‐forward gazing GTV with a 1 mm PTV, then resulting coverage was evaluated for the GTV in the three positions. As an alternative, the composite structures were computed to generate the internal target volume (ITV), 1 mm expansion free‐gazing PTV, and planning organat‐risk volumes (PRVs) for free‐gazing treatment. A comparable IMRT plan was created for the free‐gazing PTV.Results:If the patient were treated using the fixed forward gaze plan looking straight, right, and left, the V100% for the GTV was 100.0%, 33.1%, and 0.1%, respectively. The volumes of the PTVs for fixed gaze and free‐gazing plans were 0.79 and 2.21 cc, respectively, increasing the PTV by a factor of 2.6. The V100% for the fixed gaze and free‐gazing plans were 0.85 cc and 2.8 cc, respectively increasing the treated volume by a factor of 3.3.Conclusion:Fixed gaze treatment appears to provide greater organ sparing than free‐gazing. However unanticipated intrafraction right or left gaze can produce a geometric miss. Further study of optic nerve motion appears to be warranted in areas such as intrafraction optical confirmation of fixed gaze and optimized gaze directions to minimize lens and other normal organ dose in cranial radiotherapy.

À propos de 5 cas de tumeurs du nerf optique sur une période de 6 ans

Optic nerve tumors are uncommon and rarely described. By way of five patients treated at the University Hospital of Clermont-Ferrand, France, over a six-year-period, we discuss their clinical and radiological characteristics, their treatment and their course. We report two cases of optic nerve glioma in small children, two cases of optic nerve sheath meningioma and a 57-year-old patient treated for lung cancer with an optic nerve metastasis and choroidal and brain metastases. The diagnosis was confirmed by imaging in all patients and histologically for the cases of optic nerve glioma. The children with glioma were exclusively treated with chemotherapy, the two women with meningioma exclusively with radiation therapy, and the man with the optic nerve metastasis by chemotherapy and radiotherapy. The tumors were stabilized in all cases except for a progression in one case of glioma, one year after treatment. Gliomas and meningiomas are the most frequent tumors; other tumors are rare. Through a literature review and many illustrations, we discuss epidemiologic, clinical and radiologic characteristics, treatment and course of these uncommon tumors. The diagnosis of optic nerve tumors must be considered in cases of anterior or posterior progressive optic neuropathy. Treatment must be a compromise between effective treatment of the tumor and preservation of visual function. Decisions are made through multidisciplinary consultations, in which the role of the ophthalmologist is crucial for the diagnosis and success of the treatment.

Ga-68-DOTA-TATE PET/CT for Discrimination of Tumors of the Optic Pathway

Purpose: Symptomatic tumors of the optic nerve pathway may endanger vision. They are difficult to classify by imaging alone and biopsy may damage visual function. Tumor pathology influences treatment decision and a diagnostic tool with a high sensitivity and specificity would therefore be invaluable. We hypothesized that Ga-68-DOTA-TATE PET/CT may help in discriminating optic nerve tumors as uptake of somatostatin is elevated in meningiomas.Material and Methods: Ga-68-DOTA-TATE PET/CT was used to examine 13 patients with ambiguous, symptomatic lesions of the optic pathway for treatment planning. The presence or absence of meningioma was validated by histopathology or supplementary diagnostic work-up.Results: Ga-68-DOTA-TATE PET/CT identified 10 meningiomas (en plaque = 1, optic nerve sheath = 4, sphenoidal = 5) correctly via increased SSTR (somatostatin receptor) expression (mean SUVmax (maximum standardized uptake value) = 14.3 ± 15.4). 3 tumors did not show elevated Ga-68-DOTA-TATE uptake (SUVmax = 2.1 ± 1.0). Subsumizing all clinical-radiological follow-up tools available, these lesions were classified as an intracerebral metastasis of an advanced gastric carcinoma, histologically proven inflammatory collagenous connective tissue and presumed leukemic infiltration of a newly diagnosed chronic lymphocytic leukemia. In this case series, Ga-68-DOTA-TATE PET/CT demonstrated both a sensitivity and specificity of 100%. Yet, the golden standard of histopathology was only available in a subset of patients included.Conclusion: Ga-68-DOTA-TATE PET/CT proved to be a valuable diagnostic tool for the correct classification of equivocal, symptomatic tumors of the anterior optic pathway requiring therapy. PET/CT results influenced therapy decision essentially in all cases.

Pocket Guide to Ocular Oncology and Pathology, Grossniklaus H E, Bergstrom C, Baker Hubbard G, Wells J R, Singh A. ISBN: 978-3-642-28911-8 Springer

All would agree how vitally important it is for pathologists and other specialists to speak a common language when describing and discussing tumours, and when discussing specimen transport and interpretation. This is why this new “Pocket Guide to Ocular Oncology and Pathology” from Hans Grossniklaus is so welcoming and a timely addition to the ophthalmic pathology literature: it furthers this bridging between ocular oncologists and the pathology laboratory. The Sections in the Guide are organised systematically as follows: Part 1 Basic Principles and Techniques; Part II Conjunctival and Corneal Tumors; Part III Retinal, RPE and Ciliary Epithelial Tumors; Part IV Retinal Tumors; Part V Uveal Tumors: Non-Melanocytic; Part VI Uveal Tumors: Nevus and Melanoma; Part VII Optic Nerve Tumors and Phakomatoses. Each chapter is well-structured and all information is concisely stated under the following subheadings Synonyms, Definition, Etiology, Clinical Presentation, Diagnosis, Pathology, Differential Diagnosis, Prophylaxis, Therapy, Epidemiology, Cross References, and Further Reading. Such a structure enables the student to revise intensely prior to theoretical and practical exams. Most chapters contain colour illustrations, schematic figures, and/or pastel-colored tables that highlight the main points made within the text. Each chapter is accompanied by a reasonably updated list of references, which are listed alphabetically. The book would be especially useful to surgeons, pathologists, and radiologists practicing in less subspecialised institutions, particularly to those still in training. We have a few quibbles and suggestions to the authors when they consider the next iterations of this book. These are the following: 1. For the book truly to be a pocket guide we suggest that its formatting is smaller, so that it does fit into the typical coat or jacket pocket. Perhaps a soft or water-resistant cover should also be considered. 2. Changes in font or font color and dividing lines between parts would make the “

Optic nerve glioma: an update.

Optic nerve glioma is the most common optic nerve tumour. However, it has an unpredictable natural history. The treatment of optic nerve gliomas has changed considerably over the past few years. Chemotherapy and radiation therapy can now stabilize and in some cases improve the vision of patients with optic nerve gliomas. The treatment of optic nerve glioma requires a multi-disciplinary approach where all treatment options may have to be implemented in a highly individualized manner. The aim of this review article is to present current diagnostic and treatment protocols for optic nerve glioma.