Intravitreal Topotecan for Vitreous Seeds in Retinoblastoma: A Long-term Review of 91 Eyes.

PURPOSE: To study the long-term efficacy of intravitreal topotecan (IVT) for vitreous seeds in eyes with retinoblastoma and risk factors for their recurrence. DESIGN: Retrospective, non-comparative, interventional study. PARTICIPANTS: Ninety-one eyes of 90 patients with retinoblastoma treated between January 2013 and April 2019. METHODS: Patients with recurrent or refractory vitreous seeds after completion of intravenous or intra-arterial chemotherapy were treated with IVT (30 µg/0.15 ml) by the safety-enhanced technique. The injection was repeated every 4 weeks until the regression of seeds. Patients with a minimum follow-up of 12 months were included in the analysis. MAIN OUTCOME MEASURES: Primary outcome measures were vitreous seed regression and eye salvage. Secondary outcomes were risk factors for vitreous seed recurrence after treatment with IVT, vision salvage, and complications of IVT. RESULTS: The median age of the patients was 18 months, with most having group D (n = 58 [64%]) and group E (n = 26 [29%]) retinoblastoma. Vitreous seeds were refractory in 46 eyes (51%) and recurrent in 45 eyes (49%). A total of 317 IVT injections were administered, with the median being 3 injections. The median number of IVT injections required was 2.5 injections for dust, 3 injections for sphere, and 5 injections for cloud morphologic features. Recurrence of vitreous seeds after IVT was seen in 17 eyes (19%) at a mean follow-up of 7.9 months. At a mean follow-up 34 months, vitreous seed regression was achieved in 88 eyes (97%) and eye salvage was achieved in 77 eyes (85%). Older age (P = 0.018) and recurrence of retinal tumor (15/17 eyes; P < 0.01) significantly increased the risk of vitreous seed recurrence. Cataract was the most common complication seen in 17 eyes (9%). CONCLUSIONS: Intravitreal topotecan at an every 3- to 4-week regimen is effective against both refractory and recurrent vitreous seeds. The vitreous seed morphologic features correspond to the number of injections required for regression. Increasing age and recurrence of retinal tumor increase the risk of vitreous seed recurrence after treatment with IVT. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Integrated Omic Analysis Delineates Pathways Modulating Toxic TDP-43 Protein Aggregates in Amyotrophic Lateral Sclerosis.

Amyotrophic lateral sclerosis (ALS) is a multi-systemic, incurable, amyloid disease affecting the motor neurons, resulting in the death of patients. The disease is either sporadic or familial with SOD1, C9orf72, FUS, and TDP-43 constituting the majority of familial ALS. Multi-omics studies on patients and model systems like mice and yeast have helped in understanding the association of various signaling and metabolic pathways with the disease. The yeast model system has played a pivotal role in elucidating the gene amyloid interactions. We carried out an integrated transcriptomic and metabolomic analysis of the TDP-43 expressing yeast model to elucidate deregulated pathways associated with the disease. The analysis shows the deregulation of the TCA cycle, single carbon metabolism, glutathione metabolism, and fatty acid metabolism. Transcriptomic analysis of GEO datasets of TDP-43 expressing motor neurons from mice models of ALS and ALS patients shows considerable overlap with experimental results. Furthermore, a yeast model was used to validate the obtained results using metabolite addition and gene knock-out experiments. Taken together, our result shows a potential role for the TCA cycle, cellular redox pathway, NAD metabolism, and fatty acid metabolism in disease. Supplementation of reduced glutathione, nicotinate, and the keto diet might help to manage the disease.

Histopathology-guided management of ocular surface squamous neoplasia with corneal stromal or scleral invasion using ruthenium-106 plaque brachytherapy.

BACKGROUND/AIM: To evaluate the safety and efficacy of ruthenium-106 (Ru-106) plaque brachytherapy in managing invasive ocular surface squamous neoplasia (OSSN). METHODS: This is a retrospective, non-comparative, interventional case series of 42 eyes with OSSN with histopathologically-proven corneal stromal and/or scleral invasion that underwent Ru-106 plaque brachytherapy. Main outcome measures were tumour regression, eye salvage, final visual acuity, treatment complications and metastasis. RESULTS: At presentation, the mean tumour basal diameter was 9.3 mm (range 5-26 mm) and thickness 3.1 mm (range 1.5-11 mm). Prior treatment included excision biopsy in two patients (5%), incision biopsy and topical interferon in one each (2%). Following excision with 4 mm clinically clear margins, corneal stromal and/or scleral invasion of OSSN was confirmed in all 42 cases, with the excised base showing invasive squamous cell carcinoma. A total dose of 5000 cGy over a mean duration of 19.7 hours (range 7-41 hours) was provided to an axial depth of 2 mm using Ru-106 surface plaque. Over a mean follow-up of 36.9 months (range 22.3-72 months), complete tumour regression was achieved in all eyes (100%). Two eyes (5%) showed conjunctival tumour growth remote from the site of prior treatment. Visual acuity was maintained at ≥20/200 in 35 eyes (83%), with a loss of >2 Snellen lines in 1 eye (2%). There was no evidence of regional lymph node or systemic metastasis. CONCLUSION: Histopathology-guided use of Ru-106 surface plaque brachytherapy is a safe and an effective adjuvant therapy in the management of corneal stromal and/or scleral invasion of OSSN.

A fungal epidemic amidst a viral pandemic: Risk factors for development of COVID-19 associated rhino-orbital-cerebral mucormycosis in India.

PURPOSE: To study the risk factors for development of COVID-19 associated rhino-orbital-cerebral mucormycosis (ROCM) during the COVID-19 pandemic in India. METHODS: Multi-centric retrospective case-control study conducted from October 2020 to May 2021. Cases comprised of consecutive patients of COVID-19-associated ROCM (CA-ROCM) presenting at the participating ophthalmic institutes. Controls comprised of COVID-19-positive or COVID-19-recovered patients who did not develop ROCM. Comparative analysis of demographic, COVID-19 infection, treatment parameters and vaccination status between cases and controls performed. Clinical and imaging features of CA-ROCM analyzed. RESULTS: There were 179 cases and 361 controls. Mean age of presentation in cases was 52.06 years (p = .001) with male predominance (69.83%, p = .000011). Active COVID-19 infection at the time of presentation of ROCM (57.54%, p < .0001), moderate to severe COVID-19 (p < .0001), steroid administration (OR 3.63, p < .00001), uncontrolled diabetes (OR 32.83, p < .00001), random blood sugar >178 mg/dl were associated with development of CA-ROCM. Vaccination showed a protective effect (p = .0049). In cases with intracranial or cavernous sinus extension there was history of steroid administration (OR 2.89, p = .024) and orbital apex involvement on imaging (OR 6.202, p = .000037) compared to those with only rhino-orbital disease. CONCLUSION: Male gender, active COVID-19 infection, moderate or severe COVID-19, uncontrolled diabetes, steroid administration during COVID-19 treatment are risk factors for developing rhino-orbital-cerebral mucormycosis. Vaccination is protective. Random blood sugar of >178 mg/dl in COVID-19 positive or recovered patients should warrant close observation and early detection of ROCM. Presence of ophthalmoplegia, blepharoptosis at first clinical presentation and orbital apex involvement on imaging are associated with intracranial extension in ROCM.

Long term outcome of adjuvant photodynamic therapy after cyberknife radiotherapy for choroidal melanoma.

PURPOSE: To report the outcome of photodynamic therapy (PDT) for choroidal melanoma as adjuvant treatment with CyberKnife radiotherapy. DESIGN: Retrospective interventional case series. METHODS: Standard-fluence PDT using verteporfin. OUTCOME MEASURES: Regression of tumor; resolution of subretinal fluid (SRF); change in best-corrected visual acuity (BCVA), and complications of PDT. RESULTS: The study included 16 choroidal melanomas (3 pigmented, 4 lightly pigmented, 9 amelanotic) treated with adjuvant PDT after CyberKnife radiotherapy. The mean follow up time was 45.5 months after the initial PDT. 13 patients improved completely with PDT sessions and growth was seen in 3 patients. There was seen completely resolution in SRF in 10 eyes, partial resolution in 3 eyes, and stable in 3 eyes. The mean thickness of tumors was 3.9 mm before PDT and 2.3 mm after PDT. Retina pigment epithelium atrophy in 3 patients and subretinal hemorrhage in 1 patient were seen as complication of PDT. Three patients underwent enucleation for recurrence in the tumor. There was not a higher rate of change in BCVA after PDT (37.5% stable; 25% increase; 37.5% decrease. Poor final visual acuity associated with worse initial visual acuity, proximity of the tumor to the foveola and optic disc, and radiation complications. CONCLUSIONS: PDT seems to offer a good option for posterior pole choroidal melanoma as adjuvant therapy in suitable cases. Future prospective studies with larger number of patients and with longer follow-up are needed to further investigation.

The genome trilogy of Anopheles stephensi, an urban malaria vector, reveals structure of a locus associated with adaptation to environmental heterogeneity.

Anopheles stephensi is the most menacing malaria vector to watch for in newly urbanising parts of the world. Its fitness is reported to be a direct consequence of the vector adapting to laying eggs in over-head water tanks with street-side water puddles polluted by oil and sewage. Large frequent inversions in the genome of malaria vectors are implicated in adaptation. We report the genome assembly of a strain of An. stephensi of the type-form, collected from a construction site from Chennai (IndCh) in 2016. The genome reported here with a L50 of 4, completes the trilogy of high-resolution genomes of strains with respect to a 16.5 Mbp 2Rb genotype in An. stephensi known to be associated with adaptation to environmental heterogeneity. Unlike the reported genomes of two other strains, STE2 (2R+b/2Rb) and UCI (2Rb/2Rb), IndCh is found to be homozygous for the standard form (2R+b/2R+b). Comparative genome analysis revealed base-level details of the breakpoints and allowed extraction of 22,650 segregating SNPs for typing this inversion in populations. Whole genome sequencing of 82 individual mosquitoes from diverse geographical locations reveal that one third of both wild and laboratory populations maintain the heterozygous genotype of 2Rb. The large number of SNPs can be tailored to 1740 exonic SNPs enabling genotyping directly from transcriptome sequencing. The genome trilogy approach accelerated the study of fine structure and typing of an important inversion in An. stephensi, putting the genome resources for this understudied species on par with the extensively studied malaria vector, Anopheles gambiae. We argue that the IndCh genome is relevant for field translation work compared to those reported earlier by showing that individuals from diverse geographical locations cluster with IndCh, pointing to significant convergence resulting from travel and commerce between cities, perhaps, contributing to the survival of the fittest strain.

Impact of COVID-19 nationwide lockdown on retinoblastoma treatment and outcome: A study of 476 eyes of 326 children.

Purpose: The novel coronavirus SARS-CoV-2 (COVID-19) and the resultant nationwide lockdown and travel restrictions led to difficulty in providing timely and regular treatment to patients with childhood cancers such as retinoblastoma. This study is aimed at assessing the demography, clinical presentation, treatment strategies, and outcome of treatment defaulters due to the lockdown. Methods: Cross-sectional, observational study of retinoblastoma patients at a tertiary care ocular oncology center during the first wave of COVID-19 and the resulting nationwide lockdown. Results: Of the 476 eyes of 326 patients undergoing active management with a median age of 57 months (range: 4-214 months), 205 (63%) patients returned for follow-up after a mean delay of 45.8 ± 24.3 weeks (range: 8-80 weeks) and 121 (37%) were defaulters according to the data analyzed till June 30, 2021. Distance of residence was ≥1000 km for 148 patients (46%). In terms of need for active treatment, the number of emergent cases was 2 (<1%), 11 (3%) were urgent, and 313 (96%) were semi-urgent. International classification groups D (n = 107 eyes, 23%) and E (n = 173 eyes, 36%) were in majority, and 13 eyes (4%) and 4 eyes (1%) were at stages 3 and 4, respectively. Prior to lockdown, 86 eyes (18%) had active tumor, which remained unchanged (n = 26, 30%) or worsened (n = 49, 60%) after failure to follow-up. Vision (47%), eye (92%), and life salvage (98%) were achieved by individualized protocol-based management after the patients returned for further management. Five children succumbed to intracranial extension. Conclusion: The COVID-19-related nationwide lockdown has deprived retinoblastoma patients of optimal and timely management, leading to prolonged treatment interruptions, delays, permanent default, and death. It is of paramount importance for all the stakeholders to increase awareness, make necessary travel and logistic arrangements, and ensure continuity of care for children with retinoblastoma.

Magnetic resonance imaging of the orbit, Part 1: Basic principles and radiological approach.

Magnetic resonance imaging (MRI) is an eloquent, noninvasive, cross-sectional imaging modality that offers superior tissue characterization of orbital pathologies. The ophthalmologist needs to be aware of the advantages of MRI and its step-wise interpretation in liaison with a radiologist to optimize patient outcomes. In this review, we discuss the basic principles of MRI, some of the commonly used sequences and protocols, the anatomy of the orbit on MRI, and an approach to radiological interpretation.

Magnetic resonance imaging of the orbit, Part 2: Characterization of orbital pathologies.

In this article we focus on a systematic approach to assess common orbital lesions on magnetic resonance imaging (MRI). The identification of the probable compartment or structure of origin helps narrow the differential diagnosis of a lesion. Analyzing the morphology, appearance, and signal intensity on various sequences, the pattern, and degree of contrast enhancement are key to characterize lesions on MRI. Imaging features suggesting cellularity and vascularity can also be determined to help plan for biopsy or surgery of these lesions. MRI can also distinguish active from chronic disease in certain pathologies and aids in selecting appropriate medical management. MRI may thus serve as a diagnostic tool and help in guiding therapeutic strategies and posttreatment follow-up.

Epidemiology, clinical profile, management, and outcome of COVID-19-associated rhino-orbital-cerebral mucormycosis in 2826 patients in India - Collaborative OPAI-IJO Study on Mucormycosis in COVID-19 (COSMIC), Report 1.

Purpose: COVID-19-associated rhino-orbital-cerebral mucormycosis (ROCM) has reached epidemic proportion during India's second wave of COVID-19 pandemic, with several risk factors being implicated in its pathogenesis. This study aimed to determine the patient demographics, risk factors including comorbidities, and medications used to treat COVID-19, presenting symptoms and signs, and the outcome of management. Methods: This was a retrospective, observational study of patients with COVID-19-associated ROCM managed or co-managed by ophthalmologists in India from January 1, 2020 to May 26, 2021. Results: Of the 2826 patients, the states of Gujarat (22%) and Maharashtra (21%) reported the highest number of ROCM. The mean age of patients was 51.9 years with a male preponderance (71%). While 57% of the patients needed oxygen support for COVID-19 infection, 87% of the patients were treated with corticosteroids, (21% for > 10 days). Diabetes mellitus (DM) was present in 78% of all patients. Most of the cases showed onset of symptoms of ROCM between day 10 and day 15 from the diagnosis of COVID-19, 56% developed within 14 days after COVID-19 diagnosis, while 44% had delayed onset beyond 14 days. Orbit was involved in 72% of patients, with stage 3c forming the bulk (27%). Overall treatment included intravenous amphotericin B in 73%, functional endoscopic sinus surgery (FESS)/paranasal sinus (PNS) debridement in 56%, orbital exenteration in 15%, and both FESS/PNS debridement and orbital exenteration in 17%. Intraorbital injection of amphotericin B was administered in 22%. At final follow-up, mortality was 14%. Disease stage >3b had poorer prognosis. Paranasal sinus debridement and orbital exenteration reduced the mortality rate from 52% to 39% in patients with stage 4 disease with intracranial extension (p < 0.05). Conclusion: : Corticosteroids and DM are the most important predisposing factors in the development of COVID-19-associated ROCM. COVID-19 patients must be followed up beyond recovery. Awareness of red flag symptoms and signs, high index of clinical suspicion, prompt diagnosis, and early initiation of treatment with amphotericin B, aggressive surgical debridement of the PNS, and orbital exenteration, where indicated, are essential for successful outcome.

Orbital infarction syndrome secondary to rhino-orbital mucormycosis in a case of COVID-19: Clinico-radiological features.

Orbital infarction syndrome is an uncommon pathology with devastating consequences. It is frequently secondary to atherothrombotic phenomena in the internal carotid artery. We report a case of a 66-year-old male with uncontrolled diabetes and use of systemic steroids for COVID-19, who presented with a sudden loss of vision in the left eye, with total ophthalmoplegia and diffuse opacification of the retina. On imaging, he was found to have features of rhino-orbital cellulitis with ischemia of the orbital tissue secondary to isolated ophthalmic artery obstruction (OAO) with a patent internal carotid artery. KOH mount of deep nasal swab was confirmatory of mucor. This is the first reported case of orbital infarction syndrome in the setting of COVID-19.

Outcomes of Intravenous Chemotherapy (Chemoreduction) for Retinoblastoma Based on Patient Age in 964 Eyes of 554 Patients.

PURPOSE: To evaluate retinoblastoma control after intravenous chemotherapy (chemoreduction) by patient age at presentation. DESIGN: Retrospective case series. METHODS: This study included 964 eyes of 554 patients treated with chemoreduction at Ocular Oncology Service at Wills Eye Hospital. Patients received 6 monthly cycles of standard chemoreduction. Additional therapies for tumor control were performed as needed. RESULTS: Of 964 eyes, a comparison by age group (<6 months vs. 6-12 months vs. 13-24 months vs. >24 months) revealed more advanced age group with higher frequency of group E tumor (15% vs. 25% vs. 32% vs. 39%, P < 0.001). By treatment outcomes, complete tumor control was achieved with chemoreduction alone more often in less advanced age group (46% vs. 30% vs. 17% vs. 8%, P < 0.001). Additional treatment after chemoreduction was needed more often in more advanced age group with external beam radiotherapy (EBRT; 9% vs. 16% vs. 20% vs. 15%, P = 0.006) or enucleation (12% vs. 18% vs. 26% vs. 37%, P < 0.001). Over time (1994-1998 vs. 1999-2003 vs. 2004-2008 vs. 2009-2013 vs. 2014-2019), the paradigm for additional required treatment after chemoreduction shifted toward less EBRT (27% vs. 24% vs. 14% vs. 7% vs. 2%, P < 0.001) and more intra-arterial (0% vs. 0% vs. 1% vs. 25% vs. 48%, P < 0.001) and intravitreal (0% vs. 0% vs. 3% vs. 10% vs. 20%, P < 0.001) chemotherapy. CONCLUSIONS: Chemoreduction is a safe and effective treatment method for patients with retinoblastoma, demonstrating the best tumor control in the younger age groups.

Risk Factors for Tumor Recurrence Following Primary Intravenous Chemotherapy (Chemoreduction) for Retinoblastoma in 869 Eyes of 551 Patients.

PURPOSE: To identify risk factors for retinoblastoma recurrence following chemoreduction. METHODS: This was a retrospective review of patients with retinoblastoma treated from 1994 to 2019 using chemoreduction with analysis for recurrence using Kaplan-Meier, Cox regression, and logistic regression. RESULTS: There were 869 eyes of 551 patients with retinoblastoma treated with chemoreduction. Follow-up in 556 eyes revealed main solid tumor recurrence (n = 355, 64%), subretinal seed recurrence (n = 244, 44%), vitreous seed recurrence (n = 162, 29%), and/or new tumor (n = 118, 21%) requiring management with focal therapy (transpupillary thermotherapy, cryotherapy) (n = 294, 53%), intra-arterial chemotherapy (n = 125, 22%), intravitreal chemotherapy (n = 36, 6%), plaque radiotherapy (n = 120, 22%), external beam radiotherapy (n = 57, 10%), and/or enucleation (n = 49, 9%). Of all recurrences, 62% were detected by 1 year, 86% by 2 years, 94% by 3 years, 98% by 5 years, 99% by 10 years, and 100% by 15 years. Risk factors for recurrence on multivariate analysis included younger patient age at presentation (odds ratio [OR] = 1.02 [1.00 to 1.04] per 1 month decrease, P = .02), greater International Classification of Retinoblastoma group (OR = 1.24 [1.05 to 1.47] per 1 more advanced group, P = .01), shorter tumor distance to optic disc (OR = 1.11 [1.01 to 1.21] per 1 mm decrease, P = .03), and presence of subretinal seeds (OR = 1.66 [1.09 to 2.53], P = .02). CONCLUSIONS: Retinoblastoma recurrence after chemoreduction is usually detected within the first 3 years following treatment. Younger patients with more advanced, posteriorly located tumors and subretinal seeds at presentation are at increased risk, but recurrence can often be managed with globe-sparing therapy. [J Pediatr Ophthalmol Strabismus. 2020;57(4):224-234.].

Long-term (20-year) real-world outcomes of intravenous chemotherapy (chemoreduction) for retinoblastoma in 964 eyes of 554 patients at a single centre.

BACKGROUND: Intravenous chemotherapy (IVC) remains an important globe salvage therapy for retinoblastoma. METHODS: Evaluation of long-term globe salvage at 5, 10, 15 and 20 years following frontline IVC for retinoblastoma. RESULTS: Of 994 eyes, comparison by International Classification of Retinoblastoma group (A vs B vs C vs D vs E) revealed more advanced group with older mean age at presentation (8 vs 7 vs 10 vs 11 vs 15 months, p<0.001). By clinical features, more advanced group demonstrated greater mean tumour diameter (3.2 vs 6.8 vs 9.4 vs 14.3 vs 16.4, p<0.001) and thickness (2.0 vs 3.7 vs 4.4 vs 7.3 vs 9.3, p<0.001), and greater frequency of vitreous seeds ≥1 quadrant (0% vs 0% vs 44% vs 42% vs 57%, p<0.001) and subretinal seeds (0% vs 0% vs 22% vs 65% vs 54%, p<0.001). By outcomes, less advanced group demonstrated greater tumour control (without need for enucleation or external beam radiotherapy (EBRT)) by year 2 (96% vs 91% vs 91% vs 71% vs 32%, p<0.001), and with minimal change up to 20 years. In order to achieve globe salvage, additional intra-arterial chemotherapy (IAC) or plaque radiotherapy was employed by year 2 (5% vs 26% vs 28% vs 27% vs 19%, p<0.001), with little further need up to 20 years. Pinealoblastoma (2%), metastasis (2%) and death (1%) were infrequent. CONCLUSION: Frontline IVC (plus additional IAC and/or plaque radiotherapy) for retinoblastoma provided complete tumour control for groups A (96%), B (91%), C (91%), D (71%) and E (32%), avoiding enucleation or EBRT and was lasting for up to 20 years.

Multifocal choroidal melanoma in oculodermal melanocytosis in an Asian Indian.

A 51-year-old Asian Indian male with right oculodermal melanocytosis presented with a blurred visual acuity of 20/40. Upon fundus examination, he was discovered to have two independent pigmented choroidal melanomas in the temporal juxtapapillary region and inferiorly, in the region of trace clinical sectoral choroidal melanocytosis. The patient underwent enucleation, and on histopathology the two tumors were found to be in the area of choroidal melanocytosis, in continuity with each other. Multifocal choroidal melanoma is an exceedingly rare diagnosis. Ocular melanocytosis is a known predisposing factor for both unifocal and multifocal melanomas. Lifelong monitoring for uveal melanomas must be done in all patients with ocular melanocytosis.

External dacryocystorhinostomy conventional surgery versus Pawar implant: A comparative study.

Purpose: External dacryocystorhinostomy (DCR) surgeries are cost-effective with excellent success rates. The present study was designed to compare the safety and efficacy of conventional external DCR versus external DCR using Pawar silicone implant in chronic dacryocystitis. Methods: This is a prospective, comparative, interventional case series over a period of 18 months with patients managed by external DCR surgery with and without Pawar implant. Institutional review board approval was obtained before the study. The success of the surgery was objectively measured by sac patency on syringing at the last follow up. Results: A total of 65 patients with chronic dacryocystitis were included in the study. The mean age of patients in the series was 41.43 years (median, 41 years; range, 12 years-60 years). All patients presented with epiphora (100%) and underwent external DCR and were chosen for conventional surgery (n = 33, 51%, group 1) or Pawar silicone implant surgery (n = 32, 49%, group 2) on a random basis. The mean duration of the surgery from the time of skin incision to skin closure for group 1 was 27.7 minutes (median, 26 minutes; range, 21-30 minutes) while in group 2, it was 75.5 minutes (median, 75 minutes; range, 60-88 minutes), which was statistically significant (P < 0.01). The success rate of the procedure done in group 1 was 90% which increased to 97% after the management of failed cases as compared to the success rate in group 2 of 91% and 94%, before and after the management of failed cases, respectively. Conclusion: External DCR using Pawar implant is a safe surgery which is faster than conventional external DCR with almost equal success rates between both the procedures.

Primary lacrimal gland teratoma in an adult: A clinicopathologic and radiological correlation.

A 20-year-old male presented with a gradually growing mass in the left lateral brow region for 2 years. On imaging, the mass appeared to arise from the lacrimal gland, was cystic, without any bony abnormality. With a diagnosis of dermoid cyst, an excision biopsy was performed. The histopathology revealed an epithelium-lined cyst, with the wall of the cyst comprising dermal adnexa and inflammatory cells. The wall also contained fragments of skeletal muscle, nerve bundles, adipose tissue, exocrine and secretory acini, few blood vessels with occasional hair shafts, with the lacrimal gland in the periphery of the tumor. There were no immature elements, atypia, and malignant cells, thus confirming the diagnosis of a mature cystic teratoma. Primary lacrimal gland teratoma is a rare diagnosis and its presentation in adults is extremely uncommon.

Primary orbital low-grade fibromyxoid sarcoma - A case report.

An 18-year-old female presented with rapidly progressive proptosis of the left eye for one month and grade II relative afferent pupillary defect. Orbital imaging showed a well-defined homogenous extraconal mass in close relation to the lateral rectus muscle and extending up to the superior orbital fissure, associated with bony erosion. An incisional biopsy was performed, with the histopathology demonstrating stellate to spindle-shaped tumor cells (fibroblasts) embedded in a richly myxoid matrix. A diagnosis of low-grade fibromyxoid sarcoma (LGFS) was made. The patient was treated by stereotactic external beam radiotherapy. Here, we report a case of LGFS which, to the best of our knowledge, is the first at an orbital location.

Preservation of retinoblastoma group E eyes with neovascular glaucoma using intravenous chemotherapy: risk factors and outcomes.

BACKGROUND/AIM: To report the outcomes of retinoblastoma group E eyes with neovascular glaucoma (NVG) treated conservatively with intravenous chemotherapy and investigate factors associated with eye salvage and secondary enucleation. METHODS: This is a retrospective, comparative, interventional case series. The outcome measures were life salvage, eye salvage and vision salvage. RESULTS: Of the 37 eyes managed by intravenous chemotherapy, secondary enucleation was necessary in 21 eyes (group 1) and eye salvage was possible in 16 eyes (group 2). A comparison of both groups revealed significant difference with group 1 demonstrating greater duration of symptoms (18.8 weeks vs 5.4 weeks, p=0.016), greater intraocular pressure (IOP) at presentation (36 mm Hg vs 30 mm Hg, p=0.044), greater increase in corneal diameter (1.52 mm vs 0.50 mm, p=0.013) and the presence of sterile orbital cellulitis (9 vs 1, p=0.023). Further, the risk factors for secondary enucleation by univariate analysis were duration of symptoms >10 weeks (p=0.003), presenting IOP >26 mm Hg (p=0.045), buphthalmos (p=0.014) and sterile orbital cellulitis (p=0.023) and by multivariate analysis were age at presentation >6 months (p=0.012) and buphthalmos (p=0.017). At a mean follow-up of 20.5 months, none of the patients in either group developed systemic metastasis. CONCLUSION: For retinoblastoma group E eyes presenting with NVG, the chance of eye salvage with intravenous chemotherapy is better when the age at diagnosis is <6 months, duration of symptoms is <10 weeks, IOP is <26 mm Hg, and in the absence buphthalmos and sterile orbital inflammation.