Extended survival in a case of metastatic choroidal melanoma with immunotherapy.

Uveal melanoma is the most common intraocular malignancy in adults. Despite advances in local treatments, approximately 50% of all cases eventually die from metastatic disease. In cases with metastasis, 2- and 5-year survival rates are approximately 10% and <1%, respectively. Advances in molecular biology have led to the identification of a number of promising drugs including immune checkpoint inhibitors (ICIs). Ipilimumab and nivolumab are ICIs targeting the cytotoxic T-lymphocyte-associated antigen-4 and the programmed-cell death protein-1, respectively. Herein, we present a case of choroidal melanoma having liver metastasis treated with nivolumab and ipilimumab and transarterial radioembolization, achieving a 3-year survival.

Swept-source optical coherence tomography and swept-source optical coherence tomography angiography findings in circumscribed choroidal hemangioma before and after transpupillary thermotherapy.

PURPOSE: To investigate the swept-source optical coherence tomography (SS-OCT) and SS-OCT angiography (SS-OCTA) findings in circumscribed choroidal hemangioma (CCH) before and after treatment with transpupillary thermotherapy (TTT). METHODS: The clinical records of 21 eyes having CCH imaged with SS-OCT/SS-OCTA between September 2018 and December 2022 were evaluated. RESULTS: SS-OCT examination in CCH showed dome-shaped appearance (100%), choroidal shadowing (100%), expansion of choroidal structures (100%), subretinal fluid (66.7%), intraretinal edema/schisis (33.3%), retinal pigment epithelium (RPE) atrophy (19.0%), hyperreflective dots (19.0%), and epiretinal membrane (4.8%). Internal arborizing tumor vessels showing hyperreflectivity were observed in the choriocapillaris slab on SS-OCTA in all eyes. In the deep capillary plexus (DCP), flow void changes were seen in 7 eyes with intraretinal schisis/cystoid macular edema. Four CCHs > 2 mm in thickness showed outer retinal involvement due to unmasking of flow in intratumoral vessels related to RPE atrophy. Following TTT/indocyanine green-enhanced TTT (ICG-TTT) of CCH, SS-OCT findings included total/partial resolution of subretinal fluid (57.1%), complete/partial regression of the tumor (52.4%), and RPE atrophy (33.3%). After treatment; loss of choriocapillaris, decrease in tumor vascularity together with increase in the fibrous component and flow void areas were detected on SS-OCTA. CONCLUSIONS: SS-OCT/SS-OCTA are useful non-invasive tools for imaging the structural/vascular changes in CCHs managed with TTT or ICG-TTT. On SS-OCTA, hyporeflective spaces localizing to edema/schisis in the DCP and arborizing tumor vessels within a hyporeflective stromal background in the choriocapillaris slab were observed. After TTT/ICG-TTT, a decrease in tumor vessels and an increase in the fibrous component and flow-void areas inside the CCH were detected on SS-OCTA.

A rare case of orbital mantle cell lymphoma presenting as a well-demarcated mass on magnetic resonance imaging.

A 71-year-old male presented with painless and slowly progressive proptosis of the right eye. The patient had no history of cancer and was in good health. Orbital magnetic resonance imaging showed a well-demarcated, slightly lobulated intraconal orbital mass hypo-isointense on T1- weighted and isointense on T2-weighted images, demonstrating contrast enhancement in the right orbit. The lesion had a central cavitary region. An anterior orbitotomy with lower lid crease incision was performed and the lesion was excised in toto. Histopathologic examination revealed diffuse infiltration of monomorphic population of small lymphoid cells. Immunohistochemical staining demonstrated diffuse and strong positivity for CD20, CD5, and cyclin D1. Histopathological and immunohistochemical findings were consistent with mantle cell lymphoma. Positron emission tomography-computed tomography did not show systemic involvement. The patient underwent orbital external beam radiotherapy (36 Gy). There was no recurrence and/or systemic involvement at 2.5 years follow-up.

Anterior segment swept-source optical coherence tomography and ultrasound biomicroscopy in iris and ciliary body lesions.

OBJECTIVES: Differentiation of iris and ciliary body lesions as benign or malignant and cystic or solid is important. The aim of this study was to compare anterior segment swept-source optical coherence tomography (AS SS-OCT) and ultrasound biomicroscopy (UBM) findings in iris and ciliary body tumors. RESEARCH DESIGN AND METHODS: Forty-two eyes of 38 cases with iris and ciliary body tumors imaged with UBM and AS SS-OCT between September 2018 and September 2023 were evaluated retrospectively. RESULTS: Of 42 eyes, 14 had melanoma, 14 iris pigment epithelial (IPE) cysts, 7 nevi, 3 Lisch nodules, 2 iris stromal cysts, 1 pars plana cysts, and 1 iris mammillations. An equivalent (100%) visualization of the anterior tumor margin was obtained with both techniques. Compared to AS SS-OCT, UBM was superior for posterior margin visualization in melanocytic tumors and IPE cysts. Bland-Altman plots demonstrated good agreement between UBM and AS SS-OCT for melanocytic tumors < 2.5 mm in base diameter and < 2 mm in thickness. CONCLUSIONS: Although, UBM is the gold standard for ciliary body and iridociliary tumors. AS SS-OCT should be considered as an excellent alternative to UBM, especially in minimally elevated iris lesions.

Bilateral Corneal Panni Associated with Limbal Inclusion Cysts in the Left Eye in a Patient with Atopic Keratoconjunctivitis.

PURPOSE: To report a rare case of atopic keratoconjunctivitis (AKC) presenting with bilateral corneal panni associated with limbal inclusion cysts in the left eye. MATERIALS & METHODS: Retrospective case report. RESULTS: A 19-year-old female with AKC presented with bilateral corneal panni and limbal inclusion cysts in the left eye. Anterior segment swept-source optical coherence tomography showed bilateral hyperreflective epicorneal membrane and a lobulated cystic lesion in the left eye. Ultrasound biomicroscopy demonstrated a dense membrane overriding the cornea in both eyes and hyporeflective spaces separated by medium reflective septa in the cyst. The patient underwent excision of the limbal inclusion cyst and pannus in the left eye. Histopathological examination revealed subepithelial cystic lesion surrounded by non-keratinizing epithelium; areas of acanthosis, hyperkeratosis, parakeratosis, and hyperplasia in the epithelium of the pannus; as well as inflammation, fibrosis, and increase in vascularization in the stroma. CONCLUSION: To our knowledge, this is the first case of corneal pannus associated with limbal inclusion cysts in AKC. Surgical excision was done to establish the diagnosis as well as to improve vision in our case.

Wide-field Fundus Imaging and Fluorescein Angiography Findings in Various Pseudoretinoblastoma Conditions.

PURPOSE: To report wide-field fundus imaging and fluorescein angiography findings in conditions mimicking retinoblastoma (pseudoretinoblastoma). METHODS: The clinical and imaging records of 28 patients (36 eyes) imaged with RetCam 3 (Clarity Medical Systems, Inc) wide-field fundus photography and fluorescein angiography who were diagnosed as having various pseudoretinoblastoma disorders between February 2020 and August 2021 were retrospectively evaluated. RESULTS: Most patients were referred with suspicion of retinoblastoma. Other patients had unresolved eye conditions and received initial treatment elsewhere and they were referred later for exclusion of retinoblastoma. On RetCam fundus photography and fluorescein angiography, pseudoretinoblastoma conditions were diagnosed as Coats disease (9 eyes), congenital glaucoma (3 eyes), persistent fetal vasculature (3 eyes), familial exudative vitreoretinopathy (2 eyes), retinopathy of prematurity (2 eyes), myelinated retinal nerve fibers (2 eyes), optic nerve hypoplasia (2 eyes), oculocutaneous albinism (2 eyes), meridional fold (2 eyes), combined hamartoma of retina and retinal pigment epithelium (2 eyes), grouped congenital hypertrophy of the retinal pigment epithelium (1 eye), retinal astrocytic hamartoma (1 eye), morning glory syndrome (1 eye), optic glioma (1 eye), giant choroidal nevus (1 eye), vasculitis (1 eye), and chorioretinitis (1 eye). RetCam imaging and fluorescein angiography demonstrated telangiectasias, microaneurysms, submacular exudation/fibrosis, capillary non-perfusion, and exudation posterior to telangiectasias in Coats disease. Hypofluorescent optic discs, peripheral non-perfusion, and vascular leakage were observed in congenital glaucoma. Large areas of capillary non-perfusion, poor foveal morphology/formation, elongated ciliary processes with hyperfluorescent central core, and hyperfluorescent fibrovascular stalk at the posterior pole were hallmarks of persistent fetal vasculature. CONCLUSIONS: RetCam wide-field fundus imaging and fluorescein angiography play an important role in the diagnosis of conditions simulating retinoblastoma. [J Pediatr Ophthalmol Strabismus. 2023;60(1):60-74.].

Pseudoretinoblastoma: Distribution based on gender, age, and laterality.

PURPOSE: To investigate the distribution of pseudoretinoblastoma (PSRB) cases based on gender, age, and laterality. MATERIALS AND METHODS: The clinical records of 607 patients (851 eyes) who were referred for diagnosis of retinoblastoma or simulating conditions between October 1998 and May 2021 were retrospectively evaluated. Patients were stratified by age as follows: ≤1 year, >1-3 years, >3-5 years, and >5 years. RESULTS: Of 190/607 PSRB patients, 129 (67.9%) were males and 61 (32.1%) females (p = 0.001). The 3 most common diagnoses in males were Coats disease (20.2%), persistent fetal vasculature (PFV, 14.0%), and chorioretinal coloboma (6.2%). In females, the 3 most common diagnoses included PFV (21.3%), retinal dysplasia, congenital glaucoma, and combined hamartoma (each 6.6%). PFV was the most common diagnosis in ≤1 year old patient group (26.6%). Coats disease and PFV were the most common diagnoses in >1-3 years old patient group (each 16.7%). Coats disease was the most common diagnosis in >3-5 years old (30.8%) and >5 years old patient groups (13.1%). PSRBs were unilateral in 121/190 (63.7%) patients. Coats disease usually presented unilaterally (p < 0.001) while PFV, optic nerve head drusen, and retinopathy of prematurity as bilateral diseases (p = 0.019, p = 0.001, and p = 0.001 respectively). DISCUSSION: PSRB diagnoses show differences depending on gender, age, and laterality. In our study, the most common PSRB lesions were Coats disease in males and PFV in females. PFV was the most frequent diagnosis in ≤3 years and Coats disease in >3 years of age groups. Coats disease and PFV were the most common unilateral and bilateral PSRB diagnoses respectively.

Clinical applications of anterior segment swept-source optical coherence tomography: A systematic review.

Optical coherence tomography (OCT) is a non-invasive method that provides the opportunity to examine tissues by taking cross-sectional images. OCT is increasingly being used to evaluate anterior segment (AS) pathologies. Swept-source (SS) OCT allows greater penetration and achieves better visualization of the internal configuration of AS tissues due to the longer wavelength employed and high scan speeds. We reviewed the utilization of AS SS-OCT in various conditions including glaucoma, ocular surface pathologies, iris tumors, refractive surgery, cataract surgery, and scleral diseases. A systematic literature search was carried out on PubMed, Scopus, and Web of Science databases between January 1, 2008, and September 1, 2022 using the following keywords: AS SS-OCT; dry eye and SS-OCT; ocular surface and SS-OCT; cornea and SS-OCT; dystrophy and SS-OCT; glaucoma and SS-OCT; ocular surface tumors and SS-OCT; conjunctival tumors and SS-OCT; refractive surgery and SS-OCT; cataract and SS-OCT; biometry and SS-OCT; sclera and SS-OCT; iris and SS-OCT; ciliary body and SS-OCT; artificial intelligence and SS-OCT. A total of 221 studies were included in this review. Review of the existing literature shows that SS-OCT offers several advantages in the diagnosis of AS diseases. Exclusive features of SS-OCT including rapid scanning, deeper tissue penetration, and better image quality help improve our understanding of various AS pathologies.

Factors affecting eye conservation and metastasis in posterior uveal melanomas.

PURPOSE: To determine the effect of patient and tumor features and different treatments on eye removal (enucleation or exenteration) and metastasis in posterior uveal melanoma (PUM). METHODS: Retrospective analysis. Patient age (≤60 vs >60 years), sex (female vs male), visual acuity (VA, ≤20/40 vs >20/40), largest tumor basal diameter (LTBD), tumor thickness, tumor stage according to American Joint Committee on Cancer (AJCC) 8th edition, ciliary body involvement, distance to optic disc (OD)/fovea (≤3 mm vs >3 mm), OD involvement, and histopathology were evaluated. Primary treatment options were transpupillary thermotherapy, plaque radiotherapy, Cyberknife radiosurgery, exoresection, and eye removal. Risk factors for primary eye removal were determined using logistic regression test and those for secondary eye removal and metastasis with Cox regression analysis. RESULTS: Of 387 cases, 153 (39.5%) underwent primary eye removal. Multivariable risk factors for primary eye removal included AJCC tumor stage (p = 0.001, OR:4.586; p < 0.001, OR:34.545; p < 0.001, OR:103.468 for stages T2, T3, and T4 vs stage T1, respectively), and VA≤20/40 (p = 0.014, OR:2.597). Multivariable risk factors for secondary eye removal were VA≤20/40 (p = 0.019, RR:2.817) and AJCC stage T3 vs T1 (p = 0.021, RR:2.666). Eye preservation rates in patients undergoing eye-conserving treatments were 80.3%, 69.6%, and 51.5% at 5, 10, and 15 years, respectively. Metastasis-free survival rates were 81.0%, 73.0%, and 56.7% at 5, 10, and 15 years, respectively. Multivariable risk factors for metastasis included eye removal as primary treatment (p = 0.005, RR:2.828) and mixed type histopathology (p < 0.001, RR:4.804). DISCUSSION: Early diagnosis is crucial for both eye preservation and survival in PUM. Increasing AJCC tumor stage and lower VA were risk factors for eye removal in this study. Mixed type histopathology and primary eye removal were risk factors for metastasis.

Anterior segment optical coherence tomography in ocular surface tumours and simulating lesions.

This study aims to systematically review the reported literature on the use of anterior segment optical coherence tomography (AS-OCT) in ocular surface tumours and simulating lesions. A systematic literature search was done using PubMed, Scopus, and Web of Science databases between January 2002 and December 2021. On AS-OCT, ocular surface squamous neoplasia typically demonstrate epithelial thickening, epithelial hyperreflectivity, and an abrupt transition between normal and abnormal epithelium. Conjunctival nevi usually show mildly hyperreflective epithelium of normal thickness, internal hyperreflectivity, and intralesional cysts which is the hallmark of this tumour. Primary acquired melanosis presents with normal thickness epithelium, basal epithelial hyperreflectivity, and absence of cysts. Conjunctival melanoma demonstrates hyperreflective normal/thickened epithelium, hyperreflective basal epithelium, internal hyperreflectivity, and absence of intralesional cysts. Conjunctival lymphoma shows homogenous, low-medium reflective subepithelial lesions with smooth borders, and dot-like infiltrates. Benign reactive lymphoid hyperplasia findings are similar to lymphoma but the infiltrates are more hyperreflective compared to lymphoma. Pterygium shows thickened conjunctival epithelium, epithelial hyperreflectivity, and subepithelial wedge-shaped hyperreflective tissue separated from the overlying epithelium by a cleavage plane. Pinguecula demonstrates mildly thickened epithelium and similar findings with pterygium but does not extend beyond the corneal limbus. This review shows that AS-OCT, as a noninvasive tool, has potential uses in the differential diagnosis of ocular surface tumours and simulating lesions. Major limitations of AS-OCT include limited visualization of the posterior border of thick, keratinized, and pigmented tumours and lack of assessment of large conjunctival tumours in a single cut.

Bilateral Optic Nerve Head Drusen Complicated by Choroidal Neovascularization Misdiagnosed as Papilledema and Neuroretinitis.

We report a case of bilateral optic nerve head drusen complicated by choroidal neovascularization (CNV) in the left eye at presentation. The presence of optic disc and macular edema in addition to exudation led to the misdiagnosis of neuroretinitis at an outside medical center. Swept-source optical coherence tomography (SS-OCT) and SSOCT angiography were critical in establishing the diagnosis and follow-up in a noninvasive manner. Secondary CNV associated with optic nerve head drusen responded well to intravitreal injections of anti-vascular endothelial growth factor in the left eye. Asymptomatic nonexudative CNV that developed in the right eye during follow-up regressed spontaneously without treatment. [Ophthalmic Surg Lasers Imaging Retina 2022;53:518-521.].

Hamartomas of the Retina and Optic Disc.

Hamartomas are local malformation of cells that demonstrate abnormal proliferation in the area where they are normally present. Retinal and optic disc hamartomas include astrocytic hamartoma, congenital hypertrophy of the retinal pigment epithelium (CHRPE), simple congenital hamartoma of the retinal pigment epithelium (CSHRPE), combined hamartoma of the retina and retinal pigment epithelium (CHRRPE), retinal hemangioblastoma (retinal capillary hemangioma), and retinal cavernous hemangioma. Retinal and optic disc hamartomas can be observed sporadically as well as with systemic associations. Astrocytic hamartoma usually appears as a flat, transparent yellowish lesion. CHRPE is a round, pigmented, and flat lesion. CSHRPE usually presents as a dark black macular tumor. CHRRPE consists of vascular, glial, and pigment epithelial components, which can demonstrate peripapillary, macular, and peripheral localization. Retinal hemangioblastoma is a vascular tumor, red-pink in color with tortuous and dilated afferent and efferent vessels, typically located in the peripheral retina or optic disc. Retinal cavernous hemangioma is characterized by the formation of thin-walled saccular angiomatous structures in the retina or optic nerve head resembling concord grapes. Ultrasonography, fundus autofluorescence, optical coherence tomography, optical coherence tomography angiography, and fluorescein angiography methods are used in the diagnosis of retinal and optic disc hamartomas. Some retinal and optic disc hamartomas do not require treatment. However, complications including vitreous hemorrhage, macular exudation, retinal detachment, macular hole, epiretinal membrane, and choroidal neovascularization require treatment.

Surgical Approach in Intraocular Tumors

Surgery in intraocular tumors is done for excision/biopsy and the management of complications secondary to the treatment of these tumors. Excision/biopsy of intraocular tumors can be done via fine-needle aspiration biopsy (FNAB), transretinal biopsy (TRB), partial lamellar sclerouvectomy (PLSU), and endoresection. FNAB, TRB, and PLSU can be used in tumors that cannot be diagnosed by clinical examination and other ancillary testing methods. PLSU is employed in tumors involving the iridociliary region and choroid anterior to the equator. Excisional PLSU is performed for iridociliary and ciliary body tumors with less than 3 clock hours of iris and ciliary body involvement and choroidal tumors with a base diameter less than 15 mm. However, for biopsy, PLSU can be employed with any size tumor. Endoresection is a procedure whereby the intraocular tumor is excised using vitrectomy techniques. The rationale for performing endoresection is based on the fact that irradiated uveal melanomas may cause complications such as exudation, neovascular glaucoma, and intraocular pigment and tumor dissemination (toxic tumor syndrome), and removing the dead tumor tissue may contribute to better visual outcome. Endoresection is recommended 1-2 weeks after external radiotherapy. Pars plana vitrectomy is also used in the management of complications including vitreous hemorrhage, retinal detachment, and epiretinal membrane that can occur after treatment of posterior segment tumors using radiotherapy and transpupillary thermotherapy. It is important to make sure the intraocular tumor has been eradicated before embarking on such treatment.

Secondary endoresection for previously treated choroidal melanomas with a non-responsive course and persistent exudative retinal detachment.

AIM: To evaluate the results and complications of secondary endoresection via pars plana vitrectomy for choroidal melanoma and review the previously reported endoresection studies on the treatment of choroidal melanoma. METHODS: The medical records of 6 patients with choroidal melanoma who underwent secondary endoresection between March 2012 and March 2020 were retrospectively reviewed. The indications for secondary endoresection were progressive or recurrent tumor and severe exudative retinal detachment after previous treatment with plaque radiotherapy/Cyberknife radiosurgery/transpupillary thermotherapy (TTT). RESULTS: Before endoresection, 2 eyes had Iodine-125 plaque radiotherapy and TTT, 1 eye had Ruthenium-106 plaque radiotherapy and TTT, 1 eye had Cyberknife radiosurgery and TTT, 1 eye had Cyberknife radiosurgery, and 1 eye had TTT only. Preoperative visual acuity ranged from 20/63 to 20/1600 (Snellen) and from 0.5 to 1.9 (mean: 1.1) on the logMAR scale. The mean tumor base diameters were 9.5×8.7 mm and the mean tumor thickness was 5.4 mm. After secondary endoresection, transient vitreous hemorrhage developed in 2 (33.3%) eyes and retinal detachment in 1 (16.7%) eye. Cytopathological examination revealed epithelioid cell melanoma in 4 (66.7%) eyes and mixed cell melanoma in 1 (16.7%). Melanoma cell type was not specified in 1 (16.7%) eye. At a mean follow-up of 49.6mo (range: 16-90mo), mean visual acuity did not improve and 1 eye was enucleated due to tumor recurrence. Final visual acuity ranged from 20/63 to 20/1600 (Snellen) and from 0.5 to 1.9 (mean: 1.2) on the logMAR scale. Two patients with choroidal melanoma developed metastasis and eventually expired. CONCLUSION: Secondary endoresection seems to be an effective treatment option for globe salvage in choroidal melanoma not responsive to conventional treatment and displaying persistent exudative retinal detachment. There was no visual acuity increase among the treated eyes but globe salvage was possible in most cases in this study.

Varicella-zoster virus infection presenting with unilateral keratouveitis and papillitis in an immunocompetent adult.

We report a case of unilateral varicella-zoster virus (VZV) related keratouveitis and papillitis. A 54-year-old male presented with headache, left forehead rash, and decreased vision in the left eye. Examination of the left eye showed visual acuity (VA) of 0.15, relative afferent pupillary defect, anterior stromal corneal edema, nongranulomatous keratic precipitates, a swollen and hyperemic optic disc. The patient could read 6/12 of the Ishihara plates in the left eye. Optical coherence tomography (OCT) showed optic disc edema and OCT angiography (OCTA) revealed increased peripapillary vascularity. Computerized visual field testing demonstrated an enlarged blind spot and lower altitudinal defect. Serum anti-VZV IgM antibody level was elevated. The patient was put on oral acyclovir and prednisone treatment. At 1-month follow-up visit, VA increased to 0.9 and color vision was 11/12 Ishihara plates. OCT showed resolution of optic disc edema and OCTA demonstrated normalization of the increased peripapillary vascularity. Computerized visual field test demonstrated improvement in the lower altitudinal visual field defect and normalization of the enlarged blind spot. Optic nerve involvement in VZV infection occurs rarely, especially in immunocompetent patients as in our case.

Partial lamellar sclerouvectomy surgery for anteriorly located uveal tumour resection: a 20-year experience.

OBJECTIVES: To investigate the results of partial lamellar sclerouvectomy (PLSU) for anteriorly located uveal tumours. METHODS: We reviewed the tumour features, histopathologic findings, complications, visual acuity outcomes, eye preservation, metastasis, and mortality data of 56 cases with uveal tumours who underwent PLSU between February 1999 and February 2019. RESULTS: The mean largest tumour base diameters were 5.8 × 3.4 mm and the mean tumour thickness was 3.3 mm. Histopathologically, 30 (53.6%) eyes had malignant melanoma, 13 (23.2%) had nevus, 5 (8.9%) had iris stromal cyst, 4 (7.1%) had melanocytoma, 2 (3.6%) had Fuchs' adenoma, 1 (1.8%) had iris pigment epithelial cyst, and 1 (1.8%) had invasive breast cancer metastasis. The most common postoperative complications included cataract in 21 (37.5%) eyes, vitreous haemorrhage in 15 (26.8%), scleral thinning in 10 (17.9%), and hyphema in 6 (10.7%). At a mean follow-up of 40.4 (range: 10-201) months, tumour recurrence was observed in 2/30 (6.7%) eyes with melanoma and 1/5 (20.0%) eye with iris stromal cyst. Eyes with recurrent melanoma were treated with enucleation. Liver metastasis developed in only 1 (3.3%) melanoma case. All patients were alive at the end of follow-up. CONCLUSIONS: PLSU is a successful treatment method for many anteriorly located uveal tumours. In our series, the overall tumour recurrence and globe salvage rates were 5.4% and 96.4% respectively. Among melanoma cases, the metastasis rate was 3.3% and survival rate was 100%.

Recurrence and new tumor development after frontline intravenous chemotherapy for retinoblastoma: Risk factors and treatment results.

PURPOSE: To evaluate the risk factors leading to recurrence and new tumor (NT) development in patients with retinoblastoma after intravenous chemotherapy (IVC) and to review the treatment outcomes. MATERIALS AND METHODS: The records of 166 retinoblastoma cases (having 246 affected eyes) who underwent six-cycle IVC (vincristine, etoposide, and carboplatin) as primary treatment between October 1999 and August 2020 were reviewed retrospectively. RESULTS: The mean ages at presentation were 9.0 (median: 8.0) and 9.2 (median: 8.5) months in cases with recurrence and NTs respectively. Recurrence was detected in 40 (16.3%) eyes, NTs in 29 (11.8%), and both recurrence/NTs in 24 (9.8%). The mean time elapsed till recurrence and NT was 10.7 months. Multivariable analysis showed that the factors predictive of recurrence were largest tumor base diameter (LTBD) >12 mm (p = 0.039) and presence of subretinal seeds at diagnosis (p = 0.043). Multivariable risk factors for the development of NTs were bilateral familial retinoblastoma (p = 0.001) and presence of subretinal seeds at diagnosis (p = 0.010). Mean follow-up was 80.1 (median: 72.5) months. By Kaplan-Meier analysis, the 1-, 3-, and 6-year recurrence and NT rates were 21.2%, 28.1%, and 28.7% and 14.9%, 22.6%, and 23.9% respectively. The most common treatment methods used for recurrent and/or NTs included cryotherapy, transpupillary thermotherapy, and intra-arterial chemotherapy. Enucleation was eventually required in 24/93 (25.8%) eyes. No patient developed metastasis. DISCUSSION: Development of recurrence and/or NT after IVC was noted in 38% of all retinoblastoma eyes. Bilateral familial disease, LTBD >12 mm, and presence of subretinal seeds at baseline were risk factors for recurrence and NTs in this study.

Demographic and clinical features of conjunctival tumours at a tertiary care centre.

CLINICAL RELEVANCE: This study investigates the demographic and clinical features of conjunctival tumours. BACKGROUND: Conjunctival tumours include a large spectrum of conditions ranging from benign lesions to aggressive, life-threatening malignancies. Knowing the distribution of conjunctival tumours by age and gender is important for reducing cancer morbidity. METHODS: The clinical records of 375 patients (410 eyes) diagnosed with a conjunctival mass at a tertiary referral centre between February 1999 and November 2020 were retrospectively evaluated. RESULTS: Two-hundred-seventeen (57.9%) patients were male and 158 (42.1%) were female. Of 410 conjunctival tumours, 159 (38.8%) were benign, 106 (25.9%) premalignant, and 145 (35.4%) malignant. Overall, the 3 most common diagnoses were squamous cell carcinoma (SCC, 19.5%), conjunctival intraepithelial neoplasia (CIN, 18.3%), and naevus (17.8%). The most common benign, premalignant, and malignant tumours were naevus (n = 73/159, 45.9%), CIN (n = 75/106, 70.8%), and SCC (n = 80/145, 55.2%) respectively. Naevus was the most common tumour in ≤20 years and > 20-40 years old patient groups (56.2% and 25.4% respectively). CIN was the most frequent tumour in patients aged > 40-60 years (25.7%). SCC was the most common tumour in > 60-80 years and > 80 years old patient groups (44.3% and 80.0% respectively). The median patient age was greater in patients with malignant tumours (64.5 years) compared to patients with premalignant (55.5 years, p = 0.011) and benign tumours (22.0 years, p < 0.001). Malignant tumours displayed larger base diameter, greater thickness, and intrinsic vessels compared to premalignant or benign lesions (p < 0.001 for each parameter). Malignant tumours also displayed more amelanotic vs melanotic appearance (p < 0.001) and limbal vs extralimbal bulbar location compared to benign lesions (p < 0.001). CONCLUSION: Premalignant and malignant tumours comprised 61.2% of all conjunctival tumours and were usually detected in patients > 40 years of age in this study.

Transretinal biopsy via 23-gauge pars plana vitrectomy for retinal and choroidal tumors: cytopathological results, surgical complications, and patient outcomes.

PURPOSE: To evaluate the cytopathological results, surgical complications, and patient outcomes after transretinal biopsy (TRB) for diagnosis of retinal/choroidal tumors METHODS: Records of 40 cases who underwent TRB via 23-gauge (23 G) pars plana vitrectomy between March 2011 and March 2020 were reviewed. STUDY DESIGN: Retrospective. RESULTS: Twenty-six (65.0%) cases were women and 14 (35.0%) were men. The mean age at diagnosis was 57.2 (range: 18-83) years. The mean tumor base diameter was 12.0×9.8 mm and the mean tumor thickness was 4.9 mm. According to cytopathological examination, 29 (72.5%) cases had choroidal melanoma, 2 (5.0%) had non-small cell lung cancer metastasis, 1 (2.5%) had adenoma of retinal pigment epithelium, 1 (2.5%) had small cell lung cancer metastasis, 1 (2.5%) had invasive breast cancer metastasis, 1 (2.5%) had retinal astrocytic hamartoma, and 1 (2.5%) had pseudoneoplastic gliosis. Cytopathological examination of 4 (10.0%) cases revealed findings consistent with macular and extramacular degeneration. Postoperative complications were mild vitreous hemorrhage in 16 (40.0%) cases, gradually worsening cataract in 4 (11.8%), retinal detachment in 1 (2.5%), hyphema in 1 (2.5%), glaucoma in 1 (2.5%), and macular hole in 1 (2.5%). During the mean 11.1 (range: 1-55) months follow-up, 1 (3.4%) patient with choroidal melanoma developed liver metastasis. All patients were alive at the end of follow-up. CONCLUSIONS: TRB using 23 G pars plana vitrectomy can be used to make the cytopathologic diagnosis of retinal/choroidal tumors whenever the clinical diagnosis is not certain or in cases with known diagnosis to obtain information on cell type/cytogenetics. In our series, the most common diagnosis after cytopathologic examination was choroidal melanoma.

Presumed Retinal Metastasis from Lung Adenocarcinoma: A Case Report and Literature Review.

A 63-year-old Caucasian man with metastatic lung adenocarcinoma undergoing chemotherapy and external radiotherapy was referred for routine eye examination. Although he was asymptomatic, ocular examination revealed a relatively well-circumscribed whitish retinal lesion measuring 0.5 × 0.5 × 0.5 mm located along the inferotemporal vascular arcade in the right eye. Optical coherence tomography (OCT) showed a hyperreflective dome-shaped lesion occupying the inner retinal layers with few hyperreflective dots overlying the retina in the posterior vitreous consistent with tumor cells. Fluorescein angiography revealed early hyperfluorescence and late staining without leakage at the lesion site. A diagnosis of presumed retinal metastasis from lung adenocarcinoma was made. At 2 months follow-up after completion of chemotherapy, the retinal lesion was found to have regressed completely leaving minor irregularities in the inner retinal layers on OCT. To date, there have been only 41 cases of carcinoma metastasis to the retina reported in the literature including the current case. Despite its rarity, retinal metastasis should be considered in the differential diagnosis of a white-yellow retinal mass with/without overlying vitreous cells especially in patients with a history of systemic cancer.