[Adenocarcinoma of nonpigmented ciliary body epithelium (clinical cases)]. / Adenokartsinoma iz bespigmentnogo epiteliya tsiliarnogo tela (klinicheskie nablyudeniya).

The article presents two clinical cases of adenocarcinoma of nonpigmented epithelium of the ciliary body, which is a very rare malignant tumor of the organ of vision with distinctive features. Surgical treatment is necessary to verify this tumor and assess the degree of its aggressiveness in terms of the prognosis of the disease, with subsequent pathomorphological and immunohistochemical studies. The article also discusses the epidemiological aspects, morphological features, clinical manifestations of this pathological condition, as well as possible treatment options and features of follow-up monitoring of this group of patients.

[Doppler ultrasound in the assessment of blood supply to choroidal melanoma: parallels with contrast angiography and histography]. / Ul'trazvukovaya dopplerografiya v otsenke krovosnabzheniya melanomy khorioidei: paralleli s kontrastnoi angiografiei i gistografiei.

PURPOSE: Evaluation of blood supply to choroidal melanoma based on comparison of Doppler characteristics of blood flow with angiographic variant of angioarchitectonics and densitometric parameters of the tumor. MATERIAL AND METHODS: The study was performed in 135 patients (135 eyes) with choroidal melanoma. The initial prominence of the tumors varied from 0.6 to 15.2 mm (mean 5.07±3.58 mm), the diameter of the tumor base varied from 4.1 to 22 mm (mean 10.97±3.62 mm). Taking into account the biometric characteristics of choroidal melanoma, all patients were divided into 3 groups: «small¼ (n=49), «medium¼ (n=34) and «large¼ (n=52). In addition to standard diagnostic examination, the following instrumental methods for assessing the blood supply of choroidal melanoma were carried out: angiography with indocyanine green, optical coherence tomography angiography, ultrasound in color Doppler mapping mode, ultrasound histography. RESULTS: Comparative analysis of Doppler ultrasound and contrast angiography data in assessing the blood supply of choroidal melanoma established that the first angiographic type, presented by straight and parallel vessels (65%, p=0.037), is characteristic for hypovascular and avascular masses, the second type - for hypervascular choroidal melanomas, in which the new vessels can take the form of arches, loops and nets (68%, p=0.027). The study of densitometric characteristics in choroidal melanoma of various sizes indicates a natural decrease in the acoustic density of the tumor tissue with increase in the prominence of the mass, while there are significant differences in the acoustic density values in hypo/avascular (36.53±5.37 dB) and hypervascular variants (29.28±4.53 dB) of blood supply to tumor tissue. CONCLUSION: The obtained data on acoustic density of choroidal melanoma can be used in clinical practice for indirect assessment of the nature of blood supply to choroidal melanoma.

[Optical coherence tomography angiography in ophthalmic oncology]. / Opticheskaia kogerentnaia tomografiia-angiografiia v oftal'moonkologii.

The article reviews literature on various methods of optical coherence tomography (OCT). The review also describes clinical aspects of OCT angiography and its applications in ophthalmic oncology.

A change roadmap towards research paradigm in low-resource countries: retinoblastoma model in Egypt.

Research on childhood diseases represents a great global challenge. This challenge is maximized in both childhood cancer disciplines and developing world. In this paper, we aim at describing our institution experience in starting a structured childhood cancer research program in one of the developing countries in a short time based on philanthropic efforts. We used retinoblastoma as an example for what was conducted in this program. Starting in 2008, this program included improving clinical practice and its related supporting services besides developing new research services that both complement the clinical activities and pave the way towards creating a research foundation in the country. Results included developing hospital standard treatment protocols, developing national clinical trials, joining international consortia for childhood cancers clinical trials, developing data collection tools and real-time analytics, establishing a biobanking facility, and developing highly qualified team for conducting clinical, epidemiologic, and translational research studies. Moreover, this effort resulted in improving both clinical practice and patients' awareness nationally. This model can be used for other startup facilities that aim at finding answers for their national health problems in low-resource setting.

Primary Subretinal Seeding in Retinoblastoma: Clinical Presentation and Treatment Outcomes.

Introduction: The aim of this study was to describe the clinical features and treatment outcomes of primary subretinal seeding (SRS) in patients with intraocular retinoblastoma (RB). Methods: Descriptive analysis of primary SRS in 47 patients (50 eyes) with RB was performed. Results: Mean age was 19 months (range, 2-72 months), and 55% (n = 26) of the subjects were male. At presentation, the SRS involved two or more quadrants in 88% of eyes. Most seeds appeared yellowish gray (66%) and round to oval in shape (48%). Two-thirds of SRS were seen posterior to the equator and within 5 mm from the main tumor. Associated features included subretinal fluid in 50 eyes (100%), total retinal detachment in 28 eyes (56%), and vitreous seeds in 20 eyes (40%). Treatment included intravenous chemotherapy (IVC) (n = 47; 94%), enucleation (n = 2; 4%), and intra-arterial chemotherapy (n = 1; 2%). SRS treatment included adjunct use of focal transpupillary thermotherapy and/or cryotherapy (n = 20; 40%). Retinal tumor control was achieved in 36 eyes (76%) with 32 eyes (78%) showing a type 3 regression pattern, while SRS completely regressed in 24 (48%) eyes, partially in 15 (30%) and worsened in 2 (4%) eyes. Over a mean follow-up of 30 months (range, 3-68 months), SRS recurrence was noted in 12 eyes (29%), globe salvage was achieved in 39 eyes (78%), and 1 (4%) patient died of presumed metastasis. Conclusion: Primary SRS pose a therapeutic challenge during RB treatment. The SRS responds moderately to systemic IVC, with one-third cases showing SRS recurrence and one-fifth ultimately requiring enucleation.

Retinal Imaging and Optical Coherence Tomography of Choroidal Haemangioma Mimicking Central Serous Chorioretinopathy.

Intraocular tumours, such as choroidal haemangioma, can cause exudative retinal detachments, which mimic central serous chorioretinopathy. Key symptoms of a choroidal haemangioma include reduced visual acuity, visual field defects, and metamorphopsia. More rarely, it can cause photopsia, myodesopsia, and pain. Important differentials include choroidal melanoma and metastases, for which an ocular oncologist should be consulted. Prompt treatment is required for the regression of tumour and to prevent choroidal atrophy and permanent visual loss. Here, we report the case of a 44-year-old lady who was found to have a choroidal haemangioma with macular subretinal fluid, highlighting the differentiating features from other intraocular masses.

Assessing Choroidal Nevi, Melanomas and Indeterminate Melanocytic Lesions Using Multimodal Imaging-A Retrospective Chart Review.

Using multimodal imaging, the literature proposed the following risk factors for choroidal nevus growth into melanoma: increased tumor thickness, subretinal fluid, decreased visual acuity, presence of orange pigment, ultrasound acoustic hollowness, and increased tumor diameter. This study investigated the presence of the mentioned risk factors in choroidal nevi, choroidal melanomas, and indeterminate choroidal melanocytic lesions. This retrospective, single-center chart review assessed choroidal melanocytic tumors with multimodal imaging. We defined our primary outcome as the cumulative presence of mentioned risk factors. Further, we evaluated various optical coherence tomography (OCT), ultrasound, and autofluorescence findings. We analyzed 51 tumors from 49 patients during the period from April 2008 to June 2021. The median (IQR) age was 64.0 (56.0 to 70.5) years, with 23 of 49 (46.9%) patients being female. The follow-up time for all tumors was median (IQR) 25.0 (12.0 to 39.0) months. The choroidal nevi had a median (range) risk score of 0.0 (0.0 to 3.0), and the choroidal melanoma of 5.0 (3.0 to 6.0), with statistically significant different ratings (p < 0.001). Multimodal imaging creates a score that may help to distinguish choroidal nevi from choroidal melanomas objectively.

Intravenous Chemotherapy for Retinoblastoma in the Era of Intravitreal Chemotherapy: A Systematic Review.

PURPOSE: The published data on ocular survival following intravenous chemotherapy of retinoblastoma (RB) seems to be skewed by evolving practice patterns induced by use of intravitreal chemotherapy (iVitc). We aimed to explore potential role of iVitc for vitreous seeding for patients treated with intravenous chemotherapy (IVC). METHODS: A literature search was performed to identify cases of RB treated with primary IVC prior to advent of iVitc by various search engines (PubMed, Medline, and Google) from 1992 to 2018. Studies were excluded if number of cases were less than 40 or lacked data related to type of recurrence and its treatment. Rates and patterns of recurrence and its management were categorized. RESULTS: Out of 15 studies identified, only 10 studies (797 eyes) met the inclusion criteria. The mean age at presentation was 15.3 months (range 0-192.8 months). Unilateral cases represented 25% of the cohort. The ocular survival rate with primary IVC was 63% (500/797 eyes). Of the 297 eyes (37%) that failed IVC therapy, additional 99 eyes could be salvaged with EBRT (599/797 eyes, 75%). Remaining 198 eyes were enucleated (198/797 eyes 25%). K-M survival analysis could not be done due lack of sufficient data. Recurrences that occurred (mean 12.2 months) after completion of primary IVC included relapse of retinal tumor (143 eyes [48%]), vitreous seeding (73 eyes [25%]), subretinal seeding (49 eyes [16%]), or any combination (103 eyes [35%]). Out of 73 eyes with vitreous seeding, additional 66 eyes (90%) would have been salvaged with iVitc, potentially improving ocular survival rates to 71% (500 + 66/797). CONCLUSIONS: Evolving practice patterns of RB treatment have unfavorably skewed published ocular survival rates following IVC. With incorporation of iVitc, the ocular survival rates with IVC can be potentially improved to be non-inferior to those achieved with intra-arterial chemotherapy.

Distinguishing Choroidal Nevi from Melanomas Using the MOLES Algorithm: Evaluation in an Ocular Nevus Clinic.

OBJECTIVE: The aim of this study was to determine the sensitivity and specificity of the MOLES scoring system in differentiating choroidal melanomas from nevi according to Mushroom shape, Orange pigment, Large tumor size, Enlarging tumor, and Subretinal fluid (SRF). METHODS: Color photographs, fundus-autofluorescence images, and optical coherence tomography of 222 melanocytic choroidal tumors were reviewed. Each MOLES feature was retrospectively scored between 0 and 2 and tumors categorized as "common nevus," "low-risk nevus," "high-risk nevus," and "probable melanoma" according to the total score. MOLES scores were compared with the experts' diagnosis of melanoma. RESULTS: The MOLES scoring system indicated melanoma in all 81 tumors diagnosed as such by ocular oncologists (100% sensitivity) and nevus in 135 of 141 tumors given this diagnosis by these experts (95.7% specificity). Of the 6 tumors with discordant diagnoses, 4 had basal diameters exceeding 6 mm, all with SRF and/or orange pigment, and 2 small tumors showed either significant SRF with traces of orange pigment, or vice versa. CONCLUSIONS: The MOLES system for diagnosing melanocytic choroidal tumors compares well with expert diagnosis but needs to be evaluated when deployed by ophthalmologists and community optometrists in a wide variety of working environments.

High-Dose-Rate Interstitial Brachytherapy (Interventional Radiotherapy) for Conjunctival Melanoma with Orbital Extension.

PURPOSE: To evaluate local control and functional and cosmetic outcomes of postoperative high-dose-rate interventional radiotherapy (HDR-IRT) in patients affected by conjunctival melanoma with orbit invasion. METHODS: A retrospective study was conducted in 2 patients affected by conjunctival melanoma infiltrating the orbit, treated with surgical excision and HDR-IRT. The treatment procedures consisted of surgical excision of the orbital infiltrating nodule followed, 1 month after surgery, by adjuvant HDR-IRT. A target dose of 34 Gy was delivered in 10 twice-a-day fractions over 5 consecutive days. Data analysis included local tumor control and metastatic rate, acute and late toxicity, functional and aesthetic results. RESULTS: In both patients, treatment was well tolerated, and there was no orbital recurrence at a median follow-up of 37-40 months. There was an excellent functional outcome, without no significant acute or late side effects. CONCLUSIONS: HDR-IRT could be considered a promising, feasible, successful, and well-tolerated option for selected patients affected by ocular tumors with orbital invasion.

The Molecular Pathology of Eye Tumors: A 2019 Update Main Interests for Routine Clinical Practice.

Over the last few years, we have seen constant development of molecular pathology for the care of patients with cancer. The information obtained from molecular data has transformed our thinking about the biological diversity of cancers, particularly in the field of ophthalmic oncology. It has reoriented the way in which therapeutic decisions and decisions concerning patient surveillance are made, both in the area of pediatric cancers, including rhabdomyosarcoma and retinoblastoma, and adult cancers, such as uveal melanoma and lymphomas. A better definition of the molecular classification of these cancers and of the different biological pathways involved is essential to the understanding of both the pathologist and the onco-ophthalmologist. Molecular tests based on targeted or expanded analysis of gene panels are now available. These tests can be performed with tumor tissue or biofluids (especially blood) to predict the prognosis of tumors and, above all, the benefit of targeted therapies, immunotherapy or even chemotherapy. Looking for the BAP1 mutation in uveal melanoma is essential because of the associated metastatic risk. When treating retinoblastoma, it is mandatory to assess the heritable status of RB1. Conjunctival melanoma requires investigation into the BRAF mutation in the case of a locally advanced tumor. The understanding of genomic alterations, the results of molecular tests and/or other biological tests predictive of a therapeutic response, but also of the limits of these tests with respect to the available biological resources, represents a major challenge for optimal patient management in ophthalmic oncology. In this review, we present the current state of knowledge concerning the different molecular alterations and therapeutic targets of interest in ophthalmic oncology.