Ocular Oncology and Pathology最新文献

Introduction: Since 2003, using the Surveillance, Epidemiology, and End Results (SEER) database, epidemiological aspects of uveal melanoma have been reported. The aim of this study was to update trends in incidence, treatment, and survival of uveal melanoma in the USA from 1975 to 2020.

Methods: Patients were identified using International Classification of Disease for Oncology codes: C69.3 [choroid], C69.4 [ciliary body and iris], and C69.2 [retina]. Trends in age-adjusted incidence, treatment (surgery or radiation), and 5-year relative survival were calculated.

Results: A total of 5,563 cases of uveal melanoma were identified. The majority (97%) were reported by hospital inpatient/outpatient clinics. Microscopic (histopathologic or cytologic) confirmation was available in 61%. The mean age-adjusted incidence was 5.6 per million (95% CI: 5.5-5.7). As previously noted, a small but statistically significant (p < 0.05) annual percentage change of 0.5% was detected in whites. The previously reported declining trend in the number of patients treated with surgery alone (93% from 1975 to 1977 vs. 21% from 2017 to 2020) ensued, with ongoing corresponding increasing rates of radiation as the primary treatment (1% from 1975 to 1977 vs. 58% from 2017 to 2020). No change in the 5-year relative survival (82.8%) was observed (reported from 1975 to 2016).

Conclusions: Previously reported overall age-adjusted incidence of uveal melanoma is stable throughout the years, with a minor increase in incidence in whites. Treatment trend toward radiation has not led to improvement in survival.

Introduction: This study evaluated the association between gene expression profiling (GEP), PRAME (preferentially expressed antigen in melanoma), and regression rate of uveal melanoma after proton beam irradiation (PBI).

Methods: A retrospective review of uveal melanoma patients treated with PBI between 2013 and 2021, with GEP results and at least 3 post-radiation ultrasound measurements, was conducted. Patients with local recurrences were excluded. Regression rates were analyzed using a linear mixed model to predict percentage change in thickness from baseline. Cox regression was conducted to determine whether slow or fast regression, based on the median regression rate at 18 months, correlates with metastasis risk.

Results: The study included 106 patients, with GEP classifications of 1A in 43.4%, 1B in 25.5%, and 2 in 31.1%. Overall, the mean change in tumor thickness was 20.9%, 35.1%, 51.4%, and 59.3% at 1 year, 2 years, 4 years, and 6 years, respectively. No differences in regression rates between GEP classes and PRAME expression were found through 72 months post-PBI. The median regression at 18 months was 27.1%. Slow and fast regression was not associated with the risk of metastasis.

Conclusion: No association between GEP, PRAME, and regression rate was found through 72 months post-PBI.

Introduction: The aim of the study was to describe clinical features of lipid dominant toxic tumor syndrome (TTS) and report outcomes following use of intravitreal steroids.

Methods: Records of 13 patients who had lipid dominant TTS following treatment of choroidal melanoma with episcleral plaque brachytherapy (EPB) were retrospectively reviewed. Resolution of lipid exudates, subfoveal subretinal fluid, cystoid macular edema (CME), exudative detachment were main outcome measures.

Results: Of the 13 patients who developed lipid dominant TTS, 11 (85%) had medium-sized melanomas, and 2 (15%) small-sized melanomas. The average time to onset following EPB was 22 months (range 3-48 months). Seven patients (54%) were noted to have dyslipidemia. The baseline visual acuity at the time of diagnosis of TTS was 50 ETDRS letters (range 10-85). Ophthalmic characteristics were lipid exudates centered around the tumor base in 13 (100%) patients, subfoveal subretinal fluid in 4 (31%) patients, and CME in 2 (15%) patients. Exudative detachment was absent in all (100%) patients. Regressed melanoma was present in all (100%) patients. Eight (62%) patients were treated with intravitreal steroids (4 mg triamcinolone), while 5 patients (38%) were observed. The response to intravitreal steroids was noted in 7(88%) of the treated patients, with the average time to response being 1.9 months. Features characterizing a positive response were reduction in lipid exudates centered around the tumor base (100%), reduction in subfoveal subretinal fluid (100%), and reduction in CME (50%). Cataract development was seen in 10 (83%) and ocular hypertension in 3 patients (23%). Proliferative radiation retinopathy developed in 2 (15%) patients, neovascular glaucoma developed in 1(8%) while no patients required enucleation.

Conclusion: The lipid dominant TTS centered around the tumor base that occurs in a radiation responsive tumor could be considered a chronic variant in the spectrum of TTS. Intravitreal steroids in selected cases reverse the course of this variant, stabilizing or improving the vision and avoiding enucleation. Our observations would need to be verified through a larger prospective study.

Introduction: Sentinel vessels provide an important early indication of underlying ocular neoplasm. To date, there is no noninvasive technique available for imaging and mapping of their vascular supply, which remains largely unstudied. We aimed to map sentinel vessels in uveal melanomas noninvasively by laser speckle contrast imaging (LSCI).

Case presentations: This report describes a case series of 4 patients undergoing enucleation due to uveal melanoma. Perfusion was imaged using LSCI during the successive detachment of the four rectus muscles, with their ciliary arteries, and the optic nerve with its ophthalmic artery. Tumor location and possible extrascleral growth were analyzed histopathologically. Sentinel vessels in uveal melanoma could be visualized noninvasively using LSCI, appearing broader and tortuous compared to surrounding vessels. The perfusion in the sentinel vessels was 24-94% higher than in the episcleral vessels. The origin of the sentinel vessel could be determined by perfusion monitoring during the successive detachment of the rectus muscles and the optic nerve. In 3 patients, the sentinel vessel was supplied by the anterior ciliary arteries and in 1 patient by the ophthalmic artery. In one of the cases, the sentinel vessel was not visible upon visual inspection.

Conclusion: This is the first study of its kind demonstrating detailed mapping of sentinel vessels in uveal melanoma using LSCI. LSCI shows potential for the detection of sentinel vessels before they are visible in slit lamp examination. Vascular changes are a hallmark of tumor growth, and noninvasive imaging with LSCI may be a useful diagnostic tool for the detection of uveal melanoma.

Introduction: To report two cases of primary retinal ependymoma occurring in adults, both enucleated following initial suspicion of uveal melanoma. Uveal melanoma is the most frequent primary tumor of the eye in adults but other rare benign intraocular tumors such as leiomyoma, adenoma or ependymomas may also occur. To diagnose them properly is critical to adapt the patient's management, prognosis and follow-up. Ependymomas are exquisitely rare glial tumors arising from the retina, with only three cases reported yet. Primary clinical diagnosis and distinction between benign or malignant tumor is usually not possible without enucleation and histopathologic analysis. Ependymomas are glial tumors, usually occurring during childhood and arising within the central nervous system (CNS, brain or spinal cord). However, they may also occur outside the CNS, either as a primary or a secondary location.

Case presentations: Case series from two adults aged 41 and 42 years with blindness and painful eye due to neovascular glaucoma who were enucleated for intraocular tumors. Imaging demonstrated in both cases nonspecific vascularized solid tumors consistent with uveal melanoma. Histopathologic findings showed radial arrangement of cuboidal to elongated tumor cells around hyalinized fibrovascular cores, presence of myxoid material and diffuse expression of GFAP but not Olig2, orienting the diagnosis to myxopapillary retinal ependymomas. No primary of distant CNS location was identified and follow-up at one and 3 years was favorable.

Conclusions: Intraocular ependymoma is a rare intraocular primary retinal tumor which can mimic uveal melanoma at ocular fundus examination and imaging. The diagnosis requires histopathology after enucleation, and can mimic reactive retinal gliosis, whether ependymomas can arise from retina or if retinal gliosis can have ependymoma characteristics is still to be determined. Survival and metastatic prognosis after surgery are favorable according to our experience and previously published cases, confirming the presumed benign nature of the lesions.

Introduction: Female representation in ophthalmic oncology and pathology is unknown. We evaluated gender-based trends in authorship and leadership in these fields, focusing on intraocular tumors.

Methods: Articles containing "uveal melanoma" (UM), "retinoblastoma" (Rb), "primary intraocular lymphoma" (PIOL), "choroidal metastasis" (CM), and articles in Ocular Oncology and Pathology (OOP) were searched on PubMed and author names obtained. Leadership/awards from 4 international ophthalmic oncology and pathology societies were recorded. GenderAPI was used to predict gender.

Results: Among 4,245 UM, 9,660 Rb, 141 PIOL, 353 CM, and 568 OOP authors, more males were first (range, 52-67%) and last authors (range, 69-76%). Analysis of the 4 keywords showed overall female authorship increased from 2011 to 2016 to 2017-2022 (35%-40%, p < 0.001), with an increase in both first (45%-47%, p = 0.03) and last authorship (26%-33%, p < 0.001). Female first authors were more likely to publish with female last authors (Rb OR 1.72, p < 0.001; OOP OR 2.04, p = 0.01). Males held more leadership roles/awards in societies.

Conclusion: Female representation in ophthalmic oncology and pathology has grown but gender disparities remain. Female first authors were more likely to publish with female last authors. Most society leaders and awardees were male. Cultivating mentorship through professional organizations may help achieve gender parity.

Introduction: Ruthenium-106 brachytherapy is a primary treatment for uveal melanoma (UM), the most common intra-ocular malignancy in adults. This study evaluated the safety of Ru-106 applicators at 3 Tesla (T) MRI and their impact on image quality.

Methods: Magnetic attraction and eddy currents were tested on a 20-mm-diameter Ru-106 applicator using a nylon string and a porcine eye. Safety criteria were defined by ocular oncologists, comparing magnetic field interactions to the forces exerted on the eye during surgery. Five UM patients were scanned at 3T MRI with the applicator in situ using both conventional anatomical sequences and scans optimised to reduce metal artefacts.

Results: Minimal magnetic interactions were observed. Eddy currents caused slight lagging during fast movements and temporary detachment of the applicator of the porcine eye in conditions that were considered unrealistic for clinical scans. Significant susceptibility artefacts compromised image quality of the affected eye.

Conclusion: Patients with Ru-106 applicators can be safely used in 3T MRI with some simple precautions. MR image quality of the eye was poor due to major susceptibility artefacts; however, imaging of extra-ocular anatomy is feasible.

Background: Retinoblastoma (Rb) is the most common intraocular malignancy of childhood. The prognosis in Rb directly relates to the spread of disease beyond the eye, particularly to the central nervous system. Therefore, until the recent past, surgically entering an eye with active or recently active Rb was absolutely contraindicated in most centres around the world due to the risk of iatrogenic extraocular spread.

Summary: In this review article, we explore the role of intraocular surgery in Rb. We describe the techniques which have been developed in order to safely deliver chemotherapeutic agents into the eye, both intravitreal and intracameral. We discuss the role of vitrectomy and endoresection for treatment of vitreous seeds and active retinal tumours. In the context of previously treated Rb, we outline various surgical interventions performed to improve visual outcomes or to manage the ocular sequelae of the disease. In particular, we review the various techniques used to maximise the safety of cataract, retinal and glaucoma surgery in children with a history of Rb.

Key messages: The role of intraocular surgery in Rb is now well established, particularly in the delivery of chemotherapeutic agents or in the presence of previously treated diseases. The role of intraocular surgery in eyes with active Rb is less well established and remains the subject of much debate. However, it is likely that with further development of safer surgical techniques, the indications and modalities will continue to broaden.

Introduction: The aim of this study was to differentiate amelanotic choroidal lesions - amelanotic choroidal melanoma, choroidal metastasis, and choroidal granuloma using multimodal imaging.

Methods: Retrospective comparative chart review was done. Patients diagnosed with the abovementioned choroidal lesions from 2015 to 2022 were included. Baseline lesion morphology and retinal layer changes on optical coherence tomography (OCT), lesion echogenicity and dimensions on ocular ultrasonography (USG), fundus autofluorescence (FAF), fundus fluorescein angiography (FFA), and indocyanine green angiography (ICG-A) patterns and findings were assessed.

Results: Twelve eyes with melanoma, 22 eyes with metastasis, and 9 eyes with granuloma were included. On OCT, 83% of melanomas and 67% of granulomas, and 68% of metastasis had dome-shaped choroidal lesions. Presence of intraretinal fluid (IRF) and shaggy photoreceptors was 94% (95% CI: 79-99%) and 90% (95% CI: 74-98%) specific to differentiate melanomas from other choroidal lesions (AUC >0.75, p < 0.05). Similarly, presence of incomplete retinal pigment epithelium (RPE) and outer retinal atrophy (iRORA) was 77% (95% CI: 55-92%) sensitive and 77% (95% CI: 53-92%) specific (AUC = 0.8, p+ 0.03) and presence of lumpy-bumpy choroid was 55% (95% CI: 32-76%) sensitive and 95% (95% CI: 76-99%) specific (AUC = 0.75, p = 0.04) to distinguish metastasis from other choroidal lesions. Mean height: base ratio was more in melanoma compared to metastasis (0.54 ± 0.22 vs. 0.43 ± 0.12, p = 0.17) and both were hyperechoic on USG. On FAF, 100% metastasis and 60% granulomas were hypoAF, whereas 100% melanomas were hypoAF. Majority choroidal lesions were hyperfluorescent on FFA. Double vascular circulation was observed in melanomas (50%). On ICG-A, all choroidal lesions were hypofluorescent.

Conclusion: Clinical and multimodal imaging features such as the presence of IRF, shaggy photoreceptors, iRORA, and lumpy-bumpy choroid can help diagnose and differentiate amelanotic choroidal lesions, thereby avoiding the need for choroidal biopsy. Further larger studies are needed to devise a standard imaging protocol to validate our findings.