Ocular Oncology and Pathology最新文献

Introduction: Choroidal osteoma is a rare benign tumor where mature bone replaces the choroid. Possible causes include inflammation, trauma, hormones, disorders of calcium metabolism, environmental factors, genetics, or osseous choristoma. This paper discusses 4 cases and literature regarding choroidal osteoma occurring concurrently with or secondary to uveal pathologies including uveitis and pachychoroid spectrum.

Case presentations: In case 1, a 41-year-old man with central serous chorioretinopathy (CSCR) in both eyes (OU) developed a choroidal osteoma in the left eye (OS) 8 years after the initial visit. Type 1 macular neovascularization (MNV) developed 4 years later at age 53. In case 2, a 50-year-old woman with CSCR OU developed a choroidal osteoma OS 15 years after the initial visit. The lesion gradually enlarged over another 15 years of observation. In case 3, a 24-year-old woman with Vogt-Koyanagi-Harada disease treated with systemic corticosteroids for 6 months developed choroidal osteoma OU and type 2 MNV in the right eye (OD) 16 years after the initial visit. In case 4, a 55-year-old man with concurrent posterior scleritis and choroidal osteoma OS developed type 1 MNV 13 years after the initial visit. He had a history of unknown uveitis treated with high-dose corticosteroid therapy 21 years previously. In all 5 eyes, the presence of osseous tissue in the choriocapillaris and Sattler's layer was confirmed by optical coherence tomography, B-mode ultrasound, or computed tomography. These lesions demonstrated observed growth in basal diameter and/or maturation process of bone tissue throughout the follow-up period.

Conclusion: We observed 5 eyes of four patients with choroidal osteoma in the choriocapillaris and Sattler's layer of the choroid secondary to CSCR, Vogt-Koyanagi-Harada disease, or posterior scleritis over a long follow-up period of 12-30 years. Secondary choroidal osteoma, ectopic bone in the choroid, can result from the transformation of mesenchymal cells stimulated by osteoprogenitors, such as bone morphogenetic proteins. Secondary choroidal osteoma should be recognized as a rare long-term complication of uveal pathologies.

Background: Although uncommon, tumors of the iris and ciliary body present significant diagnostic challenges because of their variable appearance and overlap between benign and malignant features and the difficulty of directly visualizing lesions located in the posterior iris or ciliary body using conventional methods.

Summary: The advent of high-resolution anterior segment imaging, particularly ultrasound biomicroscopy (UBM), has greatly enhanced the ability of clinicians to evaluate these lesions in vivo. This review synthesizes current evidence on the utility of UBM in the diagnosis, classification, and management of both iris and ciliary body tumors. The emphasis is placed on differentiating melanotic from amelanotic lesions, recognizing imaging characteristics that suggest malignancy and comparing UBM with other anterior segment modalities, such as gonioscopy and anterior segment optical coherence tomography. This study provides a detailed discussion of tumor morphology, growth patterns, and secondary complications, supplemented by illustrative examples from clinical practice.

Key message: UBM serves as a noninvasive, reproducible, and dynamic view of lesions located deep within the anterior segment, particularly those obscured from direct examination, thereby guiding both diagnosis and longitudinal management.

Introduction: Renal cell carcinoma (RCC) is a rare cause of ophthalmic metastasis. Immune checkpoint blockers (ICBs) such as ipilimumab and nivolumab (ipi/nivo) are first-line therapies for advanced RCC. There are limited efficacy reports of ICBs for RCC choroidal metastases (CMs).

Case one: A 43-year-old male with metastatic (lung) clear cell RCC presented with left eye scleritis and a 3.4 mm choroidal mass. One week after starting ipi/nivo, the lesion rapidly expanded to 11.9 mm with vitritis, subtotal exudative retinal detachment (ERD), and features of necrosis (heterogenous echogenicity). The lesion regressed over 10 months to 1.29 mm with resolution of ERD and improved visual acuity from counting fingers to 20/50.

Case two: A 63-year-old male with clear cell RCC presented with a right eye 7.2 mm choroidal mass and subretinal haemorrhage. The lesion enlarged to 10.9 mm with ERD and heterogenous echogenicity after starting ipi/nivo, which then regressed to 2.4 mm by 7 months, leaving retinal folds. Vision declined to hand motions and remained stable.

Conclusion: Ipi/nivo can induce rapid and sustained regression of RCC CM but may cause profound intraocular inflammation, collateral damage to surrounding structures, and subsequent vision loss. This response may be enhanced in the presence of pre-existing scleritis.

Introduction: The brachytherapy plaques used for uveal melanoma treatment are reused following sterilization processes. Here, we report patients with adverse reaction following a new cleaning procedure for brachytherapy plaques.

Case presentations: The brachytherapy plaque sewn on the outer eyewall would be removed following the delivery of calculated radiation dose. They are reused following standard calibration testing and sterilization processes. From September 2021 to January 2022, 6 patients treated with ruthenium-106 plaque brachytherapy presented with severe unilateral pain, eyelid swelling, and conjunctival injection with hemorrhage 1-2 days following plaque insertion. The surgeons observed a frosted appearance of the plaques in contrast to the normal shiny look during removal. The unusual severe postoperative reactions and the frosted appearance of plaques led to further investigations. A change in sterilization procedures with the use of LifeClean™ instead of PeraSafe™ was noticed. Ruthenium-106 brachytherapy plaques contain a uniformly distributed radioactive source covered by a thin silver shield. A test was performed by using two plates of pure silver that underwent its respective cleaning procedure using either PeraSafe™ or LifeClean™.

Conclusion: The test results demonstrated formation of silver chloride with LifeClean™, while no such formation was demonstrated with PeraSafe™. We did not observe any new cases of severe postoperative reaction or frosting of plaques after changing back to PeraSafe™. Chlorine salt deposits probably contributed to severe inflammatory reaction of ocular surface.

Background: Early and accurate diagnosis of choroidal tumors including benign nevi, melanomas, and vascular lesions is essential for effective clinical management. Conventional imaging techniques such as fundus photography, optical coherence tomography, fundus autofluorescence, and ultrasonography have greatly advanced the accuracy of choroidal tumor assessment but do have limitations. Hyperspectral imaging (HSI) is a noninvasive modality capturing high-resolution spectral data across multiple wavelengths and has shown promise in various medical fields. Its emerging use in ophthalmology may offer novel insights, although clinical evidence remains preliminary.

Summary: This review explores the principles of HSI and its early investigational role in ocular oncology. Although clinical evidence remains limited, HSI may help improve lesion differentiation, tumor margin delineation and potentially provide indirect biochemical insights. When combined with existing imaging techniques, HSI could support a more comprehensive and individualized diagnostic approach. Key challenges and future directions are discussed.

Key messages: HSI is a promising, noninvasive imaging innovation with potential to enhance choroidal tumor characterization. While still primarily investigational, further clinical validation is essential to determine its role in ophthalmic practice.

Introduction: The aim of this study was to investigate the impact of injecting Topotecan 90 μg/0.18 cc on intraocular pressure (IOP) in children with retinoblastoma.

Methods: This was a retrospective study of 78 eye encounters of 37 patients with retinoblastoma (22 males, 15 females, mean age: 3.5 ± 2.2 years, range 0.50-7.96 years) injected with intravitreal 90 μg topotecan with 0.18 mL volume. IOP was measured with a Schiotz tonometer at baseline prior to injecting, after digital massage, and then at specified time intervals following intravitreal injection of topotecan 90 μg in 0.18 mL volume. Mean arterial pressure (MAP) was either calculated from anesthesia records or recorded during anesthesia.

Results: Mean preinjection IOP was 7.6 ± 2.5 mm Hg (range: 2-20 mm Hg). Mean IOP 60 s after intravitreal topotecan was 37.3 ± 17.4 mm Hg (range: 20-82 mm Hg). The IOP of 93.6% of patients was less than the MAP at all observed time points after injection. In patient eye encounters where IOP exceeded MAP, IOP resolved to below MAP in 4 min in all encounters. Additionally, in 4 min, 91% of patient eye encounters had IOP of below 29 mm Hg.

Conclusion: Topotecan 90 μg/0.18 cc dose is increasingly important for retinoblastoma treatment. Injection of intravitreal topotecan 90 μg/0.18 cc chemotherapy caused a transient rise in IOP with spontaneous resolution below MAP for all patients after 4 min without further intervention. This is the first study of intravitreal topotecan 90 μg/0.18 cc on IOP and provides reassurance for the safe use of higher dose and volume of topotecan 90 μg/0.18 cc.

Background: Retinal capillary haemangioblastoma (RCH) is a benign but potentially vision-threatening vascular tumour of the retina. It may occur sporadically or as multifocal, bilateral lesions in association with von Hippel-Lindau (VHL) disease, often serving as the first manifestation of this multisystem disorder. The pathogenesis involves dysregulated angiogenesis through hypoxia-inducible factor (HIF) pathways, resulting in capillary proliferation and stromal recruitment.

Summary: RCHs may present as asymptomatic peripheral lesions or as juxtapapillary tumours, leading to exudation, retinal detachment, and vision loss. Classification based on anatomical location and growth pattern (endophytic, exophytic, sessile) aids prognostication and treatment planning. Diagnosis relies on multimodal imaging, including fluorescein angiography, optical coherence tomography (OCT), OCT angiography, and occasionally indocyanine green angiography. Treatment is individualized according to tumour size, location, and complications, ranging from laser photocoagulation, cryotherapy, photodynamic therapy, and intravitreal anti-vascular endothelial growth factor (VEGF) injections to vitrectomy for tractional complications. Recently, systemic HIF-2α inhibitors such as belzutifan have shown promise in stabilizing retinal lesions in VHL patients. Accurate differentiation from mimickers such as vasoproliferative tumours, Coats disease, and cavernous haemangiomas remains essential.

Key messages: RCH is often the first sign of VHL disease, highlighting the need for systemic evaluation. Its pathogenesis stems from HIF pathway dysregulation, with tumour size and location - especially juxtapapillary lesions - determining visual risk. Multimodal imaging is vital for diagnosis, differentiation, and monitoring. Management is individualized, using focal therapies, anti-VEGF injections, or surgery for complications, while systemic HIF-2α inhibitors like belzutifan show promise in VHL-associated cases. Early detection and a multidisciplinary approach are essential for preserving vision and managing systemic disease.

Introduction: Conjunctival benign reactive lymphoid hyperplasia (CBRLH) is a rare, polyclonal lymphoproliferative disorder on the benign end of the spectrum of lymphocytic infiltrative disorders. There is currently no established standard of care, and various treatment options have been explored. This 2-patient case series highlights the successful use of subconjunctival triamcinolone acetate as an effective therapeutic approach, contributing to the growing body of literature on CBRLH management.

Case presentations: This case series presents 2 patients presenting with erythematous conjunctival thickening and histological examination suggestive of CBRLH. Treatment involved subconjunctival injection of triamcinolone acetate. Within 3 weeks, the patients demonstrated complete clinical resolution of the CBRLH lesions, with no observed recurrence at 12-month follow-up.

Conclusion: This case series underscores the effectiveness of intralesional subconjunctival triamcinolone acetate as an effective treatment option for CBRLH. The rapid and sustained resolution of lesions highlights the potential role of subconjunctival steroid therapy in the management of this condition. Further research may help establish standardised guidelines for optimal treatment strategies.

Introduction: The study aimed to investigate the effect of treated retinoblastoma on refractive parameters.

Methods: A case-control study was conducted in the ophthalmology department at Ain Shams University from September 2023 to April 2024. Refractive parameters of treated retinoblastoma eyes were compared to those of age matched: (a) fellow eyes of unilateral retinoblastoma children, (b) normal eyes of healthy controls. Cycloplegic refraction, ocular biometry, and anterior segment optical coherence tomography were performed. Image J software was used to calibrate the radii of curvature of lens surfaces.

Results: The study included 35 diseased eyes and 25 fellow eyes of 49 retinoblastoma subjects as well as 26 normal eyes of 26 healthy controls. A statistically significant difference existed between retinoblastoma eyes and eyes of healthy controls regarding refractive astigmatism (p = 0.02), corneal astigmatism (p = 0.047), anterior chamber depth (p < 0.001), and posterior lens curvature (p = 0.041). A statistically significant difference existed between RB eyes and fellow eyes regarding refractive astigmatism (p < 0.001) and corneal astigmatism (p = 0.001). There was no statistically significant difference between fellow eyes and eyes of healthy controls regarding any of refractive parameters.

Conclusion: Treated retinoblastoma eyes had shallower anterior chamber, more rounded lens, more astigmatic corneas, and more refractive astigmatism than healthy eyes.