Ocular Oncology and Pathology最新文献

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.

Introduction: We aimed to report the demographic data, clinical features, and management outcomes of patients with uveal melanoma (UM) in a single tertiary center in Egypt.

Methods: This is a single-center retrospective case series. Patients with UM who were managed at Cairo University Ocular Oncology Service between January 2019 and December 2023 were included. Records were analyzed for patients' demographics, clinical features and different management options. Outcomes included changes in best corrected visual acuity and tumor dimensions (thickness and largest base diameter), perioperative complications, globe salvage, and recurrence rates.

Results: A total of 93 eyes of 93 patients with UM were treated in our service over a period of 5 years. The mean age of patients was 52.18 ± 13.30 years, and 91.4% of tumors were choroidal or ciliochoroidal. 52 eyes (55.91%) were managed with ruthenium-106 episcleral brachytherapy, of which two eyes required additional brachytherapy, 8 eyes ended with secondary enucleation, and the globe was salvaged in 84.62% of eyes. 8 eyes (8.60%) were managed by gamma knife radiosurgery with or without endoresection. One eye with an iridociliary tumor was managed with partial lamellar sclerouvectomy, and the remaining 32 eyes (34.41%) were primarily enucleated.

Conclusion: Timely referral channels and accurate assessment of cases with UM are invaluable for achievement of higher success rates in tumor regression, as well as globe and/or vision salvage in eyes managed with brachytherapy. Patients with more advanced tumors should be counseled on the importance of more invasive options such as enucleation in achieving lower metastasis and mortality rates.

Introduction: Undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma, is a high-grade soft tissue sarcoma arising from mesenchymal stem cells. UPSs are rare and account for about 5% of all soft tissue sarcoma. UPSs arising in the head and neck are especially rare, comprising 1-3% of all UPSs.

Case presentation: In this report, we describe 2 cases of intraocular UPS. Both cases concern 68-year-old males: one developing a UPS in an eviscerated socket after a chronic fibrinous inflammation and the other years after ocular trauma.

Conclusion: Our cases may support the hypothesis of chronic inflammation playing a role in sarcoma formation as they are characterized by a longstanding history of (surgical) trauma with signs of chronic inflammation and phthisis bulbi.

Introduction: Clear evidence for the best clinical management of uveal melanoma is lacking in some areas. Therefore, reports on expert opinions in the field can be valuable.

Methods: A questionnaire comprising 10 questions was distributed to potential participants of the 58th Ophthalmic Oncology Group Meeting in Stockholm, Sweden, in June 2024.

Results: Among 34 respondents, 13 (38%) had >20 years of postresidency experience in ophthalmic oncology. The maximum recommended tumor thickness for ruthenium-106 plaque brachytherapy was 5.7 mm (SD 1.1). Twenty-three respondents (68%) indicated that radiological surveillance for metastatic disease should be conducted irrespective of primary tumor characteristics. A majority (74%) would treat a lesion with a 6 mm diameter and 1.5 mm thickness without waiting for evidence of growth if sufficient risk factors were present. Most experts did not currently recommend sampling of circulating tumor DNA or circulating tumor cells. There were no significant differences in responses based on the experience of respondents (≤20 vs. >20 years) or their annual volume of new cases (≤50 vs. >50).

Conclusion: This article reports the opinions of 34 experts in ophthalmic oncology on various contemporary topics in uveal melanoma. The responses illustrate both agreements and differences in opinions among experts.

Introduction: While pediatric intraocular malignancies are rare, management requires frequent examinations under anesthesia (EUAs) which are financial costly. The purpose of the study was to measure operating room (OR) turnover costs and to estimate potential cost savings of reducing turnover time.

Methods: Turnover time data for EUAs with a single ocular oncology provider at the University of Michigan were analyzed from 2021 to 2023. Direct cost of OR turnover was set at USD 25.73 per minute, and turnover time after business hours utilized a cost multiplier for overtime costs. A sensitivity analysis for turnover reduction savings was based on reducing turnover times to a goal turnover time. Primary outcomes were total and average turnover time and costs for pediatric ocular oncology EUAs. Secondary outcomes included the potential cost savings associated with reduced turnover time goals.

Results: 158 EUAs with valid turnover times were included. Total turnover time and costs were 5,034 min and USD 134,104. Average (standard deviation) turnover time and costs were 31.9 min (19.0) and USD 848.76 (USD 487.48). A majority of cases (65%) had turnover time less than 30 min, though a minority (23%) had a turnover time less than 20 min. By reducing maximum turnover time to 20 min, a total of USD 52,057 could be saved across these cases or USD 329 per case.

Conclusions: Turnover time accounts for a notable proportion of OR costs, particularly for shorter cases. The average pediatric ocular oncology case had nearly USD 850 of turnover costs, much of which may be avoidable. Actions aimed at reducing turnover time are likely to have high return on investments if they are successful.

Introduction: The systemic HIF-2 alpha inhibitor, belzutifan, has been approved for use in patients with von Hippel-Lindau disease (VHL)-associated renal cell carcinoma, central nervous system (CNS) hemangioblastomas, and pancreatic neuroendocrine tumors. This drug has also shown promise in controlling VHL retinal hemangioblastomas (RHs), but little work has been published on the use of the drug in this setting.

Methods: We conducted a retrospective review of patients with VHL-associated RHs followed by the retina service at our institution who were treated with systemic belzutifan. Patient age, gender, genotype, presence of systemic tumors, indication for the drug, initial dose, adjusted dose, side effects, and tumor response were recorded. We also conducted a literature search for all manuscripts describing the effect of belzutifan on VHL-associated ocular tumors.

Results: We identified 12 eyes of 7 patients with VHL-associated ocular tumors who were treated with belzutifan at our institution. Of these, 5 eyes of 3 patients had progressing ocular tumors when belzutifan was started. Of the 7 total patients, 2 were treated for renal cell carcinoma, 2 for CNS hemangioblastomas, 2 for RHs, and one for pancreatic neuroendocrine tumors. Initial dose was 120 mg PO daily in 6 patients and 80 mg PO daily in 1 patient. The dose was reduced in all but 1 patient due to side effects. The ocular tumors were controlled in all patients with an average follow-up of 13 months (range 4-24 months). Literature review identified 7 manuscripts that described belzutifan-mediated control of ocular tumors in patients with VHL-associated RHs in 21 patients.

Conclusion: The drug belzutifan shows great promise for controlling RHs and preventing vision loss in patients with VHL. Further work needs to address the optimal dose, role of the drug as a neoadjuvant therapy, and long-term efficacy and tolerability of the drug in a larger cohort of patients with ocular tumors.

Introduction: The aim of the study was to describe and evaluate characteristics of ocular tumor lysis syndrome (OTLS) in eyes with uveal melanoma.

Methods: Retrospective chart review of all patients with OTLS at the University of Colorado from 2009 to 2021. Data collected included patient demographics, tumor characteristics, radiation dosimetry, gene expression profiling (GEP), OTLS characteristics, management, and outcomes.

Results: Seven eyes of seven patients with uveal melanoma treated with I-125 brachytherapy developed OTLS. Average age was 59 years (range 32-83). Mean apical height was 8.6 mm (range 6-11); mean diameter was 12.7 mm (range 8.5-15.3). All tumors were treated with plaques ≥16 mm in diameter. On presentation, 5/7 tumors had subretinal fluid, and 6/7 had collar-button configuration. OTLS presented as extensive pigment dispersion in the vitreous in all eyes, subretinal pigment and/or retinal detachment in 4/7 eyes, vitreous hemorrhage in 2/7 eyes, and anterior chamber pigment in 3/7 eyes. Four tumors were GEP class 1, two were class 2, and one was unclassified. Biopsy route was trans-scleral in 4/7 eyes and trans-vitreal in 3/7 eyes. OTLS occurred 2-4 weeks after an intraocular procedure in 5/7 eyes. All underwent pars plana vitrectomy. Cytology of the vitreous, obtained in five cases, showed pigment laden macrophages and hemorrhage, but only 1/5 eyes had viable malignant cells. Four eyes were stable at the last follow-up, two were enucleated, and one had no light perception from pigmentary glaucoma. Poor vision (<20/200) occurred in 6/7 cases. Three patients died from metastasis (tumors were GEP class 2, GEP class without subclassification, and no GEP classification performed).

Conclusions: OTLS is a rare but devastating complication of uveal melanoma. Common characteristics included large plaque diameter, presence of subretinal fluid, and collar-button shape. The extensively dispersed pigment is typically not malignant. Though poor vision is common, enucleation may be avoided in most eyes through vitreoretinal surgical repair.

Introduction: We aim to report the anatomical and functional outcomes of ruthenium-106 brachytherapy in the management of circumscribed choroidal hemangiomas (CCH).

Methods: This is a single-center, retrospective case series including patients with unilateral symptomatic CCH treated with ruthenium-106 brachytherapy at the Cairo University Ocular Oncology Service. Patient records were analyzed for patients' demographics, best corrected visual acuity (BCVA), tumor dimensions (thickness and largest base diameter), foveal subretinal fluid, radiation-related complications, and recurrence.

Results: Seven patients were included in the study (including 6 males) with a mean age of 39.3 ± 15.4 years; ruthenium-106 plaque was used to deliver 50 Gray to the tumor apex. After a mean follow-up duration of 12.5 months, all patients had significant improvement in BCVA after treatment, mean tumor height decreased significantly from 4.76 ± 1.76 mm to 1.70 ± 1.2 mm (p value 0.01). The largest tumor base diameter also decreased significantly from 9.13 ± 2.68 mm to 4.65 ± 3.75 mm (p value 0.05). Subretinal fluid and exudative retinal detachment resolved in all patients, and no significant radiation-related complications were observed in any patient. None of the patients needed any further treatment or experienced recurrence within the follow-up period.

Conclusion: Ruthenium-106 brachytherapy is an effective tool in the management of symptomatic CCH with a good visual prognosis and safety profile.