Ocular Oncology and Pathology最新文献

Introduction: Peripheral exudative hemorrhagic chorioretinopathy (PEHCR) is one of the leading mimickers of choroidal melanoma because of overlapping features with choroidal melanoma that make the distinction between these two entities difficult.

Methods: To identify nonoverlapping diagnostic features between PEHCR and choroidal melanoma, a retrospective study of 80 patients (80 eyes); 40 patients (40 eyes) with PEHCR; and 40 patients (40 eyes) with choroidal melanoma was conducted. Ophthalmoscopic and imaging features of PEHCR and choroidal melanoma were compared. Sensitivity and specificity for identifying PEHCR and choroidal melanoma were calculated. Youden's J statistic was assessed for each diagnostic feature.

Results: The most frequent clinical features of PEHCR were presence of druse (100%), hemorrhagic PED (93%), dome-shaped mass (B-scan) (90%), and subretinal/intraretinal hemorrhage (78%). Statistical analysis confirmed high sensitivity of hemorrhagic PED (0.93; 95% CI 0.80-0.98) and high specificity of clot retraction cleft, presence of lipid exudation, and bilaterality (1.00; 95% CI 0.91-1.00) as diagnostic features of PEHCR. Statistical analysis revealed presence of subretinal fluid 0.80 (95% CI 0.54-0.91) was most sensitive and presence of orange pigment, mushroom shape on B-scan, ciliary body extension, and choroidal excavation were most specific (1.00; 95% CI 0.91-1.00) for choroidal melanoma. Nonoverlapping diagnostic features of PEHCR were hemorrhagic PED, clot retraction cleft, presence of lipid exudation, and bilaterality. All PEHCR patients (100%) had at least one of these nonoverlapping diagnostic features. Nonoverlapping diagnostic features of choroidal melanoma were the presence of orange pigment, choroidal excavation, mushroom-shaped mass, and ciliary body extension (the latter 3 detected on B-scan). Youden's J statistic was highest for hemorrhagic PED and lowest for dome-shape appearance on B-scan (0.075).

Conclusion: PEHCR and choroidal melanoma can be differentiated by identifying diagnostic features that are exclusive to each entity. The presence of hemorrhagic PED strongly supports a diagnosis of PEHCR. B-scan ultrasonography is required to detect a mushroom-shaped mass, choroidal excavation, or ciliary body extension to exclude underlying choroidal melanoma.

Introduction: Determining the nature of iris melanocytic tumors based on clinical exam alone remains challenging. Tumor-associated vasculature of iris melanocytic lesions may facilitate the ability to discern between iris nevus and melanoma.

Methods: In a single-institution, retrospective, observational study of 45 patients with pathologically confirmed iris melanoma and 15 patients with iris nevi that were either clinically stable or pathologically confirmed were included. Tumor characteristics and associated vasculature were identified on clinical exam and slit-lamp photographs. Fluorescein angiographic parameters including feeder vessels, intrinsic vessels, leakage, masking, and angiographic silence were assessed.

Results: Feeder vessels were present in 17 (43%) melanomas and were absent in the nevus group (p = 0.002). Thirty-three (83%) iris melanomas and 5 (33%) iris nevi were observed to have intrinsic vessels, and a statistically significant association of intrinsic vessels with malignancy (p = 0.001) was noted. Fluorescein leakage was also observed more frequently in iris melanoma 39 (98%) than in nevi 9 (60) with a significant difference (p = 0.001). Angiographic silence occurred in 3 nevi (20%) and was not observed in any melanoma (p = 0.017). Overall, the presence of intrinsic vessels +/- feeder vessels had high sensitivity (0.85) and high positive predictive value (0.87) for diagnosis of iris melanoma.

Conclusions: Anterior segment fluorescein angiography allows for the assessment of tumor-associated vascular patterns and demonstrates utility in differentiating iris nevi from melanoma. Feeder vessels were only observed in iris melanoma and were absent in iris nevi. The intrinsic vessels were present more frequently in melanomas and are thus associated with malignancy. Angiographic silence is indicative of iris nevi.

Purpose: Orbital plasmacytoma is a tumor of plasma cells located in the orbit, which is uncommon and only accounts for less than 1% of total orbital tumors. Sixty-five percent of orbital plasmacytoma are carrying a diagnosis of multiple myeloma. We hereby present two aggressive orbital plasmacytoma cases, a rare orbital malignancy with unsatisfactory outcomes.

Methods: This is a series of two orbital plasmacytoma cases. Both initial complaints were unilateral rapid onset of non-axial proptosis with palpable mass in the superior orbit. The first case was IgA-type multiple myeloma with multiple secondary plasmacytomas diagnosed based on systemic evaluation showing hyperproteinemia, IgA level elevation with free κ-light chains, and multiple destructive osteolytic lesions. The second patient unfortunately died before systemic evaluation was carried out.

Results: Both patients died less than 2 months after diagnosis, underscoring a very poor prognosis. It is important to perform systemic evaluation and appropriate treatment immediately once the diagnosis has been established.

Conclusions: Orbital plasmacytoma is a rare orbital malignancy and is commonly secondary to systemic multiple myeloma. Ophthalmologists should have a high index of suspicion as it has a nonspecific presentation and consider it as one of the differential diagnoses in orbital tumors.

Introduction: Tumor recurrence in choroidal melanoma has been associated with decreased overall survival due to metastatic spreading. To detect risk factors of local recurrence and side effects, we analyzed tumor planning and treatment parameters in patients with recurrence of choroidal melanoma after treatment with robotic-assisted radiosurgery (CyberKnife).

Methods: Six hundred ninety-four patients treated with CyberKnife between 2005 and 2019 were retrospectively reviewed. Age, gender, best-corrected visual acuity, tumor height, and diameter were recorded. Treatment planning and radiation doses were reviewed. Salvage therapy, overall survival, metastasis, and complications were recorded.

Results: Seventy-four patients showed local recurrence. Local recurrence occurred after 42.1 months post CyberKnife treatment (mean; range: 5-100 months). Fourteen out of 74 patients (18.9%) died during follow-up. Recurrence treatment included enucleation in 51 patients (68.9%) and radiosurgery in 19 patients (25.7%). Treatment planning without contrast medium MRI, radiation dose of less than 21 Gy, and insufficient margin delineation were identified as risk factors incrementing local control.

Discussion: Robotic-assisted radiosurgery (CyberKnife) is a suitable treatment option for large choroidal melanoma up to 12 mm. Patients with significantly better visual acuity received repeat CyberKnife treatment as salvage therapy and showed an eye retention rate of 81%.