Guneet S Sodhi, Nakul Singh, Jacquelyn Wrenn, Arun D Singh
{"title":"Peripheral Hemorrhagic Chorioretinopathy: Differentiating Features from Choroidal Melanoma.","authors":"Guneet S Sodhi, Nakul Singh, Jacquelyn Wrenn, Arun D Singh","doi":"10.1159/000528663","DOIUrl":"10.1159/000528663","url":null,"abstract":"<p><strong>Introduction: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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).</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"9 1-2","pages":"1-8"},"PeriodicalIF":0.9,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10433100/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10046621","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Janani Singaravelu, Alexander Melendez-Moreno, Jacquelyn Wrenn, Arun D Singh
{"title":"Angiographic Features of Iris Melanocytic Tumors: Nevus versus Melanoma.","authors":"Janani Singaravelu, Alexander Melendez-Moreno, Jacquelyn Wrenn, Arun D Singh","doi":"10.1159/000529073","DOIUrl":"10.1159/000529073","url":null,"abstract":"<p><strong>Introduction: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>Feeder vessels were present in 17 (43%) melanomas and were absent in the nevus group (<i>p</i> = 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 (<i>p</i> = 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 (<i>p</i> = 0.001). Angiographic silence occurred in 3 nevi (20%) and was not observed in any melanoma (<i>p</i> = 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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"9 1-2","pages":"9-16"},"PeriodicalIF":0.9,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10433095/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10051632","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Reply to Augsburger et al: Selection Bias May Impact Reported Metastasis Risk for 15-Gene Expression Profile Class 1A/B Patients.","authors":"Robert W Cook, Katherina M Alsina","doi":"10.1159/000529561","DOIUrl":"10.1159/000529561","url":null,"abstract":"","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"9 1-2","pages":"66-67"},"PeriodicalIF":0.9,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10433088/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10046619","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Case Series of Aggressive Orbital Plasmacytomas.","authors":"Nizma Permaisuari, Neni Anggraini, Mutmainah Mahyuddin, Evelina Kodrat, Wulyo Rajabto","doi":"10.1159/000527273","DOIUrl":"https://doi.org/10.1159/000527273","url":null,"abstract":"<p><strong>Purpose: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 4-6","pages":"197-202"},"PeriodicalIF":1.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10013509/pdf/oop-0008-0197.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9138120","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Valerie Schmelter, Frederick Schneider, Stefanie R Guenther, Christoph Fuerweger, Alexander Muacevic, Siegfried G Priglinger, Raffael Liegl, Paul Foerster
{"title":"Local Recurrence in Choroidal Melanomas following Robotic-Assisted Radiosurgery (CyberKnife).","authors":"Valerie Schmelter, Frederick Schneider, Stefanie R Guenther, Christoph Fuerweger, Alexander Muacevic, Siegfried G Priglinger, Raffael Liegl, Paul Foerster","doi":"10.1159/000527915","DOIUrl":"https://doi.org/10.1159/000527915","url":null,"abstract":"<p><strong>Introduction: </strong>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).</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Discussion: </strong>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%.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 4-6","pages":"221-229"},"PeriodicalIF":1.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10013483/pdf/oop-0008-0221.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9131765","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Front & Back Matter","authors":"","doi":"10.1159/000529746","DOIUrl":"https://doi.org/10.1159/000529746","url":null,"abstract":"","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"31 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77856537","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Outcomes of Second-Line Intravitreal Anti-VEGF Switch Therapy in Radiation Retinopathy Secondary to Uveal Melanoma: Moving from Bevacizumab to Aflibercept.","authors":"Ojas Srivastava, Ezekiel Weis","doi":"10.1159/000526548","DOIUrl":"https://doi.org/10.1159/000526548","url":null,"abstract":"<p><strong>Introduction: </strong>Radiation retinopathy is a dose-dependent complication of the retina following exposure to ionizing radiation. The objective of this prospective case series is to determine the clinical efficacy of intravitreal aflibercept for radiation retinopathy secondary to radiotherapy for uveal melanoma in those that failed intravitreal bevacizumab treatment.</p><p><strong>Methods: </strong>A case series of 30 patients with a mean age of 57 ± 15 years with radiation retinopathy were enrolled. Visual acuity (VA) and central foveal thickness (CFT) responses to therapy were assessed with regression analyses at 1 month, 3 months, and 6 months following the switch to aflibercept.</p><p><strong>Results: </strong>Regression analyses showed a statistically significant reduction in CFT and improvements in VA following the switch to treatment by aflibercept at 1 month, 3 months, and 6 months. The mean CFT improved from 476 μm ± 170 to 386 μm ± 139 and the mean VA improved minimally from 20/115 ± 20/63 to 20/112 ± 20/54 over 6 months. After 6 months of aflibercept, 46% of patients displayed a CFT improvement of 100 μm or greater and 23% of patients showed improvement in VA of 1 line or better.</p><p><strong>Conclusion: </strong>This pilot study suggests that patients with radiation retinopathy who have failed monthly intravitreal bevacizumab may respond to aflibercept.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 4-6","pages":"230-235"},"PeriodicalIF":1.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10013499/pdf/oop-0008-0230.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9131762","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
James J Augsburger, Cassandra C Skinner, Zelia M Correa
{"title":"Comparative Metastatic Rates in GEP Class 1A versus 1B Posterior Uveal Melanoma: Results Contrary to Expectations.","authors":"James J Augsburger, Cassandra C Skinner, Zelia M Correa","doi":"10.1159/000526770","DOIUrl":"https://doi.org/10.1159/000526770","url":null,"abstract":"<p><strong>Purpose: </strong>The purpose of this study was to determine whether the metastatic rates in patients with gene expression profile (GEP) class 1A versus 1B posterior uveal malignant melanoma supported or contradicted predictions of very low metastatic rate in GEP 1A cases and moderate rate in GEP 1B cases.</p><p><strong>Patients/methods: </strong>164 patients with a cytopathologically confirmed primary posterior uveal malignant melanoma classified by GEP testing as class 1 (100 GEP 1A, 64 GEP 1B) were evaluated. Kaplan-Meier rates of metastasis were computed and plotted for the GEP class 1 subgroups. Median follow-up of patients who were still alive without metastasis on the date of data analysis was 100.5 months for the GEP 1A patients and 97.2 months for the GEP 1B patients.</p><p><strong>Results: </strong>The actuarial 5-year rate of uveal melanoma metastasis was 10.8% (std. error = 3.2%) in the GEP 1A patients versus 0% in the GEP 1B patients, and the actuarial 10-year rate of metastasis was 12.2% (std. error = 3.5%) in the GEP 1A patients versus 2.1% (std. error 2.1%) in the GEP 1B patients.</p><p><strong>Conclusion: </strong>The results of this retrospective single-center study cast doubt on the validity of the prognostic stratification of GEP class 1 posterior uveal malignant melanomas into very low risk (GEP 1A) versus intermediate risk (GEP 1B) of metastasis subgroups provided by the commercially available GEP test.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 4-6","pages":"242-249"},"PeriodicalIF":1.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10013492/pdf/oop-0008-0242.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9131764","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Patrícia M Pereira, Ana Luís, Maria José Passos, Emanuel Gouveia
{"title":"Solitary Metastases Presentation from Uveal Melanoma: Report of 3 Cases and a Comprehensive Review of the Literature.","authors":"Patrícia M Pereira, Ana Luís, Maria José Passos, Emanuel Gouveia","doi":"10.1159/000527445","DOIUrl":"https://doi.org/10.1159/000527445","url":null,"abstract":"<p><strong>Introduction: </strong>Uveal melanoma (UM) is a rare condition accounting for only 5% of all primary melanoma cases. Still, it is the most frequently diagnosed primary intraocular malignant tumor in adults. UM is an aggressive malignancy that originates from melanocytes in the eye. UMs are usually initiated by a mutation in <i>GNAQ</i> or <i>GNA11</i>, and rarely harbor a <i>BRAF</i> or <i>NRAS</i> mutations like cutaneous melanomas. Even if the primary tumor has been successfully treated with radiation or surgery, up to half of all UM patients will eventually develop metastatic disease. The liver is the most frequent metastatic site, and solitary metastases are rare, especially without hepatic or other organs (such as lung or skin/soft tissue) involvement. Most of treatment options to the metastatic UM are still inadequate in preventing a fatal outcome.</p><p><strong>Methods: </strong>A chart review of patients diagnosed with UM between January 1998 and December 2018 at the Instituto Português de Oncologia de Lisboa Francisco Gentil was performed.</p><p><strong>Results: </strong>Three patients with solitary metastases several years after primary UV treatment without any other organ involvement were identified. Patient 1 and 2 showed a very long overall survival and progression-free survival after complete surgical removal of the isolated metastatic lesion from colon and spleen, respectively. The third patient presented with a single brain metastasis from choroidal melanoma harboring the <i>BRAF</i> V600E mutation, a condition rarely reported in UM.</p><p><strong>Discussion: </strong>The cases highlight long relapse-free survival of UM; hence, a regular long-term follow-up should be mandatory. In addition, solitary metastases from UM should be treated, whenever possible, with a surgical approach, with complete removal as a goal.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 4-6","pages":"203-210"},"PeriodicalIF":1.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10013507/pdf/oop-0008-0203.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9131758","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}