Alexander Melendez-Moreno, Janani Singaravelu, Jennifer Brainard, Jacquelyn M Davanzo, Arun D Singh
{"title":"Anterior Uveal Melanocytoma: Role of Diagnostic FNAB.","authors":"Alexander Melendez-Moreno, Janani Singaravelu, Jennifer Brainard, Jacquelyn M Davanzo, Arun D Singh","doi":"10.1159/000527596","DOIUrl":"https://doi.org/10.1159/000527596","url":null,"abstract":"<p><strong>Introduction: </strong>Anterior uveal melanocytoma (AUM) pose a diagnostic challenge as they can mimic growing melanomas. Establishing a definitive diagnosis of melanocytoma necessitates cytologic or histopathologic confirmation. We describe the clinical presentation and characteristics of fifteen pathologically proven AUM cases and assess the role of fine needle aspiration biopsy (FNAB) as a safe and effective tool for diagnosis.</p><p><strong>Methods: </strong>Retrospective review of pathologically confirmed AUM cases was performed. Demographic data, presenting symptoms, clinical features, diagnostic approach, cytological and histological features, and clinical outcomes were collected.</p><p><strong>Results: </strong>Fifteen patients with pathologically confirmed AUM were identified. The mean and median age of diagnosis were 50 and 53 years, respectively (range 3-77 years). The melanocytoma was localized to the iris (5, 33%) or ciliary body (7, 47%), and 3 patients had iridociliary involvement (20%). Presentation was due to concern for growth in 4 (29%), visual symptoms in 1 (7%), and was an incidental finding in 10 (64%) patients. Pigmentation of the tumor varied with 9 (60%) appearing brown and 3 (20%) black in color. The color of 3 (20%) ciliary body tumors could not be assessed. The diagnosis was confirmed with FNAB in 6 (40%), excisional biopsy in 7 (47%), and incisional biopsy in 2 (13%). Cytologic and histologic preparations demonstrated predominance of round to polygonal cells with heavily pigmented cytoplasm and small round nuclei. One patient who underwent excisional biopsy had prior FNAB that was interpreted as suspicious for melanoma (false-positive). Instances of false-negative cytology were not observed as demonstrated by the subsequent stable clinical course during the mean follow-up of 21.2 months (range = 1.0-63.0 months). FNAB-related complications were not observed in any case.</p><p><strong>Conclusion: </strong>FNAB offers a minimally invasive and safe diagnostic approach for pathologic confirmation of AUM. However, limitations of FNAB including false-negative and false-positive biopsies must be considered when excluding underlying malignancy. Continued observation to document tumor stability should be considered.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 4-6","pages":"211-220"},"PeriodicalIF":1.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10013327/pdf/oop-0008-0211.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9187613","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":"Recurrence of Primary Acquired Melanosis and Conjunctival Intraepithelial Neoplasia.","authors":"Karin Svedberg","doi":"10.1159/000526985","DOIUrl":"https://doi.org/10.1159/000526985","url":null,"abstract":"<p><strong>Introduction: </strong>The aim of this study was to investigate the frequency of recurrences, time to recurrence, and which patients had a recurrence after treatment of conjunctival intraepithelial neoplasia (CIN), carcinoma in situ, and primary acquired melanosis (PAM) with atypia.</p><p><strong>Methods: </strong>A retrospective chart review of all patients included in the follow-up program after completion of treatment for CIN or PAM with atypia on October 18, 2021, at the Department of Ophthalmology, Sahlgrenska University Hospital, was conducted.</p><p><strong>Results: </strong>There were five recurrences (5/31, 17%) in the group with CIN or carcinoma in situ: two for patients with CIN grade II and three for individuals with carcinoma in situ. Time to diagnosis of recurrence ranged from 6 to 288 months. No recurrence was diagnosed for the 26 patients followed after treatment for PAM with atypia.</p><p><strong>Conclusion: </strong>With the strategy of radical treatment for CIN and PAM with atypia, whenever possible, subsequent follow-up can probably be ceased after 10 years if the patient is not immunocompromised. For completely excised PAM with atypia grade I, there is most likely no need for further clinical controls.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 4-6","pages":"236-241"},"PeriodicalIF":1.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/27/fb/oop-0008-0236.PMC10013497.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9131759","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}
Jared J Ebert, Maura Di Nicola, Eric D Hansen, Sean M Collon, Matthew C Hagen, Basil K Williams
{"title":"Serous Choroidal Detachment and Scleritis Associated with Necrotic Choroidal Tumors.","authors":"Jared J Ebert, Maura Di Nicola, Eric D Hansen, Sean M Collon, Matthew C Hagen, Basil K Williams","doi":"10.1159/000527880","DOIUrl":"https://doi.org/10.1159/000527880","url":null,"abstract":"<p><strong>Purpose: </strong>The aim of the study was to describe choroidal detachments and concurrent scleritis associated with necrotic choroidal metastasis or melanoma.</p><p><strong>Methods: </strong>We conducted a retrospective case series.</p><p><strong>Results: </strong>We report 4 patients with scleritis and choroidal detachment with an underlying malignant choroidal tumor. All patients underwent fine-needle aspiration biopsy for cytopathologic characterization of their choroidal tumor, and they all demonstrated evidence of tumor necrosis. Two patients were diagnosed with choroidal metastasis from lung and esophageal adenocarcinoma. Both patients ultimately expired from systemic metastasis. The remaining 2 patients were diagnosed with choroidal melanoma and were successfully treated with plaque radiotherapy.</p><p><strong>Conclusion: </strong>Choroidal detachment with concurrent scleritis can occur as a rare sequelae of tumor necrosis of an underlying choroidal malignancy.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 4-6","pages":"191-196"},"PeriodicalIF":1.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10013487/pdf/oop-0008-0191.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9138117","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":"Uveitic Granulomas Masquerading as Ocular Tumors.","authors":"Abdulrahman AlZaid, Moustafa S Magliyah, Yahya AlZahrani, Hani Basher ALBalawi, Hassan AlDhibi","doi":"10.1159/000526817","DOIUrl":"10.1159/000526817","url":null,"abstract":"<p><strong>Introduction: </strong>Solitary uveal lesions confer a diagnostic challenge to ophthalmologists. Uveitic lesions most abundantly appear amelanotic and commonly involve the choroid. Most amelanotic choroidal lesions are either neoplastic or inflammatory in origin. In our study, we aimed to describe six uveitic granuloma cases, which were referred to a tertiary ophthalmology center as intraocular tumors.</p><p><strong>Methods: </strong>Retrospective chart review of 6 patients (7 eyes) who had uveitic granulomas and were referred to a tertiary ophthalmology center as having intraocular tumors.</p><p><strong>Results: </strong>Mean age on presentation was 47 ± 12.5 years. One lesion was involving the ciliary body only, five lesions had pure choroidal involvement, and one had ciliochoroidal involvement. Mean visual acuity on presentation was 1.7 ± 0.75 (Snellen = 20/1,000) and ranged from 20/80 to light perception. Mean basal diameter of all lesions was 7.7 ± 1.8 mm. Three lesions had moderate echogenicity, two lesions were low to moderate echoic, and one lesion had moderate to high echogenicity on ultrasonography. Three lesions were associated with retinal detachments. Five eyes showed an early hypofluorescence with late hyperfluorescence. Leakage of fluorescein at borders was noticed in 3 lesions. Final diagnosis was presumed intraocular tuberculosis in 4 patients, probable ocular sarcoidosis in 1 patient, and idiopathic solitary uveitic granulomas in 1 patient. Upon treatment, the vision improved to 0.3 ± 0.27 (Snellen = 20/40) and ranged from 20/20 to 20/100 after 4.7 ± 2.9 years of follow-up.</p><p><strong>Conclusions: </strong>Uveitic granulomas can demonstrate features of ocular tumors. Proper uveitis management leads to a favorable visual outcome and ocular preservation.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 3","pages":"181-186"},"PeriodicalIF":1.0,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10015586/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9515581","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}
Naomi Hasegawa, Alexander Rusakevich, Eric Bernicker, Bin S Teh, Amy Schefler
{"title":"Comparison of Tumor Size and Gene Expression at Presentation in Uveal Melanoma Patients before and during the COVID-19 Pandemic.","authors":"Naomi Hasegawa, Alexander Rusakevich, Eric Bernicker, Bin S Teh, Amy Schefler","doi":"10.1159/000524918","DOIUrl":"https://doi.org/10.1159/000524918","url":null,"abstract":"<p><p><b><i>Introduction:</i></b> The aim of this study was to compare the clinical and gene expression variables of uveal melanoma patients presenting before and after the start of the COVID-19 pandemic as surrogate markers in order to assess the pandemic's potential impact on care. <b><i>Methods:</i></b> We conducted a retrospective chart review of uveal melanoma patients at Retina Consultants of Texas and assessed tumor size, staging, and gene expression data during two time periods: May 2019 to February 2020 (Group 1: Before the COVID-19 pandemic declaration by the WHO in March 2020) and May 2020 to March 2021 (Group 2: After the start of the COVID-19 pandemic). <b><i>Results:</i></b> A total of 80 patients with uveal melanoma were studied (Group 1: 40 [50%] and Group 2: 40 [50%]). There was no statistically significant difference in the tumor thickness (<i>p</i> = 0.768), largest base dimension (<i>p</i> = 0.758), Collaborative Ocular Melanoma Study size class (<i>p</i> = 0.762), and American Joint Committee on Cancer stages (<i>p</i> = 0.872) between the two groups. Additionally, there was no difference in the tumors' gene expression data including gene expression profile class (<i>p</i> = 0.587) and PRAME expressivity (<i>p</i> = 0.861) between the two groups. <b><i>Discussion/Conclusion:</i></b> The COVID-19 pandemic had no effect on the presentation of uveal melanoma patients across all tumor characteristics including size, staging, and gene expression data, suggesting there was not a significant diagnostic delay in care for uveal melanoma patients at our center due to the pandemic.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 3","pages":"156-160"},"PeriodicalIF":1.0,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9372456/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9475729","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":"10.1159/000528104","DOIUrl":"https://doi.org/10.1159/000528104","url":null,"abstract":"","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"1955 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91227397","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":"Emerging New Therapeutics for Retinoblastoma.","authors":"Vishal Raval, Manoj Parulekar, Arun D Singh","doi":"10.1159/000524919","DOIUrl":"https://doi.org/10.1159/000524919","url":null,"abstract":"<p><p><b><i>Background:</i></b> Over the last few decades, chemotherapy has become the main treatment of retinoblastoma, delivered through various routes: intravenous, intra-arterial, and intravitreal. Despite its efficacy, chemotherapy-related toxicity (ocular and systemic) and recurrences due to resistant tumor clones are common, highlighting the need for novel therapeutic agents. <b><i>Summary:</i></b> Recent advances in our understanding of the molecular drivers of Rb1 tumorigenesis and mechanisms of tumor resistance have afforded opportunities to explore novel targets such as the MDMX-p53 pathway (nutlin-3), histone deacetylase inhibitors, spleen tyrosine kinase inhibitors, and genetic and immune modulatory drugs. In this review, we discuss the limitations of current therapeutic strategies, candidate cellular pathways, current evidence for newer targeted drugs, and offer a look toward the future. <b><i>Key Messages:</i></b> Advances in the understanding of the molecular drivers of the RB pathway have provided opportunities to explore novel drugs with targeted effects, improved bioavailability, and reduced chemotoxicity.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 3","pages":"149-155"},"PeriodicalIF":1.0,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10015589/pdf/oop-0008-0149.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9499974","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}
Aaron A Gurayah, Vanessa A Peters, William Jin, Karthik Kalahasty, Deukwoo Kwon, Wei Zhao, Nirav V Patel, Arnold M Markoe, Zelia M Correa, Matthew T Studenski, J William Harbour, Stuart E Samuels
{"title":"Predictors of Local Recurrence and Progression-Free Survival in Iodine-125 Brachytherapy-Treated Uveal Melanomas: A Modern Institutional Study.","authors":"Aaron A Gurayah, Vanessa A Peters, William Jin, Karthik Kalahasty, Deukwoo Kwon, Wei Zhao, Nirav V Patel, Arnold M Markoe, Zelia M Correa, Matthew T Studenski, J William Harbour, Stuart E Samuels","doi":"10.1159/000526771","DOIUrl":"10.1159/000526771","url":null,"abstract":"<p><strong>Introduction: </strong>Iodine-125 brachytherapy is an effective eye-sparing treatment for uveal melanoma. Previous work has shown that uveal melanomas cluster into distinct molecular classes based on gene expression profiles - discriminating low-grade from high-grade tumors. Our objective was to identify clinical and molecular predictors of local recurrence (LR) and progression-free survival (PFS).</p><p><strong>Methods: </strong>We constructed a retrospective database of uveal melanoma patients from the University of Miami's electronic medical records that were treated between January 8, 2012, and January 5, 2019, with either COMS-style or Eye Physics plaque. Data on tumor characteristics, pretreatment retinal complications, post-plaque treatments, LR, and PFS were collected. Univariate and multivariate Cox models for cumulative incidence of LR and PFS were conducted using SAS version 9.4.</p><p><strong>Results: </strong>We identified 262 patients, with a median follow-up time of 33.5 months. Nineteen patients (7.3%) had LR, and 56 patients (21.4%) were classified as PFS. We found that ocular melanocytosis (hazard ratio = 5.55, <i>p</i> < 0.001) had the greatest impact on PFS. Genetic expression profile did not predict LR outcomes (hazard ratio = 0.51, <i>p</i> = 0.297).</p><p><strong>Conclusion: </strong>These findings help physicians identify predictors for short-term brachytherapy outcomes, allowing better shared decision making with patients preoperatively when deciding between brachytherapy versus enucleation. Patients stratified to higher risk groups based on preoperative characteristics such as ocular melanocytosis should be monitored more closely. Future studies must validate these findings using a prospective cohort study.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 3","pages":"175-180"},"PeriodicalIF":1.0,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10329746/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9868838","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}
Philipp Rating, Norbert Bornfeld, Sabrina Schlüter, Henrike Westekemper, Tobias Kiefer, Martin Stuschke, Sophia Göricke, Petra Ketteler, Saskia Ting, Klaus A Metz, Nikolaos E Bechrakis, Eva Biewald
{"title":"Long-Term Results after Intraocular Surgery in Treated Retinoblastoma Eyes.","authors":"Philipp Rating, Norbert Bornfeld, Sabrina Schlüter, Henrike Westekemper, Tobias Kiefer, Martin Stuschke, Sophia Göricke, Petra Ketteler, Saskia Ting, Klaus A Metz, Nikolaos E Bechrakis, Eva Biewald","doi":"10.1159/000524610","DOIUrl":"https://doi.org/10.1159/000524610","url":null,"abstract":"<p><p><b><i>Introduction:</i></b> The aim of the study was to analyze the results of intraocular surgery in treated retinoblastoma eyes and to assess the long-term results with a priority on local recurrences, secondary enucleation, and metastases. <b><i>Methods:</i></b> Retrospective noncomparative case series. <b><i>Results:</i></b> From March 1964 to January 2020, 42 eyes of 40 retinoblastoma patients underwent intraocular surgery. Time interval between the last therapy and surgery was 9.5 years (mean: 114 months; median: 54.5 months). 31 eyes were treated for radiogenic cataract formation with a gain in visual acuity of 61.3%. One child developed an upper eyelid metastasis, 3 showed second primary malignancies (SPM), one a late recurrence, and 2 eyes were enucleated. Retinal surgery was performed in 17 eyes; 6 eyes were done as a combined procedure. Indications were radiogenic complications in the sense of a vitreous hemorrhage in 11 eyes and a rhegmatogenous retinal detachment in 6 eyes. 41.2% of the treated eyes had a postoperative gain in visual acuity, whereas 9.5% of the eyes could not be preserved in the long term. Regarding systemic involvement 2 patients developed late recurrences and one a SPM. <b><i>Conclusion:</i></b> Surgical therapy in treated retinoblastoma is necessary in isolated cases. In our series, cataract surgery was a safe procedure with a good option of a significant increase in visual acuity. As expected, vitreoretinal treated eyes showed a limited gain in visual acuity, a higher risk of late recurrences, and a lower globe retention rate. Therefore, a careful indication and individual risk-benefit analysis are mandatory.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 3","pages":"161-167"},"PeriodicalIF":1.0,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10015588/pdf/oop-0008-0161.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9499973","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}