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Background: The purpose of this study is to analyze the long-term evolution of patients with small choroidal melanocytic tumors (SCMTs) undergoing observation, and to assess their rate of transformation into melanomas and survival., Methods: A retrospective single-cohort study of patients with SCMTs (1-3 mm in height and 5-10 mm in base) diagnosed from January 1992 to February 2023 was carried out, with observation as the initial treatment. The main criterion for a transformation into melanoma is considered to be an increase in size of more than 1 mm in height and/or more than 1 mm in base measured on an ultrasound/retinography, recorded in two consecutive visits separated by one to three months., Results: 243 patients were included with a mean age of 65.3 years and a mean follow-up of 7.9 years (6 months-27.9 years); 27 patients showed tumor growth. The probabilities of growth at 5, 10, and 15 years are 10%, 14%, and 17%, respectively. Regarding survival, 22 patients died and only 3 deaths were due to melanoma metastasis. Survival rates at 5 and 10 years are 99% and 97%., Conclusions: Observation is a viable therapeutic option for SCMTs, avoiding the side effects of treatment, considering the majority of these tumors do not progress to melanoma. With close monitoring, patients can be treated promptly upon detecting a transformation. Additionally, the findings confirm that small melanocytic tumors can lead to metastatic disease, albeit at a low rate.

Purpose: To explore the genetic background of choroidal and ciliary body melanoma among children and young adults, with special focus on BAP1 germline variants in this age group., Methods: Patients under the age of 25 and with confirmed choroidal or ciliary body melanoma were included in this retrospective, multicenter observational study. Nuclear BAP1 immunopositivity was used to evaluate the presence of functional BAP1 in the tumor. Next-generation sequencing using Ion Torrent platform was used to determine pathogenic variants of BAP1, EIF1AX, SF3B1, GNAQ and GNA11 and chromosome 3 status in the tumor or in DNA extracted from blood or saliva. Survival was analyzed using Kaplan-Meier estimates., Results: The mean age at diagnosis was 17 years (range 5.0-24.8). A germline BAP1 pathogenic variant was identified in an 18-year-old patient, and a somatic variant, based mainly on immunohistochemistry, in 13 (42%) of 31 available specimens. One tumor had a somatic SF3B1 pathogenic variant. Disomy 3 and the absence of a BAP1 pathogenic variant in the tumor predicted the longest metastasis-free survival. Males showed longer metastasis-free survival than females (P = 0.018)., Conclusions: We did not find a stronger-than-average BAP1 germline predisposition for choroidal and ciliary body melanoma among children and young adults compared to adults. Males had a more favorable survival and disomy 3, and the absence of a BAP1 mutation in the tumor tissue predicted the most favorable metastasis-free survival. A BAP1 germline pathogenic variant was identified in one patient (1%), and a somatic variant based mainly on immunohistochemistry in 13 (42%).

Purpose: The aim of this study was to analyze the course of visual acuity (VA) in visual outcomes of patients treated with iodine-125 ( 125 I) brachytherapy in our center, based on original VA before treatment., Material and Methods: Visual acuity was prospectively assessed using a case series of 305 patients treated with 125 I between 1996 and 2022. To examine how VA behaves over time, we divided patient sample into 4 groups: (1) Patients with visual acuity of less than V ≤ 0.1 at baseline; (2) Patients with low to moderate VA, ranging 0.1 < V < 0.4; (3) Patients with moderate-high VA, ranging 0.4 < V < 0.8; (4) Patients with very high VA of V > 0.8. Each of the four groups was studied separately over a 60-month period to determine the percentage of patients with VA improvement, worsening, or with the same VA status. Finally, visual outcomes over time were estimated with 95% confidence interval (CI) using Kaplan-Meier analysis, and VA maintenance rates were reported at 1, 3, 5, 10, 15, and 20 years of follow-up., Results: The median follow-up time was 78.2 months (range, 6-254 months). The cumulative probabilities of survival analysis at 1, 3, 5, and 10 years were 16%, 3%, 2%, and none for the first sub-group; 46%, 20%, 17%, and 14% for the second; 65%, 53%, 29%, and 15% for the third; and 86%, 56%, 48%, and 41% for the fourth sub-group. The median survival in years was 0.30, 0.80, 3.10, and 4.40 for each sub-cohort, respectively., Conclusions: The decrease and maintenance of VA depends on the initial VA of patients. Most patients experience a marked worsening of their VA, regardless of their VA status before treatment with episcleral brachytherapy. Patients with a higher baseline VA retain VA best over time., Competing Interests: The authors report no conflict of interest., (Copyright © 2023 Termedia.)

Purposes: We analyzed patient, tumor and dosimetric characteristics of subjects in a Spanish population diagnosed with uveal melanoma treated with iodine 125 (I125) episcleral brachytherapy, who presented with post-treatment loss of useful visual acuity and global evolution of visual acuity., Methods: A single historic observational cohort study was undertaken. Patients with uveal melanoma were recruited between September 1995 and June 2015. Clinical, tumor and dosimetric data collection and visual acuity evaluations were performed under everyday practice conditions based on a useful visual acuity >0.1 on the decimal scale. The baseline analysis was performed using descriptive and survival analyses according to Kaplan-Meier curves., Results: A total of 286 of the 665 patients diagnosed with uveal melanoma received episcleral brachytherapy, and 198 were included in the study. The mean follow-up time was 75.3 months (95% CI = 68.0-82.6). Patients with post-treatment useful visual acuity loss (n=94, 47%) presented the following characteristics: visual symptoms (n=80, p-value = 0.001); iris color (brown n=33, hazel green n=49, p-value = 0.047); Collaborative Ocular Melanoma Study size (medium n=80, p-value = 0.159); tumor, node, metastasis stage (T2: n=38, T3: n=38, p=0.012); shape (nodular n=67, mushroom-shaped n=26, p=0.001); posterior pole involvement (n=47, p=0.04); recurrence (n=10, p=0.001); and dose administered in the fovea, optic nerve and center of the eye (p<0.002). Using Kaplan-Meier analysis, the mean overall survival of useful visual acuity was 90.19 months, and the probability of preserving useful visual acuity was 66% for one year, 45% for five years and 33% for ten years., Conclusion: Patients most likely to present with visual acuity loss were those with the following profile: elderly males with dark irises who were diagnosed with visual symptoms and exhibited a medium/large melanoma with a mushroom shape in the posterior pole (near the fovea and/or optic nerve). All patients treated with episcleral brachytherapy are likely to present with visual acuity loss, which is more pronounced in the first few years following treatment.

Competing Interests: The authors declare no interests.

Purpose: The aim of this study was to analyze regression rates and local control of uveal melanoma patients treated with iodine-125 ( 125 I) brachytherapy based on initial tumor apical height., Material and Methods: Patients treated in a single institution from January 1 st , 1996 to 2019 with 125 I plaques (ROPES and COMS) for uveal melanoma were included in this study. Patients treated with brachytherapy for iris and those treated with transpupillary thermotherapy prior to brachytherapy were excluded. The sample was classified into 4 categories depending on initial apical tumor height (h 0 ), i.e., h 0 ≤ 2.5 (small), 2.5 < h 0 ≤ 6.25 (small-medium), 6.25 < h 0 ≤ 10 (medium-large), and h 0 > 10 mm (large). Percentage of original tumor apical height (Δh) was collected during follow-ups. Patterns of regression were evaluated using linear least squares adjustments. Multivariable Cox regression were performed., Results: In total, 305 patients met the inclusion criteria, and 27, 166, 100, and 13 were considered for small, small-medium, medium-large, and large categories, respectively. Median follow-up was 82.4, 56.8, 76.1, 89.1, and 100.1 months for the entire cohort and each sub-group, respectively. Pattern of decrease when h 0 ≤ 2.5 mm was not detectable. For the rest sub-groups, changes in height could be fitted using functional form: Δh (T) = ae -bT + c, R 2 ≥ 0.97. Multivariate Cox analysis factors predictive of local control failure revealed a hazard ratio (HR) of 6.1 (95% CI: 0.7-58.2%, p = 0.05) for patients who remained similar sized after treatment for small-medium tumors. For the rest sub-groups, Cox analysis did not indicate statistical significance in any single variable., Conclusions: Height changes can be modeled by a negative exponential function for the first 7 years after treatment depending on the initial height, except for those less than 2.5 mm. Non-responding small-medium tumors multiply by 6 the probability of failure in local control., Competing Interests: The authors report no conflict of interest., (Copyright © 2021 Termedia.)

Effective cancer brachytherapy requires a treatment plan that delivers high-dose to tumor, while minimizing the dose to critical normal tissues. Therefore, an accurate knowledge of the sources and magnitude of the techniques is essential for producing robust and well optimized-plans. The purpose of this technical note is to establish general procedures and strategies for optimization and customization of the plaques loaded with radioactive seeds, particularly focusing on the definition of useful tactics to limit high doses to organs at risk and adapt the treatment time to the necessity of institution., Competing Interests: Authors report no conflict of interest.

Macroscopic study of surgical samples sent to the histopathology lab provides the first diagnostic approach. Obtaining quality photographs of these pieces facilitates proper case documentation for publication, sharing, teaching and research.This device has been originally designed for enucleated eyes but it could be used for a wide diversity of human or animal samples including thyroids, pituitary glands, prostates… It can be coupled to any smartphone camera.The Black and White Box is an affordable and easy option for taking gross pathology photographs of high quality. In this work we provide full instructions on how to make it., (© 2019 Wiley Periodicals, Inc.)

Objective: The purpose of the present study is to describe the clinical characteristics and outcomes of patients diagnosed and treated for conjunctival melanoma in the Ocular Oncology Unit of the University Hospital Clinic of Valladolid., Methods: A retrospective observational case series study was conducted on patients diagnosed with conjunctival melanoma in the Ocular Oncology Unit of University Hospital Clinic of Valladolid, from January 1992 to December 2017. Demographic information and tumour features were recorded in a Microsoft Access database., Results: Among a total of 462 consecutive patients, the tumour was classified as melanocytic in 252 cases (54.5%), with 27 patients having the pathological diagnosis of conjunctival melanoma. The mean age at diagnosis was 59.2years (16-88), and there were 41% males and 59% females, with a mean follow-up of 6.1±6.8years. As regards the origin of conjunctival melanoma, 16 cases (59%) arose from primary acquired melanosis, 26% from nevus, and 15% developed de novo. The treatment performed was incisional or excisional biopsy in all patients, local adjuvant chemotherapy in 15 cases (56%) and brachytherapy in 5 patients (18%). The median survival was 18years and the probability of survival at 5 and 10years was 89% and 69%, respectively., Conclusions: Conjunctival melanoma is a rare disease, usually undervalued by the patient as well as being underdiagnosed, leading to insufficient and delayed treatment. Early diagnosis and treatment are essential to prevent recurrences and systemic extension, as well as to preserve vision and life., (Copyright © 2019 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.)

The aim of this study is to assess the results of episcleral brachytherapy as treatment of retinal vasoproliferative tumors (RVTs) in a referral Intraocular Tumors Unit (ITU). A retrospective review of all patients diagnosed with vasoproliferative tumors of the retina and treated with episcleral brachytherapy in the ITU, University Hospital of Valladolid between 2009 and 2015 was done. Five patients accomplished the inclusion criteria. All of them presented associated exudation and secondary retinal detachments (RD). Four patients had received prior treatments. Decreased tumor size and exudation regression was found in all cases after treatment. Visual acuity remained stable or increased in all patients. No recurrences have been found after twelve-months follow up. The results of the present study suggest that episcleral brachytherapy is an efficient and safe option in the management of vasoproliferative tumors, especially when large tumor or extensive subretinal fluid is present. In these cases episcleral brachytherapy could be considered as a first line treatment.

Purpose: To assess influence of the radiobiological doses, tumor, and treatment features on local control, enucleation rates, overall and disease-specific survival rates after brachytherapy for posterior uveal melanoma., Material and Methods: Local control, enucleation, overall and disease-specific survival rates were evaluated on the base of 243 patients from 1996 through 2016, using plaques loaded with iodine sources. Clinical and radiotherapy data were extracted from a dedicated prospective database. Biologically effective dose (BED) was included in survival analysis using Kaplan-Meier and Cox regressions. The 3-, 5-, 10-, and 15-year relative survival rates were estimated, and univariate/multivariate regression models were constructed for predictive factors of each item. Hazard ratio (HR) and confidence interval at 95% (CI) were determined., Results: The median follow-up was 73.9 months (range, 3-202 months). Cumulative probabilities of survival by Kaplan-Meier analysis at 3, 5, 10 and 15 years were respectively: 96%, 94%, 93%, and 87%, for local control; 93%, 88%, 81%, and 73% for globe preservation; 98%, 93%, 84%, and 73% for overall survival, and 98%, 96%, 92%, and 87% for disease-specific survival. By multivariate analysis, we concluded variables as significant: for local control failure - the longest basal diameter and the juxtapapillary location; for globe preservation failure - the longest basal dimension, the mushroom shape, the location in ciliary body, and the dose to the foveola; for disease-specific survival - the longest basal dimension. Some radiobiological doses were significant in univariate models but not in multivariate ones for the items studied., Conclusions: The results show as predictive factors of local control, enucleation, and disease-specific survival rates those related with the features of the tumor, specifically the longest basal dimension. There is no clear relation between radiobiological doses or treatment parameters in patients after brachytherapy., Competing Interests: The authors report no conflict of interest.

Programmed cell death-1/ligand (PD-1/PD-L1) interaction negatively regulates T cell activity. PD-L1 expression in tumor cells, antigen-presenting cells, and lymphocytes of the tumor microenvironment is associated with response to treatment with PD-1/PD-L1 inhibitors, but there is still debate on the cutoff value that correlates with responders. In uveal melanoma (UM), 40% of patients will develop liver metastases and, amongst them, 90% will succumb to their disease. The aim of this study was to analyze PD-L1 expression as a prognostic marker and as a possible therapeutic target for UM. Sixty-seven enucleated eyes from UM patients with relevant clinical information were analyzed. Univariate and multivariate analysis were used to evaluate association of PD-L1 with survival. PD-L1 expression was positive relatively to tumor cells, immune cells, and the tumor and tumor-infiltrating immune cell group scoring in 46, 34 and 55% of the cases, respectively. On univariate analysis, tumor cells and the tumor and tumor-infiltrating immune cell group PD-L1 expression was associated with a longer metastasis-free survival (P = 0.04 and P = 0.007). However, on multivariate analysis, only the tumor and tumor-infiltrating immune cell group positivity was associated with longer metastasis-free survival (P = 0.01). Furthermore, tumor cells and the tumor and tumor-infiltrating immune cell group PD-L1 expression was associated with decreased tumor-infiltrating lymphocytes (P = 0.02). PD-L1, when expressed in uveal melanoma, is associated with better patient outcome and decreased tumor-infiltrating lymphocytes. These results support the consideration of anti-PD-1/PD-L1 therapy in uveal melanoma. To determine the best cutoff value, further studies from patients enrolled in clinical trials treated with PD-1/PD-L1 inhibitors are necessary.

Purpose: To assess the influence of the radiobiological doses, tumor, and treatment features on retinopathy, cataracts, retinal detachment, optic neuropathy, vitreous hemorrhage, and neovascular glaucoma at the authors' institution after brachytherapy for posterior uveal melanoma., Material and Methods: Medical records of 243 eyes with uveal melanoma, treated by iodine brachytherapy between 1996 and 2016 at a single center were analyzed. Clinical and radiotherapy data were extracted from a dedicated database. Biologically effective dose (BED) was included in survival analysis performed using Kaplan-Meier and Cox regressions. Relative survival rates were estimated, and univariate/multivariate regression models were constructed for predictive factors of each item. Hazard ratio and confidence interval at 95% were determined. Variables statistically significant were analyzed and compared by log-rank tests., Results: The median follow-up was 73.9 months (range, 3-202 months). Cumulative probabilities of survival by Kaplan-Meier analysis at 3 and 5 years, respectively, were: 59% and 48% for retinopathy; 71% and 55% for cataracts; 63% and 57% for retinal detachment; 88% and 79% for optic neuropathy; 87% and 83% for vitreous hemorrhage; 92% and 89% for neovascular glaucoma, respectively. Using multivariate analysis, statistically significant risk factors were: age, tumor apical height, dose to foveola, and location of anterior border for retinopathy; age, dose to lens, type of plaque, and tumor shape, for cataracts; age, tumor apical height, and size of the plaque for retinal detachment; age, plaque shape, longest basal dimension, and BED to optic nerve for optic neuropathy; age, tumor apical height, and tumor shape for vitreous hemorrhage; tumor apical height and BED to foveola for neovascular glaucoma., Conclusions: Tumor factors in addition to radiation treatment may contribute to secondary effects. Enhanced clinical optimization should evaluate radiobiological doses delivered to the tumor volume and surrounding normal ocular structures., Competing Interests: The authors report no conflict of interest.

Objective: The purpose of the present study is to review the frequency of intraocular metastases as first presentation of systemic disease, and to identify clinical and tumour characteristics., Methods: Retrospective study of consecutive cases diagnosed of intraocular metástasis at a referral intraocular tumours unit between 1993 and 2014. General, epidemiological and ophthalmological characteristics were recorded., Results: A total of 21 patients, with a mean age 62.7 years (31-89) were diagnosed with intraocular metástasis between 1993 and 2014. Both eyes were affected in 4 cases. Location was choroid in 20 cases. The intraocular tumour was the first manifestation of the systemic disease in 13 patients (61.9%). Primary tumour was breast in 47.6% and lung in 23.8%. Diagnosis of the primary tumour was performed by systemic studies, and only 1 patient required intraocular biopsy. Regarding the treatment, the majority of cases were controlled with systemic therapy, with 4 cases requiring additional external beam radiotherapy, and only one enucleation. No clinical differences were found between the cases with known and unknown systemic neoplasia, except in exudative retinal detachment, which was more frequent in the second group., Conclusions: Although intraocular metastases are the most frequent intraocular tumour, they are not a frequent cause of consultation. In more than half of the cases it is the first presentation of unknown systemic neoplasia as a solitary non-pigmented intraocular mass. Early diagnosis is crucial to establish the appropriate treatment, preserve visual function, and improve the prognosis of the patient., (Copyright © 2017 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.)

Purpose: To describe the time, frequency, and clinical characteristics of treatment failure after I-125 brachytherapy in patients with uveal melanoma treated and followed in a Spanish referral ocular oncology unit., Design: Prospective, consecutive, interventional case series., Methods: Patients diagnosed with uveal melanoma from 1995 to 2016 and treated with episcleral brachytherapy were included. Demographic data collection, ophthalmic evaluation, ultrasound scan, and systemic studies were performed at baseline, every 6 months thereafter for 5 years, and subsequently at annual intervals. Recurrence was defined as presence of tumor growth after treatment. Baseline analysis was performed by descriptive methods and survival by Kaplan-Meier curves., Results: From 732 patients diagnosed with uveal melanoma, 311 were treated with brachytherapy. In the follow-up (mean 79 months, standard deviation = 55), 16 local tumor recurrences (5.1%) were detected. All relapsing patients had choroidal tumors and 15 presented with visual symptoms. All patients were treated with I-125 brachytherapy, and 2 received associated transpupillary thermotherapy. All the eyes were enucleated after recurrence. Kaplan-Meier analysis showed a mean time of recurrence of 3.7 years (standard deviation = 2.94 years, ranging from 1 to 12 years). Three patients had metastasis in the follow-up. Kaplan-Meier analysis showed worse survival for patients with recurrence., Conclusion: Local treatment failure was a relatively infrequent event after I-125 brachytherapy in our series. Recurrences appear not only early but also late in the follow-up. They do not have a distinctive clinical pattern and are associated with poorer survival., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: To collect comprehensive data on choroidal and ciliary body melanoma (CCBM) in children and to validate hypotheses regarding pediatric CCBM: children younger than 18 years, males, and those without ciliary body involvement (CBI) have more favorable survival prognosis than young adults 18 to 24 years of age, females, and those with CBI., Design: Retrospective, multicenter observational study., Participants: Two hundred ninety-nine patients from 24 ocular oncology centers, of whom 114 were children (median age, 15.1 years; range, 2.7-17.9 years) and 185 were young adults., Methods: Data were entered through a secure website and were reviewed centrally. Survival was analyzed using Kaplan-Meier analysis and Cox proportional hazards regression., Main Outcome Measures: Proportion of females, tumor-node-metastasis (TNM) stage, cell type, and melanoma-related mortality., Results: Cumulative frequency of having CCBM diagnosed increased steadily by 0.8% per year of age between 5 and 10 years of age and, after a 6-year transition period, by 8.8% per year from age 17 years onward. Of children and young adults, 57% and 63% were female, respectively, which exceeded the expected 51% among young adults. Cell type, known for 35% of tumors, and TNM stage (I in 22% and 21%, II in 49% and 52%, III in 30% and 28%, respectively) were comparable for children and young adults. Melanoma-related survival was 97% and 90% at 5 years and 92% and 80% at 10 years for children compared with young adults, respectively (P = 0.013). Males tended to have a more favorable survival than females among children (100% vs. 85% at 10 years; P = 0.058). Increasing TNM stage was associated with poorer survival (stages I, II, and III: 100% vs. 86% vs. 76%, respectively; P = 0.0011). By multivariate analysis, being a young adult (adjusted hazard rate [HR], 2.57), a higher TNM stage (HR, 2.88 and 8.38 for stages II and III, respectively), and female gender (HR, 2.38) independently predicted less favorable survival. Ciliary body involvement and cell type were not associated with survival., Conclusions: This study confirms that children with CCBM have a more favorable survival than young adults 18 to 25 years of age, adjusting for TNM stage and gender. The association between gender and survival varies between age groups., (Copyright © 2016 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Clinical Case: The case is presented of a 43 year-old male patient with chronic follicular conjunctivitis, negative bacterial serology, and refractory to local treatment. The incisional biopsy performed showed to be consistent with reactive lymphoid hyperplasia. A year later, a new incisional biopsy showed follicular lymphoma, with no systemic involvement, and he was treated with local radiotherapy., Discussion: When a chronic follicular conjunctivitis is refractory to treatment, it is essential to perform an incisional biopsy to establish the histopathological diagnosis that can range from chronic inflammation, reactive lymphoid hyperplasia to lymphoma. Follicular lymphoma is rare among conjunctival lymphomas, and the staging is indispensable for the correct therapeutic approach., (Copyright © 2014 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

A 68-year-old man diagnosed with choroidal melanoma (CM) in the right eye underwent treatment with episcleral brachytherapy (I125) and transpupillary thermotherapy. Ultrasound, computed tomography, and magnetic resonance imaging were performed and revealed ocular recurrence of CM. Treatment with extended enucleation was performed. Macroscopic and microscopic examinations revealed extraocular extension and malignant cells, respectively. Immunohistochemistry demonstrated tumoral Melan-A and HMB-45 expression. No cytogenic abnormalities were detected with fluorescence in situhybridization of tumor cells using probes against chromosomes 3q27 and 8q24. The patient underwent adjuvant external beam radiotherapy for treatment of residual tumor tissue. This case represents the first reported case of recurrent CM with no cytogenetic abnormalities and the absence of metastatic disease, despite a number of the poorest prognostic factors.

Purpose: To describe the efficacy of episcleral brachytherapy in a choroidal melanoma and circumscribed hemangioma arising in both eyes of the same patient., Case Report: We present the case of a 47 year old man who presented decreased visual acuity a few months preceding initial consult. On fundoscopy, he presented a melanotic lesion in the right eye, and a red-orange choroidal mass in the macular area of the left eye., Material and Methods: B scan-ultrasound, fluorescein, and indocyanine green angiography, confirmed the diagnosis of choroidal melanoma in the right eye, and circumscribed choroidal hemangioma in the left eye. Episcleral brachytherapy with (125)I was performed in both eyes consecutively., Results and Conclusions: Bilateral episcleral brachytherapy successfully treated both tumors, preserving the eyes and useful visual function.

Objective: To analyse the clinical features, treatment and survival of uveal melanoma patients diagnosed in a referral Intraocular Tumours Unit over a twenty-year period., Methodology: A prospective study was performed including five hundred patients, diagnosed between January 1992 and December 2011. Clinical tumour characteristics and treatment were collected in a database in Microsoft@ Access@. The numeric variables were expressed as means of frequency and standard deviation, and the quantitative variables using frequency tables., Results: The mean age of the sample was 62.19 years, with 51.2% females, and 64.2% presented with symptoms. The tumours were small in 31.12% of cases, and large in 23.09%, according to COMS criteria. Hazel green was the iris colour in 42.2% of the cases. The initial treatment was episcleral brachytherapy in 42.4% of the total. The overall mortality rate was 17% and 31.3%, and melanoma-related mortality rate was 11.6% and 14.8%, at 5 and 10 years, respectively., Conclusions: In the serie studied melanomas were more frequent in women, and a higher proportion of darker irides were observed than in other previous studies. Most of the tumours were diagnosed when they became symptomatic and in the early or medium stages, allowing conservative therapies to be used, with brachytherapy being the predominant treatment. The melanoma-related mortality appeared to be lower than previously data published. However, further studies are required on the factors influencing survival., (Copyright © 2013 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.)

Clinical Case: No intraocular biopsy technique is free of risk and all have the possibility of giving false negatives due to the difficulty in obtaining a sufficient sample. A modified chorioretinal biopsy was performed on a patient with suspected choroidal melanoma after negative biopsy with 25G vitrectomy. In addition to removing a solid fragment of tumor material using bimanual surgery, material from the lesion was obtained with the vitreotome to perform cytology, which confirmed the diagnosis of melanoma., Discussion: Cytology obtained through the vitreotome in association with removing a solid sample of the choroidal lesion may improve the efficiency of intraocular biopsy., (Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.)

Purpose: To assess the results of I-125 episcleral brachytherapy (EB) in uveal melanoma: tumour control, visual acuity (VA), eye preservation and survival., Patients: Prospective and consecutive study of patients with a diagnosis of uveal melanoma at the Ocular Oncology Unit in the Valladolid University Teaching Hospital treated with EB between September 1997 and June 2008. Ocular examination and extraocular and systemic extension data were registered in a database at the time of the diagnosis and during the follow-up., Results: Among a total of 310 patients diagnosed between September 1997 and June 2008, 136 were treated with EB (mean age, 58.3). Mean follow-up was 55.3 months. As for tumour type, 66.9% were nodular and 39% mushroom shaped. With respect to size, 80.9% were medium, 7.4% small and 11.8% large. After 4.6 years of follow-up, tumours were controlled in 97.1%, with a 55.1% reduction in mean height; only 2.9% of patients showed recurrence. VA was maintained in 16.2% of patients and 17.6% showed an increase; 33% had retinopathy and 14.6% optic neuropathy. Only 5.1% of patients underwent enucleation due to complications and there has been 1 melanoma-related death to date., Conclusions: I-125 EB is effective in tumour control, allowing preservation of the eye and useful visual function for the majority of patients.

Rhabdomyosarcoma is a rare tumor, with an annual incidence of 4.3 cases per million children. Even thought, it is the most common soft tissue sarcoma in childhood, with a mean age of 6 to 8 years at diagnosis. A 4 year-old boy presented with a history of a fast growing (1-month) nodular lesion in the caruncle of his left eye. Slit lamp examination showed a vascularized solid nodular lesion in the semilunar fold. The lesion was surgically removed obtaining infiltrated edges with tumoral cells. A second surgery was performed with free tumour edges. The diagnosis of embryonal rhabdomyosarcoma, botryoid type, of intermediate differentiation was made. The treatment for botryoid rhabdomyosarcoma is basically surgical with the combined use of adjuvant polychemotherapy. In adolescent or adult patients (not in infants where growth bone disturbances can occur) external beam radiotherapy can be combined with chemotherapy. Rhabdomyosarcomas of the conjunctiva without orbital extension are rarely reported. We presented a case of a child with a rare tumor which we had a high suspicion of malignancy and early diagnosis and treatment and child is free of systemic disease 6 and half years later. Our research group believes that the key in these tumors is the high index of suspicion and early treatment.

Purpose: The aim of this study was to determine the prevalence of ocular and oculodermal melanocytosis (ODM) among patients with uveal melanoma (UM) in a Spanish population., Methods: Retrospective review of the medical records of patients with ODM among patients with UM., Results: Ten (11 eyes) of 400 patients (2.7%) with UM associated had ODM. The mean age at diagnosis of UM among patients with ODM was 62 years. One patient had bilateral tumours. UM was diagnosed during a routine-examination in two cases. All tumours were medium (7/11) or large (4/11) in size, with a mean maximum base of 13 mm and height of 7 mm. No patient had extraocular extension or metastatic disease at diagnosis. Enucleation was done in five cases and I-125-brachytherapy in six. The mean follow-up was 43 months. One patient died because of metastasis 2 years after enucleation; one patient is currently on treatment of systemic metastasis 11 years after., Conclusions: ODM is more frequent in spanish population with UM than in American population. Despite the risk of UM in ODM, it is often diagnosed late when a conservative treatment is not indicated.

Aims: To evaluate episcleral plaque radiotherapy with high-dose (125)I for the treatment of circumscribed choroidal haemangioma (CCH)., Methods: A retrospective review was performed of patients treated for CCH between 1995 and 2007. Brachytherapy was performed with ROPES plaques loaded with (125)I seeds with a target apex dose of 48 Gy. Main outcome measures were regression of CCH, resolution of retinal detachment, visual acuity changes and frequency of radiation-related complications., Results: Eight patients met the inclusion criteria. Mean tumour base was 11.3 (range 7.8-14.3) mm and mean height was 4.4 (range 2.8-6.5) mm. Six patients had an associated retinal detachment and seven macular exudates or oedema. Mean preoperative visual acuity ranged from no light perception to 0.7. Three patients had received prior argon laser treatment. All patients received one radiation treatment, since no new symptoms due to CCH developed during follow-up (mean 83 (range 23-123) months). Tumour regression was found in all cases. Three patients presented radiation retinopathy and one subretinal fibrosis. Visual acuity remained stable in six patients and decreased two or more lines in two patients., Conclusions: Episcleral brachytherapy led to tumour regression and resolution of non-rhegmatogenous secondary retinal detachments in this case series that included large CCH. Visual stabilisation was achieved in most cases. Given the acceptable rate of side effects detected, (125)I episcleral brachytherapy should be considered in large tumours, in tumours with a subfoveal location or extensive subretinal fluid, and in tumours that have failed to respond to other treatments.

Purpose: To design and validate a standard, simple, and reliable iris color classification and to study its distribution in a Spanish population. Iris color has a geographic distribution and has been correlated with different ocular diseases. However, there is no standard and validated iris color classification allowing comparison among different studies., Methods: Classification was made in three grades (blue-gray, hazel-green, brown) and was validated by 3 independent readers. Initially, a preliminary study was made in 50 iris photographs to detect technical hitches. Afterwards, based on this procedure, 221 iris photographs were graded., Results: Measures of interobserver reliability were 0.786 by kappa index with an agreement of 89.6%.Iris color distribution in the Spanish cohort was blue-grey 16.29%, hazel-green 55.2%, and brown 28.5%., Conclusions: This classification is simple, reliable, and easy to use in clinical research and by ophthalmologists or generalists in practice. The Spanish cohort from this study shows a different iris color distribution from those previously published in other countries.

Melanoma is a malignant tumour derived from melanocytes (dendritic cells originated from the neural crest and capable to produce melanin synthesis) that could be established on the skin or less frequently on the uvea. The cellular origin from both kind of melanoma seems to be the same but the melanocytes migrates to the epithelia for cutaneous melanoma, while for uveal melanoma, they migrate to mesodermic tissues. Despite the common origin, both melanomas show extreme differences in their metastatic potential, clinical response to treatments, immune response and genetic alterations. We will describe some of those differences in this review.

Purpose: To report a case of a 5-year-old child with CHARGE association and bilateral conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma treated with topical interferon-alpha., Methods: Case report., Results: A 5-year-old girl, diagnosed with nonclassical CHARGE association and hyper-immunoglobulin M (IgM) syndrome, was referred with a 2-month history of suspected purulent bilateral streptococcal conjunctivitis. Clinical symptoms did not resolve despite multiple antibiotic treatments, and her clinical course was attributed to underlying immunodeficiency. Biomicroscopy showed salmon-colored, nodular lesions occupying both fornices and caruncles. Inferior conjunctival biopsy was performed, and MALT lymphoma was diagnosed. Bilateral treatment was initiated with topical interferon-alpha, applied 3 times a day at a concentration of 5 x 10 U/m/d, for 4 months. Complete regression of symptoms and conjunctival lesions was achieved. No recurrences have been observed after 1-year follow-up., Conclusions: We report the use of topical interferon-alpha as treatment for bilateral conjunctival MALT lymphoma in a young child with CHARGE association and hyper-IgM syndrome.

We report a 29-year-old white female with conjunctival pigmentation after a Stevens-Johnson syndrome (SJS) episode triggered by sulfasalazine. The patient developed bilateral tarsal and forniceal conjunctiva and black pigmentation. Diagnostic biopsy showed stromal monocyte infiltration consistent with chronic phase SJS and conjunctival pigment of melanic origin and not due to drug deposition. Treatment with topical steroids and unpreserved artificial tears resulted in improvement of clinical symptoms; however, pigmentation was unchanged after 2 years.

Unlabelled: To report the major intraretinal pathological changes in retinas with proliferative vitreoretinopathy (PVR) and retinal shortening, 13 human retinal samples from postoperative PVR after primary surgery for retinal detachment were immunostained for vimentin, glial fibrillary acidic protein (GFAP), cytokeratins, and CD68. One more sample was studied with electron microscopy. Retinal disorganization, neuronal loss, and gliosis were observed in 12 out of 13 samples, but all 13 were positive for GFAP. Muller cell processes showed different degrees of intermediate filament hyperplasia. CD68-positive cells were present in 11 of 13 retinal samples., Conclusion: A gliotic response plays a major role in retinal shortening in PVR. In addition, the presence of macrophage-like cells in retinal tissues suggests a possible role of these cells in the pathogenesis of this variety of PVR., (Copyright (c) 2006 S. Karger AG, Basel.)

Case Report: We report an unusual case of cavitary choroidal melanoma. The results of ultrasonography, magnetic resonance imaging, computed tomography, and immunohistochemical studies are presented for a 38-year-old woman who developed an amelanotic tumor in the posterior choroid. B-scan ultrasonography disclosed intratumoral cavitations. Systemic and extraocular extension studies were negative. Enucleation was performed and histopathologic examination showed a choroidal melanoma of spindle cell type, with intratumoral cavitations lined by flattened tumor cells., Comments: The majority of previous reports of intraocular cavitary tumors describe cavitary ciliary body tumors. Uveal melanoma should be included in the differential diagnosis of choroidal cavitary lesions. As far as we know, this is the second documented clinicopathologic correlation of a cavitary choroidal melanoma.