New insights on circumscribed choroidal hemangioma: "bench to bedside".

Circumscribed choroidal hemangioma is a rare vascular hamartoma of the choroid, presenting as a red-orange mass at the posterior pole on fundoscopic examination. Despite its benign origin, associated complications such as subretinal fluid, serous retinal detachment, retinoschisis and neovascular glaucoma may lead to serious visual impairment in more than half patients. Because of its similarity to amelanotic choroidal melanoma and choroidal metastasis, differential diagnosis is still challenging for specialists. Multimodal imaging such as ultrasonography, fluorescein angiography, indocyanine green angiography, optical coherence tomography, and optical coherence tomography angiography guides the clinician to the correct diagnosis and the proper follow-up. Treatment is indicated in symptomatic cases in order to resolve exudation and improve visual acuity. Treatment options include photocoagulation, transpupillary thermotherapy, radiation therapy, photodynamic therapy and anti-vascular endothelial growth factor therapy. Currently, photodynamic therapy is the treatment of choice due to its effectiveness and safety. The purpose of this review is to describe the latest knowledge in the etiopathogenesis of the circumscribed choroidal hemangioma, the most recent multimodal imaging findings, and the available treatment options.

Swept-source optical coherence tomography and swept-source optical coherence tomography angiography findings in circumscribed choroidal hemangioma before and after transpupillary thermotherapy.

PURPOSE: To investigate the swept-source optical coherence tomography (SS-OCT) and SS-OCT angiography (SS-OCTA) findings in circumscribed choroidal hemangioma (CCH) before and after treatment with transpupillary thermotherapy (TTT). METHODS: The clinical records of 21 eyes having CCH imaged with SS-OCT/SS-OCTA between September 2018 and December 2022 were evaluated. RESULTS: SS-OCT examination in CCH showed dome-shaped appearance (100%), choroidal shadowing (100%), expansion of choroidal structures (100%), subretinal fluid (66.7%), intraretinal edema/schisis (33.3%), retinal pigment epithelium (RPE) atrophy (19.0%), hyperreflective dots (19.0%), and epiretinal membrane (4.8%). Internal arborizing tumor vessels showing hyperreflectivity were observed in the choriocapillaris slab on SS-OCTA in all eyes. In the deep capillary plexus (DCP), flow void changes were seen in 7 eyes with intraretinal schisis/cystoid macular edema. Four CCHs > 2 mm in thickness showed outer retinal involvement due to unmasking of flow in intratumoral vessels related to RPE atrophy. Following TTT/indocyanine green-enhanced TTT (ICG-TTT) of CCH, SS-OCT findings included total/partial resolution of subretinal fluid (57.1%), complete/partial regression of the tumor (52.4%), and RPE atrophy (33.3%). After treatment; loss of choriocapillaris, decrease in tumor vascularity together with increase in the fibrous component and flow void areas were detected on SS-OCTA. CONCLUSIONS: SS-OCT/SS-OCTA are useful non-invasive tools for imaging the structural/vascular changes in CCHs managed with TTT or ICG-TTT. On SS-OCTA, hyporeflective spaces localizing to edema/schisis in the DCP and arborizing tumor vessels within a hyporeflective stromal background in the choriocapillaris slab were observed. After TTT/ICG-TTT, a decrease in tumor vessels and an increase in the fibrous component and flow-void areas inside the CCH were detected on SS-OCTA.

Management of circumscribed choroidal hemangioma at a tertiary care center.

PURPOSE: To review long-term outcomes of circumscribed choroidal hemangioma (CCH). METHODS: Hospital charts of all CCH cases diagnosed from 2008 to 2019 were retrospectively reviewed. RESULTS: All 172 patients were managed with either observation, transpupillary thermotherapy, argon laser photocoagulation, photodynamic therapy, plaque brachytherapy or stereotactic radiosurgery. The most common 3 modes of management were clinical observation (30.2%), transpupillary thermotherapy (52.9%) and argon laser photocoagulation (8.7%). Median follow-up time was 10 months (range: 3, 160). Anatomical outcomes were stable in 87.1% of observation group and improved in 60.5% of thermotherapy group. Quantified optical coherence tomography angiography findings showed statistical differences in vascular and perfusion densities in fellow eyes of hemangioma patients. CONCLUSION: Circumscribed choroidal hemangioma can be treated in various ways. Transpupillary thermotherapy is an anatomically effective treatment in selected cases. The diagnosis of CCH may have vascular implications in fellow eyes of the patients.

[Predicting the effectiveness of organ-preserving treatment of choroidal melanoma using optical coherence tomography data]. / Prognozirovanie effektivnosti organosokhrannogo lecheniya melanomy khorioidei po dannym opticheskoi kogerentnoi tomografii.

Optical coherence tomography (OCT) is currently widely used for the diagnosis of choroidal melanoma (CM), but the problem of predicting the outcomes of planned CM treatment remains unsolved. PURPOSE: This study was conducted to identify OCT signs that adversely affect the outcome of organ-preserving CM treatment. MATERIAL AND METHODS: OCT scan images of 30 patients who underwent organ-preserving treatment and were under observation were selected for this study. Brachytherapy (BT) as monotherapy was performed in 27 patients (in 2 cases - twice, and in 1 case - three times), in one patient - in combination with the previous transpupillary thermotherapy (TTT). Multiple TTT (4 sessions within 4 months) as monotherapy were performed in 2 patients. In 9 cases, a single organ-preserving treatment (BT - 6 patients, TTT - 3 patients) was ineffective. In these cases, the effectiveness of the first stage of organ-preserving treatment was taken into account. RESULTS: Seven signs of an unfavorable prognosis of the performed treatment were identified by analyzis of tomograms and statistical processing of the obtained data. These signs include: the presence of intraretinal edema, detachment of the neuroepithelium (NED) over the tumor, including with a break in the photoreceptors, accumulation of transudate over the tumor, the presence of large cysts, intraretinal cavities and NED near the tumor (secondary retinal detachment). A combination of three or more signs were observed in all cases of inefficiency of the first stage of treatment. Most often, intraretinal edema and NED over the tumor were combined with the accumulation of subretinal transudate and NED near the tumor. The presence of 6 or all 7 signs took place in cases of a negative therapeutic effect after local destruction. CONCLUSION: When planning organ-preserving CM treatment, in addition to biometric parameters, it is necessary to pay special attention to the identification of such morphological signs as NED over and near the tumor, accumulation of transudate under the NED, the presence of intraretinal edema, large intraretinal cysts and cavities.

Alterations of choroidal circulation and choroidal thickness before and after chemoradiotherapy in a case of metastatic choroidal tumor.

BACKGROUND: Metastatic choroidal tumors are hematogenous intraocular metastases of malignant tumors in systemic organs; however, the details of choroidal circulation and morphological changes in the choroid are unknown. The aim of this study is to present a case of metastatic choroidal tumor and examine laser speckle flowgraphy (LSFG)-based choroidal circulation and central choroidal thickness (CCT) before and after chemoradiotherapy. CASE PRESENTATION: A 66-year-old woman with a medical history of breast cancer 16 years ago was referred to our department struggling with blurred vision in her right eye. At the time of initial examination, her best-corrected visual acuity (BCVA) was 0.4 oculus dexter (OD) and 0.9 oculus sinister. Fundus revealed a yellowish-white choroidal elevated lesion measuring 8 papillary diameters with serous retinal detachment (SRD) in the posterior pole. Fluorescein angiography showed diffuse hyperfluorescence and fluorescent leakage due to SRD, and indocyanine green angiography demonstrated no abnormalities in the macula but hypofluorescence in the center of the tumor. Based on these clinical findings, she was diagnosed with metastatic choroidal tumor. After chemoradiotherapy, the metastatic choroidal tumor became scarred, and SRD disappeared. The rate of changes in macular blood flows assessed by mean blur rate on LSFG and CCT of her right eye were 33.8 and 32.8% decrease at 5 months after the initial visit, respectively. BCVA was 0.5 OD 27 months after the initial examination. CONCLUSION: Chemoradiotherapy resulted in regression of the metastatic choroidal tumor and disappearance of SRD, with a decrease in central choroidal blood flow and CCT. The choroidal blood flow on LSFG could reflect an increased oxygen demand by cancer cells invading the choroid and substantial blood supply.

Choroidal melanoma tumor profile and treatment pattern for newly diagnosed patients at a reference public hospital in Sao Paulo, Brazil.

BACKGROUND: Choroid, ciliary body, and iris melanomas are often grouped as uveal melanoma, the most common intraocular primary malignancy. The purpose of the current study was to analyze the tumor profile of newly diagnosed cases of choroidal melanoma at a reference center in Sao Paulo, Brazil, and to investigate the frequency of eyes treated by enucleation that could have been treated with brachytherapy if available in the service. METHODS: Medical records of patients referred to our service with initial diagnostic hypothesis of choroidal melanoma from July 2014 to June 2020 were analysed on demographics, diagnosis confirmation, tumor measurement by ultrasonography and established treatment. Data were evaluated on clinical and demographic characteristics as age, sex, affected eye, ultrasound parameters, and treatment management of patients with clinically diagnosed choroidal melanoma. Among the patients submitted to enucleation, we investigated how many could have been selected to receive brachytherapy. RESULTS: From the 102 patients referred with the choroidal melanoma diagnosis hypothesis, 70 (68.62%) were confirmed. Mean measurements from the tumors in millimetres were: 9.19 ± 3.69 at height and 12.97 ± 3.09 by 13.30 ± 3.30 at basal. A total of 48 cases (68.57%) were enucleated, 8 (11.43%) were treated by brachytherapy in a different service, and 14 patients (20.00%) returned for enucleation at their original referral center. Out of the 48 patients enucleated, 26 (54.17%) could have been selected to brachytherapy treatment. CONCLUSIONS: The results indicate a late diagnosis of choroidal melanoma cases referred to our service. Most enucleated cases could have been treated with brachytherapy if it was broadly available at the national public health insurance. Further public health political efforts should focus on early diagnosis and better quality of life post-treatment for oncologic patients.

No differences in the long-term prognosis of iris and choroidal melanomas when adjusting for tumor thickness and diameter.

OBJECTIVE: To assess the long-term prognosis for patients with iris melanomas and compare it with the prognosis for small choroidal melanomas. DESIGN: Retrospective observational case series. METHODS: All patients treated for iris melanomas at a single referral institution between January 1st 1986 and January 1st 2016 were included. Patients treated for small choroidal melanomas during the same period were included for comparison. The cumulative incidence of melanoma-related mortality was calculated. Patient and tumor characteristics and size-adjusted hazard ratio (HR) for melanoma-related mortality were compared between iris and small choroidal melanomas. RESULTS: Forty-five iris melanomas and 268 small choroidal melanomas were included. Twenty-four iris melanomas (53%) had been treated with local resection, 12 (27%) with Ruthenium-106 brachytherapy, 7 (16%) with enucleation and 2 (4%) with proton beam irradiation. Twenty-one (68%), 7 (16%) and 2 (4%) of the iris melanomas were of the spindle, mixed and epithelioid cell types, respectively. Twenty-three patients had deceased before the end of follow-up. Median follow-up for the 22 survivors was 13.3 years (SD 9.4). Patients with iris melanomas were more often asymptomatic at presentation and had a trend towards significantly lower age (59 versus 63 years, Student's T-tests p = 0.057). Further, iris melanomas had significantly smaller basal diameter (5.8 versus 8.0 mm, p < 0.0001) and tumor volume (79 mm3 versus 93 mm mm3, p < 0.0001) but greater thickness (3.0 versus 2.5 mm, p < 0.0001). The cumulative incidence of iris melanoma-related mortality was 5% at 5 years after diagnosis, and 8% at 10, 15 and 20 years. The incidence was not significantly different to small choroidal melanomas (Wilcoxon p = 0.46). In multivariate Cox regression with tumor diameter and thickness as covariates, patients with choroidal melanomas did not have increased HR for melanoma-related mortality (HR 2.2, 95% CI 0.5-9.6, p = 0.29). Similarly, there were no significant survival differences in matched subgroups (Wilcoxon p = 0.82). CONCLUSIONS: There are no survival differences between iris and choroidal melanomas when adjusting for tumor size. The reason for the relatively favorable prognosis of iris melanomas compared to melanomas of the choroid and ciliary body is likely that they are diagnosed at a smaller size.

Survival in small choroidal melanocytic lesions with risk factors managed by initial observation until detection of tumour growth.

BACKGROUND: The main objective was to describe metastatic and survival rates in patients with small choroidal melanocytic lesions initially managed by observation. METHODS: Retrospective, observational study of consecutive cases recruited from 2001 through 2018, followed for a median (mean, range) of 81.0 (89.3, 10-204) months in a tertiary referral centre for ocular oncology. Seventy-five consecutive patients diagnosed with small choroidal melanocytic lesions with risk factors for growth initially observed and who showed progression during follow-up. Treatment was performed (plaque radiotherapy or enucleation in 96% and 4% of cases, respectively) at detection of tumour growth. RESULTS: Median (mean, range) tumour thickness was 2.2 (2.23, 1.08-3.40) mm, and median maximum basal diameter was 8.5 (8.16, 4-12) mm. At diagnosis, a median (mean, range) of 5 (5.48, 1-8) risk factors for progression were present. Lesions grew at a median (mean, range) rate of 0.42 mm/y (1.12, 0-7.68) in thickness and 1.05 mm/y (3.14, 0-4.8) in maximum diameter. Median (mean, range) time until growth was 17.00 (32.6, 1-161) months post-diagnosis, at which time tumours were treated. Five patients developed local recurrence after brachytherapy requiring enucleation. Four patients developed hepatic metastasis. Melanoma-specific survival was 98% at 5 years (95% CI, 94.2-100%) and 91.6% (95% CI, 82-100%) at 10 and 15 years. CONCLUSION: In small melanocytic lesions with risk factors for growth, initial observation until detection of tumour growth results in a seemingly low risk of metastasis, suggesting that this may be an initial approach to consider in tumours with indeterminate malignant potential.

[Evaluation of the effectiveness of organ preservation treatment of choroidal melanomas by optical coherence tomography findings]. / Otsenka effektivnosti organosokhrannogo lecheniya melanom khorioidei po dannym opticheskoi kogerentnoi tomografii.

Achieving local tumor control is generally considered to be the criterion of effectiveness of organ preservation treatment. In most cases it can be assessed by ophthalmoscopy and ultrasonography. However, in presence of pigmentation areas, it can be difficult to interpret the ophthalmoscopy and ultrasonography findings. The works concerning the informativity of optical coherence tomography (OCT) after organ preservation treatment of choroidal melanomas (CM) generally cover the complications (post-radiation maculopathy, neuropathy) and the identification of vitreomacular tractions. In evaluation of local control, attention is paid to the presence of subretinal fluid and/or retinal pigment epithelium (RPE) detachment. PURPOSE: To analyze OCT findings in terms of the condition of eye fundus tissues in various outcomes of organ preservation treatment of CM. MATERIAL AND METHODS: The study included 20 patients (20 eyes) who underwent the following organ preservation therapy 1 to 374 months prior to enrollment: transpupillary thermotherapy (TTT) - 8 eyes, brachytherapy (BT) - 8 eyes, BT with subsequent TTT - 4 eyes. Mean age of patients at the time of therapy start was 59.25±10.86 (33-81) years. In multi-step therapy (12 eyes), the mean interval between the stages was 28.08±20.39 months. According to echobiometry findings, tumor prominence at the time of therapy start was 3.35±2.08 (1-7.37) mm. At the time of post-treatment OCT scan, there was no prominence in 5 eyes, in other eyes it was 1.26±1.24 (0.4-5.7) mm. RESULTS: Comparison of ophthalmoscopy picture with OCT findings allowed identification of the following changes: a scar and hyperplasia of pigment epithelium in the scar, a scar with reactive pigment hyperplasia at the level of RPE and choroid, radioreaction, growth area from under and inside the scar, incomplete local control, absence of local control. CONCLUSION: The author's own expertise in evaluating the effectiveness of local choroidal melanoma therapy suggests that optical coherence tomography should be used to monitor all patients being followed up after organ preservation treatment of choroidal melanoma: after transpupillary thermotherapy - for 3 months, after brachytherapy - during first 24 months, and in case of doubts for longer.

Choroidal melanocytic tumors in dogs: A retrospective study.

OBJECTIVE: To describe clinical features of dogs diagnosed with choroidal melanocytic tumors at a veterinary teaching hospital. ANIMALS STUDIED: Retrospective case series of 13 dogs (14 melanocytic tumors) with choroidal melanocytic tumors. PROCEDURES: Medical records of dogs evaluated by the Cornell University ophthalmology service with a clinical diagnosis of a choroidal melanocytic tumor between 2008 and 2020 were reviewed. A choroidal melanocytic tumor was clinically defined as any well-demarcated, raised pigmented choroidal lesion underlying the retina. Histopathology results were consulted when available. Signalment and clinical features were recorded, including fundoscopic location, histopathologic findings, treatment, and outcome. RESULTS: Choroidal melanocytic tumors were identified in 14 eyes of 13 dogs. The mean (±standard deviation) of dogs was 8.6 (±3.5) years. Seven different breeds were represented, with Labrador/Labrador mix being the most common. Ten of the 14 melanocytic tumors were diagnosed incidentally. Four dogs presented for vision loss and ocular discomfort, and diagnosis was made on histopathology examination following enucleation. Pulmonary metastasis was suspected in one dog with an incidentally found choroidal melanocytic tumor OD. Fundoscopic location was available for 8 melanocytic tumors, with 6 (75%) located in the tapetal fundus. Histopathologic diagnosis was melanocytoma for 3 and malignant melanoma in 1 globe, with optic nerve head invasion in 50%. Local recurrence was not seen in any orbit following enucleation. CONCLUSION: Choroidal melanocytic tumors are uncommon in dogs. Metastasis appears to be rare and was only suspected in one dog, but intraocular tumor growth can lead to retinal detachment, glaucoma, and necessitate enucleation.

Treatment response to intravitreal bevacizumab in small pigmented choroidal lesions with subretinal fluid.

BACKGROUND: To describe the effects of intravitreal bevacizumab injection (IVB) and/or transpupillary thermotherapy (TTT) in the treatment of small pigmented choroidal lesions with subfoveal fluid (SFF), and to investigate prognostic value of the therapeutic response in future tumor growth. METHODS: Retrospective chart review of 19 patients, who were diagnosed with choroidal neovascularization (CNV)-free small pigmented choroidal lesions and treated with IVB and/or TTT, was performed. RESULTS: Complete resolution of SFF was achieved in two eyes (2/14; 14.3%) after IVB, and in three eyes (3/4; 75%) after TTT. Best corrected visual acuity was improved in two eyes (2/9; 22%) after IVB, and in three eyes (3/4; 75%) after TTT. Among five patients who underwent TTT after IVB, four patients (4/5; 80%) demonstrated additional advantage. All IVBs could not reduce tumor sizes. Rather, tumor growth was detected in seven out of 14 eyes (7/14; 50%) that underwent IVB. None of the patients who underwent TTT showed tumor growth. The lack of treatment response to IVB was suggestive of malignancy, as most small pigmented lesions that had no response to IVB showed tumor growth (86%, p = 0.010). CONCLUSION: IVB was not effective in reducing tumor size and subfoveal fluid in small pigmented choroidal lesions. Therapeutic response to IVB can be used as an indicator between melanoma and nevus in small pigmented choroidal lesion.

Retinal fluid changes and therapeutic effects in symptomatic circumscribed choroidal hemangioma patients: a long-term follow up study.

BACKGROUND: Changes in retinal fluid patterns associated with circumscribed choroidal hemangioma (CCH) have not been investigated yet. A long-term follow-up study was performed to evaluate the changes of retinal fluid patterns and treatment responses. METHODS: We retrospectively reviewed medical records of all CCH patients diagnosed between November 2005 and March 2017. Enrolled patients had visual symptoms, were treatment-naïve, and had been followed-up for more than 2 years. Best corrected visual acuities (BCVA) and the presence, severity, and pattern change of the subretinal fluid (SRF) and intraretinal fluid (IRF) in the macula on optical coherence tomography (OCT) were analyzed at initial presentation and follow-up visits. RESULTS: Twenty-six patients were enrolled. All patients received one or more of the following treatments: PDT, TTT, and intravitreal bevacizumab (Avastin) injection (IVB). Primary therapy consisted of PDT in 9 patients (34.6%), TTT in 7 patients (26.9%) and IVB in 10 patients (38.5%). At initial presentation, the SRF-only pattern was mostly observed. Despite treatment, IRF occurred over time; eventually, advanced cystoid macular oedema (CME) developed. In terms of retinal fluid reduction, PDT was most efficacious (9/9, 100%), and TTT and IVB showed moderate efficacy (TTT: 4/7, 57.1%; IVB: 5/10, 50%) as a primary therapy. After advanced CME developed, IVB and TTT showed no or minimal effect (TTT: 0/1, 0%; IVB: 0/19, 0%), and PDT was the only effective therapy (6/10, 60%). CONCLUSION: The pattern of retinal fluid accompanied by CCH evolved from an SRF-only pattern initially to an advanced CME pattern. The effectiveness of treatments decreased over time, and advanced CME generally showed resistance to treatments. PDT would be the most recommended treatment.

[Local treatment of choroidal melanoma: possibilities and limitations]. / Lokal'noe lechenie melanom khorioidei: vozmozhnosti i ogranicheniia.

Local treatment of choroidal melanoma (CM) attracts the attention of many ophthalmology specialists, especially in recent years as the capabilities to target irradiation at small objects and possibilities of surgical interventions on the eyeball have expanded. The article discusses the medical indications for local treatment of CM. Review of literature of the last 16 years and author's own observations on CM patients who underwent almost all kinds of conventional methods of local treatment allowed thorough analysis of indications and counter-indications for their usage. Among the authors who favor local destruction and removal of large CM, the main indication is the possibility to preserve vision and anatomical structures of the eye. This led to unreasonably wide spread of local destruction (removal) of large CM, primarily the endovitreal resection method. However, such metastasis risk factors as CM size and its localization are being overlooked. Literature analysis and author's own observations helped validate the unsafety of the local treatment of large CM. The article features long-term results of contact and distant radiation therapy, and presents CM metrics for best therapeutic effect.

[Late complications of choroidal melanoma brachytherapy and possibility of their prevention]. / Pozdnie oslozhneniia brakhiterapii melanom khorioidei i vozmozhnosti ikh profilaktiki.

Complications and the frequency of their occurrence as the criteria of eye preservation after brachytherapy (BT) are presented in literature with inconsistencies due to the assessment being done after varied follow-up periods (1 month to 5-10 years). The evaluation of complications occurring after applying radioiodine can be found in literature. The complications after BT beta particle emitting ophthalmic applicators are seldom discussed in articles. PURPOSE: to examine the frequency of complications, the characteristics of their development after choroidal melanoma (CM) brachytherapy with ruthenium ophthalmic applicators (OA) during long-term follow-up and determine the possibility of their prevention. MATERIAL AND METHODS: Characteristics of postradiation period after BT were studied in 200 patients with CM (200 eyes). Among them, 127 patients were treated by authors of this study, 73 were provided treatment by other specialized medical facilities in Moscow. The study involved 84 male and 116 female patients aged 56.14±12.8 in average. The follow-up periods spanned 2 to 39 years (mean length 9.84±6.16 years). BT was done only with ruthenium OA (beta radiation) manufactured in Russian. RESULTS: Follow-up period after BT for 83% of patients was 5 years. Complications occurred in 68.5% of patients. Complications were found in 74.63% of patients with CM localized pre-equatorially and in 65.4% of patients with CM localized in the posterior part of the eye. Complications associated with radiation-induced injuries in crystalline lens, retina and choroidal blood vessels were more frequent. CONCLUSION: The frequency of complications depends on the size of CM and its localization. Complications were the least frequent in patients with CM thickness of 5 mm or less.

The Pediatric Choroidal and Ciliary Body Melanoma Study: A Survey by the European Ophthalmic Oncology Group.

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.

BRACHYTHERAPY ALONE OR WITH NEOADJUVANT PHOTODYNAMIC THERAPY FOR AMELANOTIC CHOROIDAL MELANOMA: Functional Outcomes and Local Tumor Control.

PURPOSE: To compare visual outcomes and local tumor control between two groups of patients with amelanotic choroidal melanoma treated with brachytherapy alone, or neoadjuvant photodynamic therapy before brachytherapy. METHODS: Patients diagnosed with amelanotic choroidal melanoma were recruited for the study and divided into two groups: brachytherapy alone (Group A) and photodynamic therapy preceding brachytherapy (Group B). Patients of both groups were selected to be comparable. RESULTS: Twenty-six patients with amelanotic choroidal melanoma were enrolled in the study. Within Group B, 1 month after photodynamic therapy, ultrasonography showed reduction of tumor height in 11 patients (73.4%). The mean doses of irradiation to macula and optic nerve, at baseline were 74.37 and 52.07 Gy, whereas after photodynamic therapy there was a decrease of 17.26% (P = 0.008) and 21.22% (P = 0.025), respectively. In terms of visual acuity, a mean decrease of 14 ETDRS letters and 5 ETDRS letters was observed at 24 months follow-up, in Groups A and B, respectively (P = 0.001). CONCLUSION: Photodynamic therapy as neoadjuvant therapy before brachytherapy reduces tumor thickness in 73.4% of cases. As a result, a decrease of radiation toxic effects on visual function could be obtained, without compromising disease control.

Recurrence of choroidal melanoma after Ru- 106 brachytherapy.

AIM: The aim of the study was to analyse the recurrence rates of choroidal melanoma treated with Ru-106 brachytherapy with or without adjunctive transpupillary thermotherapy. MATERIAL AND METHODS: We analysed medical records of 97 patients treated with Ru-106 brachytherapy with or without adjunctive transpupillary thermotherapy who subsequently presented with recurrence. All patients were treated at the Department of Ophthalmology and Ocular Oncology in Krak6w between 1995 and 2006. RESULTS: The mean time to recurrence was 29.15 months and tended to be shorter in larger melanomas. The 5-year recurrence rate determined using Kaplan-Meier estimator was 28.69%. Marginal recurrence was the most common type of recurrence, especially in tumors adjacent to the optic disc. The recurrence rates were the highest for all types of recurrence after the use of COB plaque with or without adjunctive transpupillary thermotherapy. CONCLUSION: The highest incidence of recurrence was shown in the second year following the Ru-106 brachytherapy and the time to recurrence tended to be shorter in larger melanomas. Marginal recurrence was the most common type of recurrence, especially in tumors adjacent to the optic disc.