Uveal melanoma incidence and survival analysis in Portugal between 2013 and 2022.

PURPOSE: This study aims to provide a comprehensive overview of the clinical characteristics and epidemiology of uveal melanoma (UM) in the Portuguese population, evaluated at the National Reference Centre (NRC). METHODS: A prospective observational study was conducted, involving patients consecutively diagnosed with UM at the Portuguese NRC between July 2013 and December 2022. The study collected data on demographic and tumour characteristics, clinical staging according to the American Joint Committee on Cancer (AJCC), treatment approaches, local disease control, patient survival, and the occurrence of distant metastases. RESULTS: The study included a total of 316 patients, 53.8% female. The mean age at diagnosis was 61.8±14.2 years, and 75.0% of patients presented with symptoms. The mean annual age-adjusted incidence of uveal melanoma in Portugal between 2014 and 2022 was 2.4 cases per million (95% confidence interval [CI]: 2.1-2.8). For choroidal/ciliary body tumours, the overall cumulative survival and distant metastases-free survival (DMFS) rates at 5 years were 84.9% (95% CI: 78.7-91.1) and 79.4% (95%CI: 72.8-86.0), respectively. Notably, higher AJCC stages at presentation, the need for enucleation, and increased tumour thickness were associated with lower DSS and DMFS rates. CONCLUSION: This study represents the most extensive analysis of UM epidemiology within the Portuguese population. The findings underscore the importance of early diagnosis and treatment in UM, as lower AJCC stages and smaller tumour thickness at diagnosis correlate with improved DSS and DMFS.

Epidemiological and histopathological aspects of ocular melanomas in Northeastern Romania.

Ocular melanoma is a rare but complex disease in current medical practice. Our retrospective study spans over a period of 28 years and analyzed uveal and conjunctival melanomas that were consecutively admitted, diagnosed, and treated in the 2nd Ophthalmology Clinic of Prof. Dr. Nicolae Oblu Emergency Clinical Hospital, Iasi, Romania. The patients were selected from the records of the Department of Pathology of our Hospital, being diagnosed by standard histopathological techniques. The aim of this study was to summarize the epidemiological and pathological aspects of uveal and conjunctival melanomas in Northeastern region of Romania. In our study, we did not notice a predilection of uveal and conjunctival melanoma to one particular gender. The most common histological subtypes of ocular melanomas were the heavily pigmented spindle cell subtype, followed by the epithelioid subtype. Our patients sought medical help in a timely manner, before the systemic invasion of the disease could develop.

Incidence of Strabismus Post-Plaque Brachytherapy in Patients With Uveal Melanoma.

PURPOSE: To determine the incidence and type of strabismus in patients with uveal melanoma treated with plaque brachytherapy. DESIGN: Multicenter, retrospective incidence estimation study. METHODS: A total of 438 eyes of 438 patients with uveal melanoma treated with plaque brachytherapy between October 2011 and May 2021. Intervention was Iodine 125, and Palladium 103 plaque brachytherapy. The variables reviewed included incidence of nonresolving strabismus post-plaque brachytherapy, type of strabismus developed, extraocular muscles operated, and modality of treatment received. RESULTS: A total of 438 patients underwent plaque brachytherapy treatment for uveal melanoma. Eleven patients developed strabismus post-plaque brachytherapy (2.5%, n = 11/438). Of these patients, 5 (1.1%, n = 5/438) developed strabismus immediately postoperation. Specifically, 2 patients (0.5%, n = 2/438) developed strabismus immediately postoperation due to slipped muscles, 2 patients (0.5%, n = 2/438) due to decompensated phorias, and 1 patient (0.5%, n = 1/438) due to a fibrotic muscle. Six patients (1.4%, n = 6/438) developed late-onset sensory strabismus. A total of 355 patients (81.1%, n = 355/438) had their extraocular muscles disinserted during surgery, with the lateral rectus being the most common, accounting for 45.4% (n = 161/355), followed by the superior rectus at 26.8% (n = 95/355). Strabismus surgery was the most common treatment modality, comprising 72.7% (n = 8/11) of patients. CONCLUSIONS: The incidence of strabismus after plaque brachytherapy treatment for uveal melanoma was low and primarily classified as late-onset sensory strabismus. Previous studies may underestimate the long-term incidence of strabismus after plaque brachytherapy by focusing primarily on strabismus present immediately postoperatively.

Trends in the Clinical Presentation of Uveal Melanoma During the COVID-19 Pandemic.

BACKGROUND AND OBJECTIVE: The objective was to evaluate factors associated with clinical presentation of uveal melanoma (UM) during the initial two years of the coronavirus disease 2019 pandemic. PATIENTS AND METHODS: This was a multi-site, retrospective cohort study of patients treated for uveal melanoma during the first (early) and second (late) year of the pandemic compared with the year prior (control). RESULTS: A total of 48, 67, and 75 patients were in the control, early, and late cohorts, respectively. The early cohort had a higher frequency of large tumors (control: 29.2%, early: 40.3%, late: 29.3%; P < 0.001) at presentation. Both the early and late cohorts had higher rates of enucleation (control: 8.33%, early: 20.9%, late: 18.67%; P ≤ 0.0338) compared to the control cohort. CONCLUSIONS: While there was an increase in large tumors along with a rise in enucleation during the first year of the pandemic, enucleation rates remained elevated even while tumor sizes normalized. [Ophthalmic Surg Lasers Imaging Retina 2024;55:278-284.].

Iris melanoma: Review of clinical features, risks, management, and outcomes.

Primary uveal melanoma is rare and affects approximately 8,000 persons per year worldwide. This malignancy can involve the iris, ciliary body, and choroid. Of these three structures, the iris is the least commonly affected site, representing only 4% of all uveal melanomas. Iris melanoma can arise from iris melanocytic nevus, iris melanocytosis, or de novo. In a longitudinal study of 1,611 patients with iris nevus, transformation into melanoma, using Kaplan-Meier estimates, was found in 2.6% by five years and in 4.1% by 10 years. The factors that predicted growth of iris melanocytic nevus into melanoma are denoted by a letter (ABCDEF) guide: A for age ≤40 years old at presentation (hazard ratio [HR] = 3, P = .01), B for blood (hyphema) (HR = 9, P < .0004), C for clock hour of tumor inferiorly (tumor location) (HR = 9, P = .03), D for diffuse flat tumor configuration (HR = 14, P = .02), E for ectropion uveae (HR = 4, P = .002), and F for feathery ill-defined margins (HR = 3, P = .02). At diagnosis, iris melanoma has a mean cross-sectional diameter of 5.5 mm and thickness of 2.1 mm, often with tumor seeding (28%) and secondary glaucoma (35%). We provide a comprehensive review of iris nevus and melanoma to explore relevant demographic and clinical data, risk factors for tumor growth, management, and prognosis, with the hope that clinicians will be more comfortable in understanding this rare malignant condition.

Additional primary malignancies in a Polish cohort of uveal melanoma patients: a review of 644 patients with long-term follow-up.

AIM: To investigate the frequency and location of additional primary malignancies in a Polish cohort of uveal melanoma (UM) patients registered in a single centre database. MATERIAL AND METHOD: Retrospective data analysis of patients treated for uveal melanoma at the Department of Ophthalmology, Poznan University of Medical Sciences, Poland between 1991 and 2017. Data on the diagnosis of the additional malignancies were obtained during the follow-up visits in our Department and/or from the Greater Poland Cancer Registry. The exclusion criteria comprised no confirmed follow-up or incomplete clinical entry data. RESULTS: Among 644 UM patients registered in the database up to 2017, the additional malignancy was diagnosed in 126 (20%) patients: 71 men, 55 women at the median age of 67 years (range: 34-94). In 48 patients (38%), the additional malignancy occurred prior to the diagnosis of UM, in 73 (58%) patients - after it. The most common locations of second cancer were skin (20 cases / 15%), breast (17 cases / 13%) and lungs (15 cases / 12%). The median follow-up was 36 months (range: 3-242). 87 patients (69%) died by the study close, 32 (37%) of them due to metastatic disease from uveal melanoma, 41 (47%) due to another cancer. CONCLUSIONS: The frequency of additional primary malignancies was higher in our cohort than reported by most of other groups. If there is a certain predisposition to a specific type of additional primary carcinoma in UM patients, the analysis of larger database is required.

Uveal and conjunctival melanomas: disease course and outcomes in Chinese patients.

INTRODUCTION: Epidemiological studies of ocular melanomas have largely focused on Caucasian populations. This study reviewed the course and outcomes of uveal melanoma (UM) and conjunctival melanoma (CM) in Chinese patients. METHODS: This retrospective study included patients with UM and CM who received treatment in a tertiary eye centre in Hong Kong from January 1994 to December 2019. Data were recorded concerning patient demographics, tumour laterality, tumour characteristics, investigations performed, treatment regimen, and final outcomes. RESULTS: During the 25-year study period, there were 13 patients with UM and 11 patients with CM who did not display nodal or systemic involvement at diagnosis. The mean ± standard deviation ages at diagnosis of UM and CM were 59 ± 15.8 and 57 ± 13.9 years, respectively. There were more men among patients with UM than among those with CM (P=0.042). Most patients with UM underwent primary enucleation (n=12; 92.3%), whereas most patients with CM underwent orbital exenteration (n=9; 81.8%). The prognosis was significantly worse for CM than for UM. The median disease-free survival were 5.2 years (range, 0.7-20.5) and 2.1 years (range, 0.1-24.9) for UM and CM, respectively. Melanoma-related mortality was significantly higher among patients with CM than among those with UM (P=0.006). CONCLUSION: Compared with UM, CM has higher rates of systemic metastasis and tumour-related mortality in Hong Kong Chinese patients, regardless of prior definitive treatment.

Clustering of uveal melanoma: County wide analysis within Ohio.

PURPOSE: To determine if a greater than expected number of cases (clustering) of uveal melanoma occurred within Ohio for any specific region or time period as compared to others. DESIGN: Analysis of population database. METHODS: Ohio Cancer Incidence Surveillance System (OCISS) database (2000-2019) was accessed for the diagnosis of uveal melanoma using the International Classification of Disease for Oncology codes: C69.3 (choroid), C69.4 (ciliary body and iris). Counties within Ohio were grouped by geographic regions (7) and socioeconomic variables. Age- and race-standardized incidence ratios (SIR) were calculated to determine temporal or geographic clustering. RESULTS: Over the twenty-year period, the total number of uveal melanoma cases reported within Ohio were 1,617 with the overall age-adjusted annual incidence of 6.72 cases per million population (95% CI 6.30-7.16). There was an increase in the incidence of uveal melanoma over 20 years (p<0.001) across seven geographic regions, but no significant difference in incidence rates between the regions. There was no difference in incidence based on county classification by age composition (p = 0.14) or education level (p = 0.11). Counties with a low median household income (p<0.001), those classified as urban (p = 0.004), and those with a greater minority population (p = 0.004) had lower incidence. Less populated counties had a higher incidence of uveal melanoma (p<0.001). CONCLUSIONS: There is no evidence of geographic or temporal clustering of uveal melanoma within Ohio from 2000 to 2019.

Impact of COVID-19 on a rare disease (uveal melanoma) in a national reference unit of intraocular tumors in Spain.

OBJECTIVE: To analyse the impact of the COVID-19 pandemic on the diagnosis and management of uveal melanoma (a tumour included in the Orphanet catalogue of rare diseases) in a Spanish national reference unit for intraocular tumours during the first year of the pandemic. METHOD: An observational retrospective study of patients with uveal melanoma in the National Reference Unit for Adult Intraocular Tumors of the Hospital Clínico Universitario de Valladolid (Spain) was performed, analysing the pre- and post-COVID-19 periods: from March 15, 2019 to March 15, 2020 and from March 16, 2020 to March 16, 2021. Demographic data, diagnostic delay, tumour size, extraocular extension, treatment and evolution were collected. A multivariable logistic regression model was used to identify factors that were associated with the variable: enucleation. RESULTS: Eighty-two patients with uveal melanoma were included, of which 42 (51.21%) belonged to the pre-COVID-19 period and 40(40.78%) to the post-COVID-19 period. An increase in tumour size at diagnosis and in the number of enucleations was observed during the post-COVID-19 period (p < 0.05). Multivariable logistic regression demonstrated that both medium-large tumour size and patients diagnosed in the post-COVID-19 period were independently related to an increased risk of enucleation (OR 250, 95%CI, 27.69-2256.37; p < 0.01 and OR 10; 95%CI, 1.10-90.25; p = 0.04, respectively). CONCLUSIONS: The increase in tumour size observed in uveal melanomas diagnosed during the first year of the COVID-19 pandemic may have favored the increase in the number of enucleations performed during that period.

Genetic Status Affects Disease-Specific Mortality But Not the Incidence of Local Recurrence in Patients with Uveal Melanoma.

PURPOSE: Increased disease-specific mortality has been observed among patients with local recurrence (LR) from uveal melanoma (UM), but the underlying mechanism is unknown. The purpose of this study was to determine if copy number alterations of chromosomes 3 and/or 8q, at the time of diagnosis, increase the incidence of LR and if disease-specific mortality among patients with LR depends on the chromosome status of the primary tumor. DESIGN: Retrospective cohort study. PARTICIPANTS: The study included 239 consecutive patients with primary UM (choroidal or ciliary body) treated with Ruthenium-106 (Ru-106) brachytherapy from January 2009 to December 2019 at a single national referral center. METHODS: Cox regression modeling and Kaplan-Meier analyses were used to assess the effect of the status of chromosomes 3 and 8q on the incidence of LR and disease-specific mortality after the event of LR. Multistate models were used to illustrate the probabilities over time of patients being alive and disease-free, alive with LR, dead from UM metastases, or dead from other causes split on the status of chromosomes 3 and 8q. MAIN OUTCOME MEASURES: Incidence of LR and disease-specific mortality. RESULTS: Local recurrence was observed in 42 patients (16%). Overall incidence of LR was not affected by aberrations of chromosomes 3 and/or 8q (P = 0.87). Although LR occurred earlier in patients with aberrations of chromosomes 3 and/or 8q compared with patients with a normal copy number of chromosomes 3 and 8q, the median time from primary diagnosis to LR was 1.6 years (interquartile range [IQR], 1.0-2.0) and 3.2 years (IQR, 2.1-5.0), respectively. Cox regression found LR to be an independent risk factor for disease-specific mortality (hazard ratio [HR], 2.7; 95% confidence interval [CI], 1.5-5.0) among all patients, but multistate models demonstrated a low risk of disease-specific death among patients with normal chromosomes 3 and 8q status, even after an LR. CONCLUSIONS: Copy number alterations of chromosome 3 and/or 8q in the primary UM did not increase the overall incidence of LR. However, the development of an LR enhanced the risk of disease-specific mortality among patients with copy number alterations of chromosomes 3 and/or 8q. Even after an LR, disease-specific mortality remained low among patients with normal copy numbers of chromosomes 3 and 8q. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Retinoblastoma and uveal melanoma in Jordan: incidence, demographics, and survival (2011-2020).

PURPOSE: We present an epidemiologic analysis of retinoblastoma (RB) and uveal melanoma (UM) in Jordan to aid national strategies for improved ocular cancer surveillance and control. METHODS: A retrospective cohort of all Jordanian patients with RB and UM diagnosed over 10 years (2011-2020). Outcome measures included incidence, demographics, and outcomes. RESULTS: Retinoblastoma (n = 124) was more common than UM (n = 82); there was no sex predilection for either group. The median age at diagnosis of RB was 15 months(Six and 28 months for bilateral and unilateral cases, respectively), and the mean age-adjusted incidence was 8.2 cases per-million-children per year for children aged five years or less(one per 15,620 newborn per year). Fifty-one(41%) had bilateral disease, and 18(15%) had familial disease. Ninety-six(55%) eyes were group D or E(78% were T3/T4), and the five-year survival rate was 96%.For UM, the median age at diagnosis was 45 years with an incidence of 1.39 new cases per year per one million population. All(100%) had nonfamilial unilateral disease. Seventy-three(89%) had the tumor in the choroid, and 48(58%) had an advanced tumor that had invaded the sclera or the orbit (T3/T4) tumor. Sixty-two(76%) were treated by I-125 radioactive plaque, with globe salvage in 59(95%); the five-year survival rate was 85%. CONCLUSION: In Jordan, RB is more common and has better survival than UM. RB in Jordan and Western countries is equal in terms of incidence, globe salvage, and survival. UM is less common, with lower age at diagnosis (that was associated with better survival) in Jordan than in Western countries.

Incidence and Risk Factors for Secondary Glaucoma in Eyes with Uveal Melanoma.

PURPOSE: To estimate incidence of and analyze risk factors for developing secondary glaucoma in eyes with uveal melanoma before and after diagnosis. DESIGN: A cross-sectional, population-based cohort study. PARTICIPANTS: Seven hundred eighty-one patients (median age, 64 years; range, 14-93) consecutively diagnosed with uveal melanoma from 1997 to 2012 in a national ocular oncology service, 708 (91%) of whom received ruthenium (50%) or iodine (50%) brachytherapy. METHODS: Patient, tumor, treatment, and follow-up data were collected prospectively. Frequency and associations of melanoma-related glaucoma at tumor diagnosis were assessed. Incidence of developing secondary glaucoma after diagnosis was estimated by Kaplan-Meier analysis. Independent risk factors were modeled using Cox regression. MAIN OUTCOME MEASURES: Melanoma-related glaucoma and related risk factors. RESULTS: Forty-five patients (5.8%; 95% confidence interval [CI], 4.2-7.6) had tumor-related secondary glaucoma at diagnosis, 34 (76%) from a narrow-to-closed angle (25 had direct angle invasion) and 10 (22%) from anterior neovascularization. Synchronous metastases were common in patients with initial secondary glaucoma (11% vs. 1.2% with incident glaucoma, P = 0.005). Patients with secondary glaucoma were often male (58% vs. 48% without glaucoma; P = 0.010) and had larger tumors (median thickness, 9.1 vs. 4.0 mm; P < 0.001) involving the ciliary body (43% vs. 21%; P < 0.001) with retinal detachment (53% vs. 30%; P < 0.001). One hundred and sixty-eight patients 165 of which were treated with brachytherapy developed incident tumor- or treatment-related secondary glaucoma a median of 1.7 years (range, 0.1-13.6) after tumor diagnosis. Cumulative proportion of developing secondary glaucoma was 23% (95% CI, 20-27) at 5 years. The most common mechanism was neovascularization in 119 patients (71%; 95% CI, 63-78). By multivariable regression, initial retinal detachment 3 to 4 quadrants (hazard ratio [HR], 2.18; P < 0.001), initial intraocular pressure 17 mmHg or higher (HR, 1.64; P = 0.01), and tumor thickness predicted incident secondary glaucoma. CONCLUSIONS: Secondary glaucoma at initial uveal melanoma diagnosis predicts high risk of synchronous metastases. Although anterior neovascularization is the most common mechanism for secondary glaucoma after diagnosis, other mechanisms such as angle narrowing and anterior chamber hemorrhage are not infrequent. Initial retinal detachment and intraocular pressure with tumor thickness could inform interim assessments of intraocular pressure and neovascularization.

Global Incidence and Trend of Uveal Melanoma from 1943-2015: A Meta-Analysis.

BACKGROUND: Uveal melanoma (UM) is the most common primary intraocular malignancy in adults arising from uveal tissue melanocytes. Considering limited population based studies, we performed a meta-analysis of uveal melanoma incidence rate in worldwide. METHODS: For this meta-analysis, the electronic database of PubMed, Scopus, Embase and Google Scholar were utilized. Qualified Cohort studies and registry system databases were included in the study. Trend analysis and the estimation of incidences were reported. Thereafter subgroup analysis on gender and diagnostic tests were performed. Analyses were done using random effect models. RESULTS: A total of 22 publications were eligible to include in the meta-analysis. The incidence rates of uveal melanoma were 5.74 (95%CI: 4.37-7.11) and 7.30 (95%CI: 6.36-8.24) in North America and Europe respectively. The analysis showed a significant decreasing trend in North America. It was expected that the risk of UM decreased approximately 0.09 (SE=0.04) per million persons per year. No significant trend was detected in Europe. There was not any significant difference between the incidence rate in male and female (6.58, 95%CI: 5.42-7.75 vs. 5.44, 95%CI: 4.40-6.48). In addition, the reported incidence rates in different diagnostic approaches were similar (6.61, 95%CI: 5.63-7.58 in clinical vs. 6.67, 95%CI: 5.83-7.42 in histological). CONCLUSION: This analysis confirmed in recent decades, there has been a steady decline in the incidence in North America. However, in European countries where they still have the highest incidence worldwide, the trend was stable. It seems that international melanoma registration collaboration can conduct a unified, multicentral study to estimate the worldwide incidence of UM and impact of different factors on its rate.

Sunlamp use is a risk factor for uveal melanoma: a meta-analysis.

OBJECTIVE: Advancements in the treatment of uveal melanoma have not improved survival; therefore, identifying modifiable risk factors is critical to improving outcomes. This study aims to investigate the association between sunlamp use and the development of uveal melanoma. DESIGN: This study is designed as a meta-analysis. METHODS: Literature was searched and reviewed through the MEDLINE (with both OVID and PubMed), EMBASE, MD Consult, and Web of Science databases. These databases were searched from 1966 to 2019 using the following keywords to identify articles examining risk factors for uveal melanoma: ultraviolet, sun, sunlight, uveal melanoma, eye cancer, eye melanoma, nevus, and risk factor. All articles were evaluated for inclusion based on methodology and data reporting association between sunlamp use and uveal melanoma. The Meta-analysis of Observational Studies in Epidemiology guidelines and the Newcastle-Ottawa Scale were used to assess data quality and validity. A random effects model was employed. RESULTS: A total of 5 studies, enrolling a total of 1753 uveal melanoma cases and 3399 controls were included in this meta-analysis. The results of this study showed a positive association between sunlamp use and uveal melanoma (odds ratio = 2.15; 95% confidence interval 1.27-3.64). Meta-regression of between study heterogeneity did not reveal a statistically significant association when publication year, site latitude, melanoma tissue location (specifically, inclusion of iris tumors), or control type (population versus clinic) were evaluated. CONCLUSION: This meta-analysis identified a statistically significant association between sunlamp use and uveal melanoma, supporting sunlamp use as a modifiable risk factor for uveal melanoma.

Phantom vision after eye removal: prevalence, features and related risk factors.

AIM: Phantom eye syndrome is a poorly understood and underestimated complication of eye removal (ER). Seeing with the amputated eye, referred to as phantom vision (PV), is undoubtedly the most intriguing and confusing complication experienced by anophthalmic patients. The aim of the study was to assess PV prevalence, clinical features and risk factors after ER. METHODS: A multicentric questionnaire-based study was conducted between April 2016 and July 2017. Patients >18 years who underwent ER >3 months ago had a socket examination before inclusion. Data recorded included patients' demographics, and preoperative, surgical and postoperative features. RESULTS: One hundred patients (53 men) with a mean age of 65.1 years (29-92; SD=13.0) were included. ER indications were: uveal melanoma (n=24, 24%), trauma (n=20, 20%), retinal detachment (n=20, 20%), glaucoma (n=14, 14%) and endophthalmitis (n=12, 12%). Thirty (30%) patients experienced PV. Elementary and complex visual hallucinations were experienced by 80% and 20% of patients, respectively. PV usually appeared within the first postoperative month and tended to decrease over time. Risk factors for PV were the preoperative use of proton beam therapy (p=0.006), uveal melanoma (p=0.014), enucleation (p=0.015), anxiety with a Hospital and Anxiety Depression (HAD) score ≥8 (p=0.042), depression with a HAD score ≥8 (p=0.030), phantom eye pain (p=0.044) and phantom eye sensations (p=0.002). CONCLUSION: PV was reported by one-third of our patients. Despite being widely misunderstood, ophthalmologists and neurologists should be aware of this complication to adequately reassure patients.

The epidemiology of uveal melanoma in Germany: a nationwide report of incidence and survival between 2009 and 2015.

PURPOSE: To calculate the overall incidence of uveal melanoma in Germany and to compare incidences in different German states. In addition, we computed the overall and cancer-specific survival rates nationwide. METHODS: Incidence data for the period between 2009 and 2015, covering the entire German population, was collected through the German Center for Cancer Registry. ICD-O-3 topography codes C69.3-C69.4 and histology codes for melanoma subtypes were used to collect the incidence data. Confidence Intervals with a level of 95% (95% CI) were calculated for rates. Survival was calculated using the Kaplan-Meier. The log-rank test was used for survival comparisons. RESULTS: This study comprised 3654 patients with uveal melanomas, including 467 (12.8%) with iridial and ciliary body tumors. The overall age-standardized incidence rate (ASIR) was 6.41 person per million. Generally, the ASIR was higher in males than females (6.67 (95% CI 6.37-6.98) vs. 6.16 (95% CI 5.88-6.45 per million). Higher crude incidence rates were noted in the northeastern states (12.5 per million (95% CI 10.5-14.7) in Mecklenburg-Vorpommern) compared with the southwestern states (2.1 per million (95% CI 1.7-2.6) in Hessen). The 5-year overall survival stood at 47%, while the cancer-specific survival stood at 84%. Multivariate analysis showed that women, younger patients, and patients living in Berlin achieved significantly higher overall survival. CONCLUSION: Overall ASIR of uveal melanoma in Germany indicates that the disease is more common in males and that it follows the same geographical distribution previously noted in central European countries, with the highest incidence in northern parts of Germany.

Uveal Melanoma - Standardised Procedure in Diagnosis, Therapy and Surveillance. / Das uveale Melanom ­ standardisiertes Vorgehen in Diagnostik, Therapie und Nachsorge.

BACKGROUND: Uveal melanoma is a rare intraocular tumour, for which there is currently no national evidence-based guideline in Germany. The aim of this project was to provide a common standard operating procedure (SOP) for the diagnosis, treatment and follow-up care of uveal melanoma, within the network of German leading oncology centres funded by German Cancer Aid. The SOP was created as part of a moderated consensus process. RESULTS AND CONCLUSION: In a multistage process, a common SOP was developed for the diagnosis, therapy and follow-up of uveal melanoma, as based on current knowledge of the subject.