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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. A retrospective cohort of all Jordanian patients with RB and UM diagnosed over 10 years (2011–2020). Outcome measures included incidence, demographics, and outcomes. 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%. 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. [ABSTRACT FROM AUTHOR]

Purpose: To study the role of external beam radiation therapy (EBRT) for the treatment of retinoblastoma eyes that were not cured by combined systemic chemotherapy and focal consolidation therapy. Methods and Materials: A retrospective case series of 28 eyes for 24 retinoblastoma patients treated by EBRT after the failure of tumor controlled by chemotherapy and focal therapy. The main outcome measures included: international intraocular retinoblastoma classification stage (IIRC) and Reese Ellsworth (RE) stage, tumor seeding, treatment modalities, eye salvage, and survival. Results: The median age at diagnosis was 11 months. There were 14 (58%) males and 20 (83%) bilateral cases. All eyes were treated initially by systemic chemotherapy (range; 6–8 cycles). The dose of radiation used for all eyes was 45 Gray (Gy). The mean follow-up was 75months, and the overall eye salvage rate after EBRT was 13 (46%) eyes: 67% (2/3) for IIRC group B, 71% (5/7) for group C, and 33% (6/18) for group D eyes. Vitreous seeds and tumor stage migration during management by chemotherapy were the most important significant predictive factors for tumor control (p =.001 and 0.033, respectively). Conclusion: Eyes with retinoblastoma that failed chemotherapy followed by focal therapy were controlled with EBRT. However, the presence of vitreous seeds, stage migration during the course of chemotherapy, as well as good vision in the other eye may not justify the known risks of EBRT. [ABSTRACT FROM AUTHOR]

Background: Early diagnosis and timely management of Retinoblastoma (RB) patients are essential to improve eye salvage and survival rates. The objective of this study is to evaluate the level of knowledge regarding retinoblastoma among first-contact physicians, namely students in last year of medical school, pediatricians, and ophthalmologists. Methods: A questionnaire about RB, preluded by a photograph of a child with leukocoria was completed by 138 medical students, 65 pediatricians, and 65 ophthalmologists. Descriptive statistics from the population were obtained and all answers were analyzed. Results: The majority (n = 253, 94%) of participants in the 3 groups recognized leukocoria as an abnormal sign. However, 62 (45%) medical students did not recognize it as a sign of a life-threatening disease. Only 3 (2%) medical students, 1(2%) pediatrician, and 9 (14%) ophthalmologists achieved a proficiency grade, compared to 136 (67%) medical students and pediatricians who failed to achieve the sufficiency score (70%) in this questionnaire. Ophthalmologists showed a better level of knowledge about RB than medical students and pediatricians, however, 27(42%) of them failed to achieve the sufficiency score. Of interest, only 72 (52%) of medical students, 41 (63%) of pediatricians, and 46 (71%) of ophthalmologists knew that RB needs urgent management. Conclusion: Most medical students and pediatricians involved in this study lack the sufficient knowledge to detect and timely-refer patients with signs of RB. It is necessary to develop continuous medical education programs for first-contact physicians. [ABSTRACT FROM AUTHOR]

Purpose: To determine ocular survival and factors affecting globe survival in patients diagnosed with retinoblastoma at King Hussein Cancer Center (KHCC). Methods: A retrospective review of medical records of 71 Jordanian patients (45 males and 26 females) diagnosed with retinoblastoma (114 eyes) between June 2003 and May 2013 was conducted. Patient sociodemographic and relevant characteristics were collected from records. Patients with bilateral retinoblastoma were treated with chemoreduction and focal consolidation. Lens-sparing radiation therapy and enucleation were reserved for eyes that failed chemoreduction combined with focal therapy. In cases of unilateral retinoblastoma, primary enucleation was recommended for eyes with advanced unilateral disease (Reese-Ellsworth classification groups IV and V). Kaplan-Meier survival and Cox regression multilevel analysis were used to analyze the data. Results: Median age at diagnosis was 12 months. The follow-up period ranged from 0.25-160 months (mean 26.9 months). The Kaplan-Meier estimate of globe survival of the 114 eyes was 68.0% at 1 year, 63.3% at 2 years, and 62.1% at 5 years. The mean survival time was 101.6 months (95% confidence interval, CI, 87.6-115.6 months). In multivariable-adjusted analysis, advanced stage of the disease (hazard ratio, HR, 5.1, 95% CI 2.3-11.6), unilateral disease (HR 3.3, 95% CI 1.4-8.1), and delay in diagnosis (HR 2.4, 95% CI 1.1-5.5) were significantly associated with increased hazard of enucleation. Conclusion: The overall ocular survival rate for eyes with retinoblastoma was close to regional and international figures. Disease stage, laterality, and delay in diagnosis were significant predictors of enucleation. [ABSTRACT FROM AUTHOR]