Global Retinoblastoma Study Group IMPORTANCE Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale.OBJECTIVES To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. DESIGN, SETTING, AND PARTICIPANTSA total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. MAIN OUTCOMES AND MEASURESAge at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. RESULTSThe cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low-and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI,, and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI,). CONCLUSIONS AND RELEVANCEThis study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs.
ABSTRACT. Purpose: To evaluate intraobserver and interobserver reproducibility of measurements in images obtained by the 50 MHz HumphreyA Ultrasound Biomicroscope. Methods: The first stored image of central cornea, central anterior chamber, and angle structures obtained at 50 examinations were measured twice by three observers. Nine different parameters were measured in angle images and two in images of central cornea and anterior chamber. Intraobserver reproducibility was assessed by calculating the coefficient of variation and interobserver reproducibility by a two-ways ANOVA. Results: Intraobserver reproducibility was high for all measurements of central cornea thickness and anterior chamber depth with a coefficient of variation (%) AE3.8%. In angle images the intraobserver reproducibility of measurements was often lower (coefficient of variation (%) 1.3-52.6%). The lower reproducibility was most noticeable in measurements involving the less defined structures. Interobserver reproducibility was poor. Conclusions: Comparison of measurements are best done of well defined structures and by only one observer.Key words: anterior eye segment ultrasonography -observer variation -reproducibility of results -microscopy -ultrasonography instrumentation.
Low-to-negative HSP-27 protein expression in uveal melanoma correlates strongly with monosomy 3. Further validation is necessary to determine whether immunohistochemical assessment of HSP-27 expression correlates with metastatic mortality.
ABSTRACT.Purpose: To evaluate the precision of anterior chamber dimensions measured by the 50 MHz Humphrey A Ultrasound Biomicroscope, the influence of various subprocedures, and the importance of clear definition of structures. Methods: Fourty-eight right eyes of 26 normal subjects and 22 glaucoma patients were scanned centrally and at the chamber angle at three positions under standardized conditions. Eight different parameters were measured in angle images and two in images of central cornea and anterior chamber. Four variance components were calculated. Results: Measurement of the clearest defined structures had the highest precision. In angle images the precision expressed as the coefficient of variation was ∞21%, except for measurement of iris-ciliary process (range: 3-59%). All subprocedures contributed to the overall variance, image to image variation the most. Conclusions: The precision of most measured parameters seems reasonable for clinical use. All subprocedures contributed significantly to the overall variance. The parameters that were related to clearly defined structures had the highest precision.
Key Points Question Are heritability and treatment associated with the incidence of second primary cancer in Danish retinoblastoma survivors? Findings In this national cohort study of 323 patients in Denmark diagnosed with retinoblastoma, the incidence and mortality of second primary cancer were significantly higher in patients with heritable retinoblastoma vs patients with nonheritable retinoblastoma. The data did not show an increased risk in patients with heritable disease who were treated with external radiotherapy. Meaning The findings of this study suggest that patients with a genetic predisposition to retinoblastoma may be at greater greatest risk for developing second primary cancer later in life.
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