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.

Single nucleotide polymorphisms (SNPs) of p53 rs1042522, MDM2 rs2279744 and p21 rs1801270, all in the p53 pathway, which plays a crucial role in DNA damage and genomic instability, were reported to be associated with cancer risk and pathologic characteristics. This case-control study was designed to analyse the association between these SNPs and retinoblastoma (RB) in a Chinese Han population. These SNPs in 168 RB patients and 185 adult controls were genotyped using genomic DNA from venous blood. No significant difference was observed in allele or genotypic frequencies of these SNPs between Chinese RB patients and controls (all P > 0.05). However, the rs1042522 GC genotype showed a protective effect against RB invasion, as demonstrated by event-free survival (HR = 0.53, P = 0.007 for GC versus GG/CC). This effect was significant for patients with a lag time >1 month and no pre-enucleation treatment (P = 0.007 and P = 0.010, respectively), indicating an interaction between p53 rs1042522 and clinical characteristics, including lag time and pre-enucleation treatment status. Thus, the rs1042522 SNP may be associated with RB invasion in the Han Chinese population; however, further large and functional studies are needed to assess the validity of this association.

IntroductionRetinoblastoma (Rb) is the most common intraocular malignant tumor in children, accounting for approximately 3%-4% of all malignant tumors in children aged 0-14 years (1,2). Currently, in developed countries with advanced tools for medical diagnosis, Rb can be detected early, thus leading to an improved survival rate of more than 95% for this high-mortality disease. However, the survival rate from this disease is still as low as approximately 50% worldwide (3). This may be due to the poor diagnostic and therapeutic capacity, as well as generally poor health conditions, experienced in low-income countries. This leads to late diagnosis of the disease and thus a high incidence of extraocular complications and a high risk of distant metastasis (4). Rb can be cured if the disease is diagnosed early, if disease severity is appropriately assessed, and if proper treatments are applied (5). Therefore, this study aimed to identify clinical characteristics of Rb patients, to find the potential relationship between clinical manifestations and survival rates from Rb, and to provide a clinical basis for prediction of disease outcomes and treatment options. Materials and methods Clinical dataThis retrospective study with complete follow-up data included 314 patients who were diagnosed as having Rb at the Zhongshan Ophthalmic Center at Sun Yat-sen University from January 2003 to February 2011. Clinical data, including laterality of eyes, sex, age at diagnosis, presenting signs, lag time before treatment, survival conditions, and survival time, were recorded. Patients were followed until death or the cutoff date (February 2013), whichever occurred first. The mean follow-up time was 41.4 ± 27.9 months (median, 33 months; range, 1-110 months).Patients' clinical presentations at diagnosis were categorized into 6 groups based on the description given by the patients or their families: 1) leukocoria (white reflection from the pupil); 2) strabismus; 3) inflammation Background/aim: The aim of this study was to investigate the potential correlation between clinical characteristics and prognosis of patients with retinoblastoma (Rb). Materials and methods:This retrospective study included 314 Rb patients. Clinical data including laterality of eyes, sex, age, presenting signs, lag time, and survival were recorded and analyzed.Results: Leukocoria is the most common clinical presentation of Rb. Patients with isolated leukocoria had shorter lag time and exhibited a high survival rate (85%, 5 years). Patients with strabismus and blurred vision, and who were older and had longer lag time, exhibited an excellent survival rate (100% and 92.3%, respectively, 5 years). Patients with exophthalmos had the longest lag time and the lowest survival rate (17.8%, 5 years). The 5-year survival rate of patients with a lag time of ≤6 months was 84.7%, which was significantly higher than that of patients with a lag time of >6 months (64.7%). Conclusion:Leukocoria, strabismus, and blurred vision are mild clinical manifestations of Rb that are associ...

Purpose: This study aimed to develop an effective nomogram for predicting survival in surgically treated non-small cell lung cancer patients.Methods: We retrospectively evaluated 856 NSCLC in this study. Cox regression analyses were performed to identify significant prognostic factors for developing a nomogram to predict overall survival (OS). The discriminative ability was assessed with the concordance index (C-index).Results: On multivariate analysis of the 856 cohort, independent factors for survival were CRP, fibrinogen, tumor status, nodal status, distant metastasis and clinical stage, which were entered into the nomogram. The C-index of the established nomogram 0.720 (95% CI: 0.671-0.769) was higher than that of the seventh edition TNM staging system 0.689 (95% CI: 0.668-0.709) for predicting OS (P < 0.05). Compared with patients with low CRP levels (< 8.6 g/L) and low fibrinogen levels (< 3.7 g/L), patients with high CRP and fibrinogen levels had shorter OS. Subgroup analyses revealed that the nomogram was a favorable prognostic parameter in stage I-IV NSCLC (P < 0.05).Conclusion: A nomogram integrating CRP and fibrinogen, which could be convenient and feasible to obtain from the serum preoperatively, may assist in risk stratification for individual patient with resected NSCLC.