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.

BackgroundThe travel distance from home to a treatment centre, which may impact the stage at diagnosis, has not been investigated for retinoblastoma, the most common childhood eye cancer. We aimed to investigate the travel burden and its impact on clinical presentation in a large sample of patients with retinoblastoma from Africa and Europe.MethodsA cross-sectional analysis including 518 treatment-naïve patients with retinoblastoma residing in 40 European countries and 1024 treatment-naïve patients with retinoblastoma residing in 43 African countries.ResultsCapture rate was 42.2% of expected patients from Africa and 108.8% from Europe. African patients were older (95% CI −12.4 to −5.4, p<0.001), had fewer cases of familial retinoblastoma (95% CI 2.0 to 5.3, p<0.001) and presented with more advanced disease (95% CI 6.0 to 9.8, p<0.001); 43.4% and 15.4% of Africans had extraocular retinoblastoma and distant metastasis at the time of diagnosis, respectively, compared to 2.9% and 1.0% of the Europeans. To reach a retinoblastoma centre, European patients travelled 421.8 km compared to Africans who travelled 185.7 km (p<0.001). On regression analysis, lower-national income level, African residence and older age (p<0.001), but not travel distance (p=0.19), were risk factors for advanced disease.ConclusionsFewer than half the expected number of patients with retinoblastoma presented to African referral centres in 2017, suggesting poor awareness or other barriers to access. Despite the relatively shorter distance travelled by African patients, they presented with later-stage disease. Health education about retinoblastoma is needed for carers and health workers in Africa in order to increase capture rate and promote early referral.

The primary orbital location of a malignant hemopathyin children is unusual.Our aim was to orient the ophthalmologist and the pediatrician to a rare etiology of pediatric exophthalmos.We report the observations of three patients hospitalized for exophthalmos with a normal initial blood count but in whom the myelogram made the diagnosis of acute myeloid leukemia or plasmacytoma. Conclusion:The first orbital involvement in acute leukemia is called granulocytic sarcoma or chloroma. This diagnosis should be considered in the event of any tumoral proptosis and the myelogram should be part of the initial assessment even if the blood count is normal, because early treatment improves the functional and vital prognosis.

Background and Objectives: Haematological malignancies account for approximately 40% of all cancers by the age of 15 years. Acute leukaemia (AL) account for one-third of childhood cancer cases; consisting of Acute Lymphoblastic Leukaemia (ALL) and Acute Myeloid Leukaemia (AML). The aim of this work is to describe the epidemiological, clinical, biological and evolutionary characteristics of children with acute leukaemia in the western and south-western region of Algeria. Patients and Methods: A three-year retrospective study was undergone from January 2016 to December 2018 on children with acute leukaemia. The study was conducted at the paediatric oncology department of the anti-cancer -Emir AEK- of Missreghine in Oran. Results: During this period, we identified 135 cases of diagnosed AL. The sex ratio M/F was 1.1. The “two to five-year” age group was the most affected. The prevalence of ALL, AML, and biphenotypic acute leukaemia (BAL) was 60.45%, 23.88%, and 15.57%, respectively. The clinical signs were mainly presented by the tumour syndrome dominated by the presence of lymphadenopathy (63%) and splenomegaly (56.3%). The most frequent abnormal blood abnormalities were anaemia (66.66% in ALL and 28.14% in AML), thrombocytopenia (75.9% in ALL and 24.4% in AML) and leukocytosis (76.3% ALL and 23.7% AML). Conclusion: Paediatric acute leukaemia is a real public health problem that requires special care and attention. This management must involve all epidemiological, clinical and biological aspects for this highly sensitive age group.