Background. To determine clinical profile and management of retinoblastoma among children at Kinshasa in Democratic Republic of Congo. Patients and methods. The medical records of patients with a diagnosis of retinoblastoma seen at the University Hospital of Kinshasa from January 1985 till December 2005 were retrospectively reviewed. Demographic profile, clinical data, modes of treatment and outcome were analysed. Results. A total of 49 children, of whom 40 had adequate data on record were identified as retinoblastoma (28 males and 12 females). Nine cases had bilateral disease. The median age at the first symptoms was 9 months (range, 1 month to 6 years) for unilateral retinoblastoma and 18 months (range, 1 month to 3.5 years) for bilateral retinoblastoma. The median age at the first oncology consultation was 2.4 years (range, 6 months to 6 years) for unilateral retinoblastoma and 2.4years (range, 9 months to 4 years) for bilateral disease. Leukokoria was present in 67.5% of subjects. Seventy-five percent abandoned the treatment. The mortality was 92.5%. Conclusion. In Democratic Republic of Congo, retinoblastoma remains a life threatening disease characterized by late referral to a specialized unit and affordability of chemotherapy; all leading to an extension of the disease and high mortality. 1. Introduction Retinoblastoma is the most frequent childhood intraocular tumour with an approximately incidence of one in 15,000–20,000 births in the world [1]. In Africa, it is the most important life-threatening ocular malignancy [2–4]. Management of retinoblastoma has been changing during years with a survival rate of more than 95% in developed countries [5, 6]. However, in developing countries these cures are less than 50%, primarily because of advanced disease at time of diagnosis [7–9]. Previous studies had reported epidemiological and clinical characteristics of the disease [10–12]. In the Democratic Republic of Congo (DRC), retinoblastoma represents 5/188 of malignant tumors in children [13], 8% of exophthalmos [14]. In other previous study, retinoblastoma was the most common histologic form representing 31.7% of all malignant of the eye [15]. However, there is still a paucity of information on clinical findings and on the outcomes of children with retinoblastoma. The last paper on presenting signs of retinoblastoma in Congolese patients gave a five-year ophthalmology view [16]. At the other side, it is necessary for health plan to have the main characteristics of all children with retinoblastoma followed in Democratic Republic of Congo. This
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