ABSTRACT
PURPOSE: To report the relative frequency of presenting signs in Tunisian children with retinoblastoma and to evaluate their prognostic impact. METHODS: A retrospective study was undertaken of 200 patients with retinoblastoma examined and treated between January 1993 and June 2009. RESULTS: There were 123 boys and 77 girls. For all cases, mean age at diagnosis was 29.8 months (range, 1 month to 9 years). There were 138 (69%) unilateral cases and 62 (31%) bilateral cases. Nineteen children (9.5%) had a family history of retinoblastoma. The most common signs were leukocoria (80%) and strabismus (28%) followed by proptosis. Orbital inflammation, hyphemia and phthisis are rare presenting findings in retinoblastoma. In our series, 26 children (13%) presented with extraocular retinoblastoma. Leukocoria and proptosis are significantly associated with a worse prognosis. CONCLUSION: The ability to recognize the presenting signs and symptoms of retinoblastoma can lead to earlier diagnosis and better prognosis.
