Extraocular retinoblastoma in Saudi arabia

Advanced retinoblastoma has a reported 95% mortality rate, usually within six months of disseminated disease. Analysis of 146 consecutive retinoblastoma patients from 1983-1989 revealed 58 (39.7% with extraocular disease. Of these, 26 (44.9% manifested frank orbital tumor. The ages of this select group ranged from three months to 9.6 years, with a mean of 2.7 years. A delay in presentation appeared to be a major factor in the orbital tumors. Treatment included a combination of external beam radiation therapy delivered in divided doses (250 rads three times weekly for four weeks) and systemic intravenous chemotherapy (consisting of Vincristine, Cytoxan, and Adriamycin) administered monthly over a one-year period. Out of 42 children who had at least one year follow-up after treatment, IS (36% were still surviving. Two years after completion of treatment, however, the survival statistics decreased to 13.8% (8/58) for all patients. This total includes a subset of 12/58 patients receiving palliative radiotherapy for advanced neoplasms, carrying 100% mortality. The overall data indicates that aggressive multimodality therapy can, in part, arrest progression of extraocular disease and thereby prevent the high attrition rate associated with metastatic disease.