Radiotherapy is effective in local treatment for retinoblastoma. However, asymmetric facial hypoplasia after radiation is a serious late effect. This study was performed to investigate the effects of enucleation and chemotherapy with or without radiotherapy in advanced intraocular and intraorbital retinoblastoma.

Between 1985 October and 2006 December, the records of thirty five patients who were diagnosed as retinoblastoma at Yeungnam University Hospital were reviewed. Advanced intraocular and intraorbital retinoblastoma patients classified as Reese-Ellsworth group III, IV, and V and Grabowski- Abramson class II were selected for the study.

Eighteen patients were enrolled in this study. All patients were enucleated and had received chemotherapy. Nine patients received radiotherapy and nine patients didn't receive radiotherapy. Tumor cells were found on resection margin of optic nerve in five of nine patients who received radiotherapy, but none of nine who didn't receive radiotherapy. Chemotherapy included vincristine, adriamycin, cyclophosphamide, VM-26, cisplatin before 2001, and vincristine, etoposide, and carboplatin after 2001. There were no recurrences or metastases in nine patients who didn't receive radiotherapy. But two of nine patients who received radiotherapy had metastases to brain. However, all survivors who received radiotherapy had significant facial asymmetry.

In advanced intraocular and intraorbital retinoblastoma without tumor cell on resection margin of optic nerve, enucleation and chemotherapy without local radiotherapy appears to be safe for long-term survival. However, in those with tumor cells on resection margin of optic nerve, enucleation and chemotherapy with local radiotherapy seems to be necessary to improve survival.