Treatment results in patients with retinoblastoma and invasion to the cut end of the optic nerve

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Abstract
Background There is little information on the outcome of patients with retinoblastoma and tumor at the resection margin of the optic nerve. Procedure Retrospective evaluation of three successive prospective protocols. Twenty‐six consecutive patients were analyzed (International Staging System—IRSS‐stage 2 = 21, stage 3 = 5) from three successive prospective protocols (1988–2006). Patients with stage 2 were enucleated upfront and those with stage 3 had neoadjuvant chemotherapy followed by enucleation and adjuvant therapy. Both groups received adjuvant chemotherapy and orbital radiotherapy after enucleation. Patients in protocol 1 received 1 year of the lower‐dose chemotherapy regimen including cyclophosphamide, vincristine and doxorubicin along with intrathecal chemotherapy. Patients of protocols 2 and 3 received a more intense and shorter intravenous regimen including carboplatin and etoposide alternating with cyclophosphamide, idarubicin and vincristine with no intrathecal treatment. The components of protocol 2 and 3 were similar except for the dose of carboplatin which was 10% lower in protocol 3. Results Thirteen were treated in protocol 1 and 13 in protocols 2 and 3. The probability of event‐free survival was 0.70 at 5 years. Events included: CNS relapse = 3, second malignancies = 3, death in complete remission = 2. There were no significant differences in outcome between protocols or stages. Endocrinological disturbances related to the hypothalamus–hypophysis axis were evident in 6/8 patients evaluated. Severe orbital sequelae occurred in 12 cases. Conclusions A substantial number of patients with tumor at the resection margin of the optic nerve can be cured with current therapy; however, therapy related sequelae are frequent. Pediatr Blood Cancer 2009;52:218–222.