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

CHANTADA GL, GUITTER MR, FANDIÑO AC, RASLAWSKI EC, DE DAVILA MT, VAIANI E, SCOPINARO MJ.

MEDICAL AND PEDIATRIC ONCOLOGY

Año: 2009 vol. 52 p. 218 - 222

0098-1532

BACKGROUND: There is little information on the outcome of patients withretinoblastoma and tumor at the resection margin of the optic nerve. PROCEDURE:Retrospective evaluation of three successive prospective protocols. Twenty-sixconsecutive 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 hadneoadjuvant chemotherapy followed by enucleation and adjuvant therapy. Bothgroups received adjuvant chemotherapy and orbital radiotherapy after enucleation.Patients in protocol 1 received 1 year of the lower-dose chemotherapy regimenincluding cyclophosphamide, vincristine and doxorubicin along with intrathecalchemotherapy. Patients of protocols 2 and 3 received a more intense and shorterintravenous regimen including carboplatin and etoposide alternating withcyclophosphamide, idarubicin and vincristine with no intrathecal treatment. Thecomponents of protocol 2 and 3 were similar except for the dose of carboplatinwhich was 10% lower in protocol 3. RESULTS: Thirteen were treated in protocol 1and 13 in protocols 2 and 3. The probability of event-free survival was 0.70 at 5years. Events included: CNS relapse = 3, second malignancies = 3, death incomplete remission = 2. There were no significant differences in outcome between protocols or stages. Endocrinological disturbances related to thehypothalamus-hypophysis axis were evident in 6/8 patients evaluated. Severeorbital sequelae occurred in 12 cases. CONCLUSIONS: A substantial number ofpatients with tumor at the resection margin of the optic nerve can be cured with current therapy; however, therapy related sequelae are frequent. (c) 2008Wiley-Liss, Inc.