Survival in extra-orbital metastatic retinoblastoma: treatment results

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ABSTRACT

Introduction. Retinoblastoma (RB) is the most frequentmalignant eye tumor in childhood. In developingcountries advanced stages are common. The purposeof this paper is to present our 21-year clinicalexperience with metastatic extra ocular RB patientstreated with 5 different chemotherapy schemas at asingle Mexican Pediatric referral center.Materials and methods. A retrospective analysiswas carried out reviewing the clinical characteristicsof patients with metastatic RB. The information analyzedincluded the delay in diagnosis after firstsymptoms, age, sex, ocular staging, and anatomicsite of metastases, treatment scheme, initial responseand status at the last contact or date of death.Results. Eighty-one patients were included; agerange was from 3 to 80 months. The most commonsite of metastasis was central nervous system (83.9%).From those patients treated with chemotherapy(n = 74), 89.2% presented a complete initial response(n = 66). Early mortality occurred in 7 cases beforeany treatment. Fifty-six received treatment anddied with progressive disease. All patients withoutradiotherapy died with tumor activity (n = 15). Theuse of cisplatin was related with longer disease freeintervals; no other variable was related with survival.Four patients were alive and disease free at 33to 144.3 months of follow up from diagnosis. Theprevalent cause of death was tumor progression.Conclusions. In our experience, metastatic RB hasa very high mortality rate in spite of the use of differentchemotherapy regimens