نتايج درمان و پيش آگهي در بيماران مبتلا به رتينوبلاستوما بعد از شيمي درماني اوليه و درمان كانوني بر اساس طبقه بندي بين المللي رتينوبلاستوما

Outcomes and Prognosis of Treatment for Retinoblastoma with Primary Chemotherapy and Focal Treatment

Purpose: To determine globe and patient survival in children with retinoblastoma at a major referral center in Iran. Methods: Hospital records of 156 eyes of 105 patients with retinoblastoma referred to the Ocular Oncology Center at Rasoul Akram Hospital, Tehran, Iran from 2001 to 2007 were reviewed. Demographic data, family history, presenting symptoms, duration of symptoms, ocular findings and treatment modalities were evaluated. Main outcome measures included patient survival with the event of death, and globe survival with the event of enucleation. Results: Mean age at the time of diagnosis was 28.5+23.0 (range 3-120) months. Mean follow-up was 30.9±29.0 (range 3-72) months. Male to female ratio was 47/58. Five patients had positive family history. Involvement was unilateral in 52% and bilateral in 48% of patients. Enucleation was performed as primary treatment in 76 eyes (48.7%) and as secondary treatment for recurrence in 11 eyes (7.1%). Sixty nine eyes (44.2%) were salvaged by different globe preserving modalities. Kaplan-Meier 5-year survival estimate for globe preservation according to the International Classification of Retinoblastoma (ICRB) was 100% for group A, 93.5% for group B, 86-7% for group C, 57.1% for group D and 0% for group E. Kaplan-Meier estimates for 5-year patient survival was 100% for ICRB groups A and B, 92.3% for group C, 73.3% for group D and 79.9% for group E. Five year patient survival rate was better in ICRB groups A to D than in group E (P=0.004). Conclusion: Advances in treatment, early diagnosis and prompt referral have improved the prognosis of patients with retinoblastoma in terms of globe and patient survival