Bilateral enucleation for retinoblastoma: A study of 14 patients

Abstract

Purpose: To study the clinical profile of patients who underwent bilateral enucleation for retinoblastoma (RB).

Methods: Retrospective study of 14 cases.

Results: Of >3000 RB cases, 14 (<1%) underwent bilateral enucleation for treatment of RB. The mean age at diagnosis of RB was 26 months (median, 24 months; range, 5-72 months). All patients had bilateral RB at presentation. Intraocular RB was evident in 23 (82%) eyes and orbital tumor extension was noted in 5 (18%) eyes. Based on the International Classification of Intraocular RB, tumors were classified as Group B (n = 1; 4%), D (n = 4; 14%), or E (n = 14; 50%) at presentation. Based on the International RB Staging System, tumors were classified as Stage 1 (n = 23; 82%) or Stage 3 (n = 5; 18%). Two patients (four eyes with intraocular RB) had undergone prior treatment before presenting to us and thus could not be classified. Primary treatment included systemic chemotherapy (n = 27; 96%) or enucleation (n = 1; 4%). Five patients were lost to follow-up for a mean duration of 15 months (median, 12 months; range, 7-24 months) during treatment and presented with the orbital extension of RB in one (n = 4; 29%) or both (n = 1; 7%) eyes. Secondary enucleation was performed in 27 (96%) eyes. Over a mean follow-up period of 49 months (median, 29 months; range, 3-340 months), there was no evidence of metastasis and 1 (7%) child died due to pneumonia.

Conclusion: Bilateral enucleation is rare in the treatment of RB. Advanced tumor presentation or noncompliance to treatment necessitates bilateral enucleation.

Keywords: Cancer; chemotherapy; enucleation; external-beam radiotherapy; eye; retinoblastoma.