Neonatal retinoblastoma: A study of five cases

Abstract

PURPOSE:The purpose was to study the clinical features, treatment, and outcome of retinoblastoma (RB) in neonates.METHODS:This was a retrospective study of five patients with RB detected in the 1st month of life.RESULTS:The mean age at diagnosis of RB was 22 days (median, 24 days; range, 14–28 days). There were one female and four males with neonatal RB. Two patients had a known family history of RB, and the tumor was detected by routine fundus screening in these children. Two patients were brought in with complaints of leukocoria and one patient with complaints of red-eye. All patients had an intraocular tumor at presentation. Four patients had bilateral involvement, whereas only one had unilateral involvement. Based on the International Classification of Intraocular Retinoblastoma, the tumors were classified as Group A (n = 2), Group B (n = 3), Group C (n = 1), Group D (n = 1), and Group E (n = 2). Macular involvement was noted in 6 (67%) eyes. The primary treatment included systemic chemotherapy with/without focal treatment in all patients. One patient subsequently underwent secondary enucleation as the globe became phthisical. One child died while on treatment due to pneumonia secondary to chemotherapy-induced neutropenia. Of the four patients who completed treatment, globe salvage was achieved in 6 (86%) eyes over a mean follow-up period of 89 months (median, 92 months; range, 29–144 months).CONCLUSION:Neonatal RB though rare, if detected early, has a favorable outcome of ocular and life salvage. Sporadic RB can occur in neonates, and a family history may not always be elicited.