An unusual case of retinoblastoma

The authors describe the treatment and complications of a case of retinoblastoma with an atypical presentation. A 20-month-old girl was diagnosed as having unilateral retinoblastoma by cytopathology during pars plana vitrectomy and the right eye was enucleated. Following the enucleation, computed tomography detected a questionable focus of retinoblastoma in the residual optic nerve. Exploration of the orbit and sampling of the optic nerve stump had to be performed because the enucleated globe was lost. Histopathology of the optic nerve stump showed granulation tissue and fibrosis but no evidence of retinoblastoma. Histopathology of the enucleated globe and transected optic nerve is the gold standard for staging retinoblastoma and formulating a treatment plan. Treatment of the patient with retinoblastoma becomes more complex when histopathologic findings are not available.