Radiation Retinopathy and Benign Lymphoproliferative Orbital Tumor Presenting 47 Years Following Treatment of Hereditary Retinoblastoma.

Abstract

To better understand onset of radiation retinopathy and secondary orbital tumors in the setting of retinoblastoma treated with radiation and chemotherapy. Case report. Here we present a 48-year-old female with a history of bilateral hereditary retinoblastoma status-post enucleation of the left eye and radiation therapy to the right eye, along with systemic chemotherapy. She underwent bladder leiomyosarcoma resection at age 24. In 2020, she presented with significantly delayed radiation retinopathy complicated by cystoid macular edema, and bevacizumab injections were initiated. An incidental benign lymphoproliferative tumor in the right lacrimal gland was found on B-scan ultrasound and was successfully excised. It is rare for radiation retinopathy to present with significant delay after local radiation treatment, with only 2 other cases found in the literature describing a similar late onset. In addition, there have been no other published cases of a secondary benign lymphoproliferative tumor in the setting of retinoblastoma treated with radiation and chemotherapy.