Late recurrence of tumor necessitating enucleation in an adult onset retinoblastoma

Abstract

Retinoblastoma is the most common primary intraocular malignancy in children. Although rare cases of adult onset retinoblastoma have been reported, the numbers are very few worldwide. We report a case where tumor recurrence following a quiescent period of 31/2 years led to enucleation of the eye in an adult onset retinoblastoma. A 33-year-old female was noted to have an intraocular mass lesion in the inferonasal quadrant of the retina. Computed Tomography, B-scan and vitreous biopsy confirmed the diagnosis of retinoblastoma. The patient underwent plaque brachytherapy, cryotherapy, and laser indirect ophthalmoscopy which resulted in complete regression of the tumor mass with no areas of activity. At the last follow up 31/2 years after the treatment the right eye showed anterior chamber infiltration with a tumor-like mass. Fundus examination showed vitreous hemorrhage, vitreous seedings and a small focus of tumor reactivation. B-scan, ultrasound biomicroscopy and MRI confirmed the mass lesion. In view of the tumor infiltrating the anterior chamber and involving the ciliary body prompt enucleation of the right eye was performed. The diagnosis of retinoblastoma was confirmed by histopathology. It is important that even after complete regression of the tumor frequent surveillance with fundus examination is necessary to detect early recurrences and to institute prompt management. © 2013 Informa Healthcare USA, Inc.