Globe-sparing interventions in the management of intraocular retinoblastoma

ABSTRACT

RETINOBLASTOMA is the most common malignant intraocular tumor in children; it is also one of the most highly curable pediatric solid tumors if detected early. The conventional treatment of retinoblastoma is primary enucleation. Recent research reported a trend toward decreasing frequency of enucleation in the management of retinoblastoma. The trend toward globe-sparing interventions has been largely attributed to earlier diagnosis and recent success with conservative globe-sparing treatment options. Currently, globe-sparing interventions include first-line chemotherapy or chemoreduction, subconjunctival chemotherapy, systemic chemotherapy for metastasis, transpupillary thermotherapy (TIT), chemothermotherapy (CTT), laser photocoagulation, cryotherapy, brachytherapy, and external beam radiotherapy (EBRT). Expanded clinical options currently available have markedly decreased the overall enucleation rate for retinoblastoma CLINICAL SCENARIO A 10-month old boy is brought to an ophthalmologist because of cats eye reflex in the left eye. The patient had undergone enucleation of his right eye for glaucomatous stage retinoblastoma 6 months earlier. Examination revealed the presence of a solitary retinal mass of about 12 mm in diameter, located nasal to the disc. There was no evidence of vitreous seeding Realizing that this was the only eye of the patient, the ophthalmologist wants to do everything humanly possible to preserve it. He has heard about chemothermotherapy (CTT) but is not sure if this was the best alternative he can offer CLINICAL QUESTION Among patients with retinoblastoma, is chemoreduction combined with adjuvant treatment effective in preserving the globe and vision? (Author)