Atypical presentations of retinoblastoma.

PURPOSE: To study the atypical presentations of retinoblastoma in our institution.

METHOD: Retrospective, clinical study.

RESULTS

A total of 392 cases of retinoblastoma were reviewed; 72.2% of the patients had leukocoria, 13% had proptosis, 10% had strabismus, 1.5% were asymptomatic (detected on screening), and 3.3% had atypical presentations. Fourteen different atypical findings were observed including endophthalmitis (0.76%), secondary glaucoma (0.76%), uveitis (0.5%), corneal edema (0.5%), phthisis bulbi (0.5%), orbital cellulitis (0.5%), cataract (0.25%), pseudohypopyon (0.25%), iris nodules (0.25%), hyphema (0.25%), iris neovascularization (0.25%), microphthalmos (0.25%), exposure keratopathy (0.25%), and corneal blood staining (0.25%). All of the patients had Reese-Ellsworth grade V disease. On histopathology, invasion of neighboring structures was seen in 10 patients. All patients underwent enucleation with adjuvant radiotherapy, chemotherapy, or both.

CONCLUSIONS

Retinoblastoma can mimic any orbital or ocular pathology. Atypical presentations of retinoblastomas are usually associated with advanced disease. The possibility of ocular tumor should be entertained whenever there is an unusual presentation that is unresponsive to the usual therapy. Ultrasonography and computed tomography should be performed in all such patients, especially if the posterior segment is not visible.