Retinoblastoma: The Accuracy of Clinical Diagnosis in Children Treated by Enucleation

We correlated the pathologic diagnosis with the preoperative clinical diagnosis of retinoblastoma in children treated by enucleation within the United States and Canada between the years 1974 and 1980. In order to avoid inappropriate selectivity and institutional bias, we studied only those cases submitted directly to the Registry of Ophthalmic Pathology in which the enucleated eyes had not been examined initially at the local hospital. Of the 56 eyes removed because of suspected retinoblastoma, 15 (26.8%) did not contain a malignant tumor. In two cases enucleation was delayed because retinoblastoma was not considered initially in the differential diagnosis of spontaneous hyphema. During the same period, two of 268 eyes that were enucleated for reasons other than suspected retinoblastoma were found to contain a retinoblastoma. The diagnostic error rate in our study reflects the persistent difficulty in diagnosing retinoblastoma in eyes with opaque media and retinal detachment.