Pseudoretinoblastoma: Spectrum of Pathology and Frequency in Different Age Groups. Analysis of 14 years of Experience

Abstract

Purpose. To evaluate the spectrum and the occurrence frequency of lesions simulating retinoblastoma in different age groups. Material and methods. A retrospective study of 608 patients (871 eyes) with suspected retinoblastoma was performed in the Ocular Oncology and Radiology Department of the Fyodorov Eye Microsurgery Federal State Institution in the period from 2007 to 2020. The mean patient age at presentation was 21 months (from 1 to 209 months). In 475 patients (78.1%) (715 eyes), the diagnosis of retinoblastoma was confirmed after a comprehensive assessment of the eye. 133 patients (156 eyes) (21.9%) had symptoms mimicking retinoblastoma-pseudoretinoblastomas. All patients with pseudoretinoblastomas were divided into different groups based on the simulating condition and age: from 0 to 1 year, from 1 to 2 years, from 2 to 5 years, older than 5 years. Results. There were 25 conditions simulating retinoblastoma. The most common conditions were Coats disease (n=17; 12.8%), combined hamartoma of retina and retinal pigment epithelium (n=12. 9%), retinal detachment (n=11; 8.3%), vitreous opacities caused by intrauterine uveitis (n=11; 8.3%), choroidal hemangioma (n=11; 8.3%), vasoproliferative tumor (n=10; 7.5%), retinopathy of prematurity (n=7; 5.3%), morning glory syndrome (n=7; 5.3%). Most patients had unilateral lesions (n=116; 87.2%). Pseudoretinoblastomas differed based on age at presentation, because children 1 year of age or younger were most likely to have vitreous opacities caused by intrauterine uveitis (n=9; 21.4%) and retinopathy of prematurity (n=7; 16.7%); children aged from 1 to 2 years – Coats disease (n=4; 20%) and choroidal hemangioma (n=3; 15%), from 2 to 5 years – Coats disease (n=8; 21.6%), older than 5 years – Coats disease (n=5; 14.7%) and vasoproliferative tumors (n=7; 20.6%). Conclusion. Timely referral of children with suspected retinoblastoma in specialized ocular oncology centers helps to avoid unnecessary treatment for retinoblastoma, enucleation and improve the vital prognosis.