alexa Lesions simulating retinoblastoma (pseudoretinoblastoma) in 604 cases: results based on age at presentation.
Neurology

Neurology

International Journal of Neurorehabilitation

Author(s): Shields CL, Schoenberg E, Kocher K, Shukla SY, Kaliki S,

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Abstract PURPOSE: To determine the types and frequency of ocular conditions that simulate retinoblastoma (pseudoretinoblastoma) based on age at presentation. DESIGN: Retrospective case series. PARTICIPANTS: Two thousand seven hundred seventy-five patients. METHODS: Chart review. MAIN OUTCOME MEASURES: Conditions simulating retinoblastoma. RESULTS: Of 2775 patients referred for management of retinoblastoma, 2171 patients (78\%) had confirmed retinoblastoma and 604 patients (22\%) had simulating lesions (pseudoretinoblastomas). In the pseudoretinoblastoma cohort, the mean patient age at presentation was 4 years (median, 2 years). There were 27 different pseudoretinoblastoma conditions, and the 10 most common included Coats' disease (n = 244; 40\%), persistent fetal vasculature (PFV; n = 158; 28\%), vitreous hemorrhage (n = 27; 5\%), ocular toxocariasis (n = 22; 4\%), familial exudative vitreoretinopathy (FEVR; n = 18; 3\%), rhegmatogenous retinal detachment (n = 18; 3\%), coloboma (n = 17; 3\%), astrocytic hamartoma (n = 15; 2\%), combined hamartoma of retina and retinal pigment epithelium (n = 15; 2\%), and endogenous endophthalmitis (n = 10; 2\%). Simulating lesions differed based on age at presentation, and children younger than 1 year were most likely to have PFV (49\%), Coats' disease (20\%), or vitreous hemorrhage (7\%); those 2 to 5 years of age were most likely to have Coats' disease (61\%), toxocariasis (8\%), or PFV (7\%); and those older than 5 years were most likely to have Coats' disease (57\%), toxocariasis (8\%), or FEVR (6\%). CONCLUSIONS: The most common pseudoretinoblastomas include Coats' disease, PFV, and vitreous hemorrhage, but the spectrum varies depending on patient age. Copyright © 2013 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved. This article was published in Ophthalmology and referenced in International Journal of Neurorehabilitation

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