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Maria Paula Fernandez, Shaza N Al-Holou, Oliver Gordon Fischer, Audina M Berrocal, Sander R Dubovy; Fluorescein Angiography Findings in Diffuse Retinoblastoma: Two Case Reports With Clinicopathologic Correlation. Invest. Ophthalmol. Vis. Sci. 2016;57(12):3673.
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© ARVO (1962-2015); The Authors (2016-present)
Diffuse retinoblastomas are characterized by minimal vertical growth along the retina. Vascular changes produced by retinoblastoma as well as the Fluorescein Angiography (FA) findings have been described in the literature before. Though, there is still not much information available for the diffuse type. Herein, we present the FA findings and the clinicopathlogic correlation of two cases of patients with diffuse retinoblastoma.
The database at the Florida Lions Ocular Pathology Laboratory was searched for surgical specimens diagnosed with diffuse retinoblastoma on light microscopic examination from the period of 1998 to 2015. The specimens and the medical records were reviewed for clinical presentation and FA findings. Clinicopathologic features were then evaluated. IRB/Ethics committee approval was obtained prior to the beginning of this study.
Case 1. A 2-year-old female was referred with a diagnosis of retinitis OD. On examination there was an absent red reflex in the right eye. Fundus examination revealed a hazy media with some hemorrhage present in the macula and overlying the optic nerve. FA revealed tortuous vessels extending from the optic nerve head into the inferior periphery towards a large retinal mass. He underwent enucleation and pathologic evaluation disclosed a full thickness, diffuse retinoblastoma with extension into the vitreous cavity. Case 2. A 16-year old male presented with a post traumatic retinal detachment in the right eye status post repair. Initial fundus examination showed diffuse vitreous hemorrhage and engorged retinal vessels. Initial diagnosis of Coats’ disease vs. uveitis was made. FA revealed tortuous vessels extending out to the periphery towards multiple retinal lesions with complex branching patterns. He underwent enucleation and pathologic evaluation disclosed a diffuse retinoblastoma with greatest thickness at the posterior pole overlying the optic nerve head. Collection of more cases is still ongoing.
FA provides valuable information concerning some of the general anatomical and physiological vascular alterations caused tumors, and therefore these principles are applicable to the atypical diffuse type. These findings correlate with the vascular changes observed on histopathology, and therefore explain how the FA can aim in the diagnosis of atypical retinoblastomas when the clinical presentation is equivocal.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.
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