2Departments of Ophthalmology and Oncology, Johns Hopkins Medicine, Baltimore, MD and University of Cincinnati College of Medicine, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA
Abstract
Early diagnosis and treatment of retinoblastoma are essential because the probability of cure is high while the tumor is still intraocular. However, this prognosis gets significantly worse when it spreads outside the eye ultimately leading to death.
The most common presenting sign of this tumor in developed countries is leukocoria (60%) followed by strabismus (20%). The remainder 20% shows atypical presentations that can masquerade as other ocular or orbital pathology. Although these presentations are less common, they pose a diagnostic challenge and are often associated with delayed diagnosis and consequently advanced disease that impacts the outcome and patient survival of patients.
The purpose of this manuscript is to review and emphasize atypical clinical presentations simulating orbital cellulitis, uveitis and endophthalmitis among others that encompass the clinical spectrum of retinoblastoma and comment on current management recommendations.
In order to accomplish such charge, the authors performed a comprehensive search in PubMed and Web of science of English language publications that described atypical presentations of retinoblastoma between 1960 and the present time. The distinct atypical clinical presentations encountered are revised in detail in this manuscript.
Keywords: Eye Cancer; Retinoblastoma Unilateral; Retinoblastoma Bilateral; Leukocoria; Strabismus; Chemotherapy/Intra-Venous; Chemotherapy/Intra-Arterial; Cancer Treatment
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