Lesions Simulating Retinoblastoma at a Tertiary Care Center

PURPOSE: To determine the types and frequency of ocular conditions simulating retinoblastoma (pseudo-retinoblastoma) at Farabi Eye Hospital, Tehran, Iran. METHODS: We reviewed data of patients who were referred with a diagnosis of retinoblastoma to Farabi Eye Hospital oncology clinic, from January 2009 to July 2013. Examination under general anesthesia was performed for all patients. Other investigations, such as ultrasonography, were performed as required. RESULTS: Of a total of 331 patients (aged 1-60 months), 138 (42%) were found to be suffering from a benign disorder. Among these pseudo-retinoblastoma cases, Coats’ disease was the most prevalent codition (n = 36, 26%); persistent hyperplastic primary vitreous (PHPV) and familial exudative vitreo-retinopathy (FEVR) were the next two common pseudo-retinoblastoma cases in our series. CONCLUSION: The rate of misdiagnosis upon referral to our center was close to 40%. The most common pseudo-retinoblastomas entities include Coats’ disease, PHPV and FEVR. An accurate diagnosis is essential for management of pseudo-retinoblastoma cases.