Massive Extraorbital Extension in a Recurring Untreated Retinoblastoma: A Rare Case from Lebanon

Retinoblastoma is the most common intraocular malignancy occurring in children, with incidence ranging from 1 in 15,000 to 1 in 18,000 live births [1-2] Mortality rates are usually low if treatment is initiated before metastasis occurs [3]. Orbital retinoblastoma usually carries a poor prognosis and is associated with mortality rates as high as 50-90% in developing countries on account of delayed presentations in patients of low socioeconomic backgrounds [4-7]. Additionally, the risk of metastatic disease is reported to be higher in patients with delayed diagnosis [8]. In developing countries, metastatic retinoblastoma remains a leading cause of retinoblastoma related mortality and has been reported to range in frequency from 9-11% [9,10]. In Lebanon, Traboulsi et al. reported data on 58 patients with retinoblastoma followed over 35 years. Data showed that although the epidemiology, genetics and clinical features of the disease were similar to those being reported worldwide, the difference was the striking delay in diagnosis resulting in higher incidences of extraocular extension, advanced disease stages and poorer survival rates [11]. We describe a case of a 2 year old boy with a massive unilateral retinoblastoma as a consequence of delayed presentation.