Peertechz Journal of Pediatric Therapy Citation: Pirouzian A (2015) Retinoblastoma (RB) in Saudi Arabia- Retrospective Comparative Review of a Registry. Peertechz J Pediatr Ther 1(1): 015-020. 015 Abstract Purpose: Published data regarding retinoblastoma in Saudi Arabia (KSA) is sparse. The study will present comparison of clinical and pathologic features, and evolving patterns of the treatment during 1983-1997 (Early group=EG) to 1998-2012 (Late group=LG) periods from an RB registry representing all Rb cases in KSA. Methods: This health record review was conducted in 2014. From the RB registry, information about gender, age, clinical features and stage at presentation; laterality, regional distribution, history, co-morbid diseases, pathologic features, surgical treatment modality and treatment outcomes were collected. Results: The EG and LG group had 343 and 430 Rb patients respectively. The incidence of Rb in 1997 and in 2012 was 0.06 and 0.03 per 1,000 live births in Saudi Arabia respectively. The gender distribution was similar. The median age of presentation was 24 (range 11, 39) months in EG vs. 18 (8, 31) months in LG (p <0.001). Incidence of unilateral Rb was 58% [200/343] in EG vs. 56% [278/485] in LG. Positive family history of Rb was <10% in both groups. However, in EG, 120 (35%) parents were irst cousins vs. 124 (27%) in the LG (p=0.12). Leukocoria was the most common presentation [71% (n=255) in EG and 66% (n=343) in LG]. Tumor conined to the eye at presentation was more common in the LG group (67% EG vs. 84% LG). Vitreous seeding was more common in LG [62% (n=302) LG vs 45% (n=156) EG; p<0.001]. In the non-surgical group, photocoagulation rate increased in the LG [23.19% (106) LG vs 3.8 %( n=13) EG; p<0.001]. The rate of chemotherapy increased from 4.1% (n= 14) in EG to 14.2% (n=65) in LG (p <0.001). The rate of external beam radiation decreased [37% (n=125) EG vs. 23.4% (n=107) LG]. The rate of extraocular tumor extension was similar in both groups (53.7% EG vs. 51.6% LG; p<0.001). The rate of full thickness choroidal involvement decreased from 13% (n=35) EG to 7.7 % (n=30) LG, (p =0.04). Optic nerve (ON) involvement in LG decreased to 3.4% (n=22) compared to 9.8 %( n=48) in EG (p <0.001). Conclusions: Most clinical parameters of Rb remained unchanged over 30 years except an earlier median age of presentation suggesting earlier tumor detection. However, an increasing total number of Rb cases were seen in the last ifteen years. The pathologic features in LG were less advanced. A signiicant increase in the non-surgical treatment approaches of Rb was noted in the last 15 years. Research Article Retinoblastoma (RB) in Saudi Arabia- Retrospective Comparative Review of a Registry Introduction Retinoblastoma (RB) is the most common primary intraocular malignancy in children [1-3]. he incidence of RB ranges from 1 in 15,000 to 1 in 30,000 live births worldwide and survival rate varies by country [4,5,10]. It is estimated that RB afects 7,000–8,000 new patients worldwide yearly [4,5]. In developed countries, the survival rate has increased from 30% in the 1930s to more than 90% in the 1990s as a result of an earlier detection and signiicant advances in the overall therapeutic strategy [4]. In developing countries and the underdeveloped countries, however, the mortality rate remains as high as 70% due to poor public awareness and lack of access to innovative treatments [6,7]. Around the world, majority of the estimated 8,000 newly diagnosed patients with retinoblastoma will die each year [8]. In an earlier published report on retinoblastoma in kingdom of Saudi Arabia (KSA), survival rate of 80% was noted among 74 diagnosed cases from the period of 1983-1987 [9]. In a more recent report evaluating demographic information of ocular tumors from tumor registry in KSA, the incidence of malignant ocular tumors in children was found to be 3.6 per million/year (M/Y) and that of Rb was 7.7/M/Y in <5 years olds from [10]. To assess whether implementation of various screening, diagnostic and interventional programs in the past two decades in KKESH have changed the morbidity and mortality of newly diagnosed RB cases, we undertook the current study to compare the clinical and pathologic features, and evolving patterns of the treatment from 1983-1997 (Early) to 1998- 2012 (Late) from the RB registry. Methods he RB registry at King Khaled Eye Specialists (KKESH) hospital represents all RB cases presented and diagnosed in kingdom of Saudi Arabia. KKESH is a tertiary eye center receiving and providing ocular services for the entire kingdom. It captures and maintains comprehensive population-based data on all RB patients in the kingdom. he registry list includes patient name, gender, age, date of diagnosis, laterality, geographic/regional distribution, diagnostic methods, and mode of management, complete medical/family history and co-morbid diseases. However, the registry did not capture the Amir Pirouzian* Johns Hopkins University, Wilmer Eye Institute, 600 N. Wolfe Street, Baltimore, MD 20027, USA Dates: Received: 27 August, 2015; Accepted: 01 December, 2015; Published: 02 December, 2015 *Corresponding author: Amir Pirouzian, M.D. Wilmer Eye Institute, Johns Hopkins University School of Medicine, 600 North Wolfe Street Baltimore, MD 21287; USA, Tel: 858-248-0747; E-mail: www.peertechz.com Keywords: Tumors; Retinoblastoma; Laser; Consanguinity; Children; Saudi Arabia