Retinoblastoma in Older Childre Zeynel A. Karcioglu, MD, a,b Emad B. Abboud, MD, a Saleh A. Al-Mesfer, MD, a Waleed Al-Rashed, MD, a and Delia H. Pilapil, RN, COT a Purpose: Retinoblastoma (RB) is known to present with atypical signs and symptoms in older patien reviews the clinical and histopathological features of RB encountered in children older than 5 years of age. Methods: A totalof 337 consecutive patients with RB were reviewed. Eighteen (5.3%; 10 males, 8 females) who presented after the age of 5 were selected for further analysis of clinical data and histopathological material in this retrospective, non-comparative case series. Results: The age range was from 5 to 12 years (mean age 6.4) Unilateral disease was seen in 14 children, bilateral disease in 4 children. Eleven children were the product of consanguineous marriages. Seven patients had leukocoria; the others presented with signs of uveitis, cellulitis, and/or trauma. Three patients had flat, plaque-like lesions on histopathologic evaluation. Conclusion histopathologic features were atypical in 47% and 21% respectively in the retinoblastoma patients w after the age of 5 years. (J AAPOS 2002;6:26-32) W orldwide incidence of retinoblastoma (RB) is reported to range from 1 in 15,000 to 35,000 live births per year. 1-4 The incidence of RB in Saudi Arabia is estimated to be 1 in 15,000 live births per year. 5,6 Although RB has been diagnosed in older children and adults, the average patient age at diagnosis is about 1 year and 2 years in bilateral and unilateral disease, respective- ly. 7-9 Previous studies in Saudi Arabia indicate that 50% of all RB patients present under the age of 2 years,which corresponds with findings of Pendergrass and Davis 3 in the US. Other case studies and series of RB in older individuals have been reported. 10 The significance is not simply sci- entific curiosity about the oncogenesis of this tumor at an advanced age; more importantly, RB in older patients usually manifests atypical signs and symptoms referred to as masquerade syndrome, 7-11 which may be misleading in diagnosis and management. SUBJECTS AND METHODS This retrospective study included a review of all 337 pa- tients with RB at the Tumor Registry of King Khaled Eye SpecialistHospital,Riyadh,SaudiArabia,seen between July 1983 and November 1997. Patients who were 5 years or older at the time oftheir initialRB diagnosiswere selected and analyzed further. The clinical and histopatho logical data on these patients were reviewed with particu lar attention paid to unordinary clinical manifestations for RB and consequent pitfalls in diagnosis and management RESULTS The study included 18 children, 10 males (55.6%) and 8 females (44.4%) with RB, 4 of whom had bilateral disease No family history of RB could be established in any of the cases. Ten patients (55.5%) were diagnosed at the age of years, 2 patients (11.1%) were diagnosed at 6 years of ag 1 patient (5.5%) was diagnosed at 6.5 years of age, and 5 patients (27.7%) were diagnosed between the ages of 7 a 12 years. Seven patients presented with only leukocoria. Two patients presented with leukocoria plus uveitis-like symp- toms; 1 of these patients had a history of trauma. Anothe child came to our attention with leukocoria, strabismus, and hyphema. One patient had a uveitis-like presentation without other symptoms, 1 presented with uveitis symp- toms and strabismus, 1 presented with proptosis seconda to extraocular extension of RB, and 1 child was seen for strabismusbuthad no othercomplaints. Four patients came in with a complaint of poor vision, 1 of whom was associated with trauma and pseudohypopyon. One of the bilateral cases presented for examination of the fellow eye, at which time the bilateral nature of the disease was established. Another patient with bilateral dis ease presented at the age of 12 with a large active RB in left eye and a regressed tumor in the right eye (Table, Ca 3). She had aphakia of the left eye and the parents insiste that she underwent cataract surgery at the age of 3 years at which time there was no evidence of tumor in either ey Fourteen (63.6%) eyes were enucleated as primary treat- ment,and 8 (36.4%)eyes were treated by other means (chemotherapy, externalbeam radiation therapy, I-125 From the King Khaled Eye Specialist Hospital, a Riyadh, SaudiArabia,and the Tulane University Health Sciences Center, b NewOrleans, Louisiana. Supported in part by an unrestricted grant provided by St. Giles Foundation of NewYork City, NewYork (Dr Karcioglu). Submitted March 28, 2001. Revisions accepted September 25, 2001. Reprint requests: Zeynel A. Karcioglu, MD, Tulane University Health Sciences Center, Department of Ophthalmology, 1430 Tulane Ave SL 69, NewOrleans, LA 70112-2699. Copyright© 2002 by theAmerican Association for PediatricOphthalmology and Strabismus. 1091-8531/2002/$35.00 ⫹ 0 75/1/120643 doi:10.1067/mpa.2002.120643 Journal of AAPOS 26 February 2002