Clinical Features and Long-Term Follow-Up of Patients with Retinoblastoma in Turkish Children Older Than 5 Years of Age

Zafer Cebeci; Samuray Tuncer; Rejin Kebudi

doi:10.1155/2020/8148013

Clinical Features and Long-Term Follow-Up of Patients with Retinoblastoma in Turkish Children Older Than 5 Years of Age

J Ophthalmol. 2020 Jan 20:2020:8148013. doi: 10.1155/2020/8148013. eCollection 2020.

Authors

Zafer Cebeci¹, Samuray Tuncer¹, Rejin Kebudi²

Affiliations

¹ Istanbul University, Istanbul Faculty of Medicine, Ophthalmology Department, Istanbul, Turkey.
² Istanbul University, Oncology Institute, Division of Pediatric Hematology-Oncology, Istanbul, Turkey.

Abstract

Purpose: The diagnosis and management of retinoblastoma can be difficult in older children. This study reports the clinical features and long-term outcomes of such patients in a Turkish patient population.

Methods: Medical records of 18 patients, between January 1992 and December 2017, were reviewed retrospectively.

Results: Of 298 consecutive patients with retinoblastoma, 18 (6%) were at the age of 5 years and older. The median age at diagnosis was 9 years (range, 5-18 years). Misdiagnosis was noted in 8 patients (44.4%). Treatment options included enucleation in 16 patients (88.8%), adjuvant systemic chemotherapy in 3 (16.6%), intra-arterial chemotherapy in one (5.5%), and additional orbital irradiation in one (5.5%). After a median follow-up of 97 months (range, 6-252 months), all patients survived without any recurrence.

Conclusions: Our series showed that 6% of patients with retinoblastoma were 5 years of age and older. Over a 15-year period, ocular salvage rate of 11.2% and survival rate of 100% were attained.