title: Clinical and epidemiological characteristics of retinoblastoma: correlation with prognosis in a Turkish pediatric oncology center.
abstract: Advanced intraocular tumors and metastatic disease in retinoblastoma patients still occur frequently in developing countries. The aim of this retrospective study was to describe the clinical and epidemiological characteristics of patients with retinoblastoma and the effects of these features on disease prognosis in the authors' pediatric oncology unit as a developing country profile to define the problem. A retrospective chart review of 91 patients who presented to the unit between May 1996 and December 2003 was conducted in this study. Patients with unilateral disease presented at a median age of 24 months and those with bilateral disease at a median age of 9.5 months (p < .01). Most of the eyes with retinoblastoma (68.6%) had Reese-Ellsworth stage V disease. Metastatic disease was diagnosed in 19 (20.9%) patients. Cases with metastatic disease presented at a median age of 24 months and those without metastatic disease at a median age of 12.5 months (p < .05). In 31 patients (34.1%) there was a delay in diagnosis. The enucleation ratio in eyes with advanced intraocular stage was significantly higher than in eyes with early intraocular stage (57.9 vs. 3.8%) (p < .001). In patients with metastatic disease, tumor recurrence was more frequent than in the nonmetastatic patients (36.8 vs. 4.2%) (p < .01). Seven children (7.7%) died due to central nervous system (CNS) metastasis (p < .01). Advanced intraocular disease and distant metastases occur more frequently in Turkish children with retinoblastoma than in children in developed countries, causing a higher rate of enucleation and mortality. Late referral might account for the delayed diagnosis.
authors: Department of Pediatrics, Ankara University School of Medicine, Ankara, Turkey.
authors: Halil
authors: H
authors: Ozdemir
authors: Nurdan
authors: N
authors: Tacyildiz
authors: Emel
authors: E
authors: Unal
authors: Gulsan
authors: G
authors: Yavuz
authors: Handan
authors: H
authors: Ugur
authors: Kaan
authors: K
authors: Gunduz
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols; D001859:Bone Neoplasms / Q000556:secondary / Q000628:therapy; D016543:Central Nervous System Neoplasms / Q000556:secondary / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008495:Medical Oncology; D011379:Prognosis; D019572:Retinal Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy; D012175:Retinoblastoma / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D014421:Turkey / Q000453:epidemiology