title: Retinoblastoma in children older than 5 years of age.
abstract: BACKGROUND
  Retinoblastoma is a malignant tumor of the embryonic neural retina. About 80% of cases are diagnosed before age 4, with a median age at diagnosis of 2 years.
  
  
  OBJECTIVE
  To determine characteristics and prognosis of retinoblastoma in children older than 5 years.
  
  
  METHODS
  From 1986 to 2002, medical records of 16 patients out of 453 cases referred to Hospital do Câncer AC Camargo, São Paulo, Brazil.
  
  
  RESULTS
  Median age at diagnosis was 73.7 months (range 65-144) and there was an equal gender distribution. Fifteen patients presented with unilateral disease. The mean time between first symptoms and diagnosis was 9.6 months (range 0-48). Most cases were diagnosed in advanced stages and 15 eyes were enucleated. Eleven patients presented with intraocular tumor (1 Reese II and 10 Reese V) and five presented with extraocular disease (one CCG II and four CCG III). Twelve patients are still alive with a median follow-up of 92 months (range 65-199).
  
  
  CONCLUSIONS
  Because of its low incidence at this age, diagnosis of retinoblastoma is usually delayed due to low level of suspicion. Therefore, it is important that physicians are aware of this disease in order to perform an earlier diagnosis, and decrease treatment-related morbidity.
authors.affiliation: Department of Pediatric Oncology, Hospital do Câncer AC Camargo, São Paulo, Brazil. caetano@residenciapedhc.com.br
authors.forename: Joaquim Caetano
authors.initials: JC
authors.lastname: de Aguirre Neto
authors.forename: Célia Beatriz Gianotti
authors.initials: CB
authors.lastname: Antoneli
authors.forename: Karina Braga
authors.initials: KB
authors.lastname: Ribeiro
authors.forename: Marcus Simões
authors.initials: MS
authors.lastname: Castilho
authors.forename: Paulo Eduardo R S
authors.initials: PE
authors.lastname: Novaes
authors.forename: Martha M M
authors.initials: MM
authors.lastname: Chojniak
authors.forename: Victor
authors.initials: V
authors.lastname: Arias
mesh_terms: D017677:Age Distribution; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001938:Brazil / Q000453:epidemiology; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D015353:Eye Enucleation; D005134:Eye Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D053208:Kaplan-Meier Estimate; D017075:Laser Coagulation; D008297:Male; D009361:Neoplasm Invasiveness; D009367:Neoplasm Staging; D018714:Radiotherapy, Adjuvant; D012175:Retinoblastoma / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D013713:Teniposide / Q000008:administration & dosage; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D013713:Teniposide; D002945:Cisplatin; D007069:Ifosfamide