Retinoblastoma in children older than 5 years of age.
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.
de Aguirre Neto
Joaquim Caetano
JC
Department of Pediatric Oncology, Hospital do Câncer AC Camargo, São Paulo, Brazil. caetano@residenciapedhc.com.br
Antoneli
Célia Beatriz Gianotti
CB
Ribeiro
Karina Braga
KB
Castilho
Marcus Simões
MS
Novaes
Paulo Eduardo R S
PE
Chojniak
Martha M M
MM
Arias
Victor
V
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
D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D013713:Teniposide; D002945:Cisplatin; D007069:Ifosfamide