Trilateral retinoblastoma.
BACKGROUND
Trilateral retinoblastoma (TRB) is a syndrome consisting of unilateral or bilateral hereditary retinoblastoma (Rb) associated with an intracranial neuroblastic tumor. Although its incidence is low, the prognosis is very poor. This article reports four cases of TRB and discusses the role of neuroimaging screening for early detection.


METHODS
From January 1986 to December 2003, 470 children with Rb were admitted to the Pediatrics and Ophthalmology Departments, A C Camargo Hospital, São Paulo, Brazil.


RESULTS
There were four patients with pineoblastoma, two of whom had a positive familial history. The age at diagnosis of Rb was 4, 6, 10, and 24 months while the age of diagnosis of TRB was 10, 25, 57, and 72 months. One patient presented TRB at initial diagnosis of Rb. Three patients had bilateral disease and all of them had one eye enucleated, followed by chemotherapy and/or external beam radiation therapy (EBRT). One child with unilateral disease was only submitted to enucleation. In spite of intensive treatment, all patients died with progressive disease within 7, 8, 12, and 12 months after diagnosis of TRB.


CONCLUSIONS
Early diagnosis as well as new therapeutic approaches are needed to achieve better results.
Pediatric Oncology Department, Centro de Tratamento e Pesquisa Hospital do Câncer A C Camargo, São Paulo, Brazil. cantoneli@terra.com.br
Célia B G
CB
Antoneli
Karina de Cássia B
Kde C
Ribeiro
Luis Henrique
LH
Sakamoto
Martha M
MM
Chojniak
Paulo Eduardo R S
PE
Novaes
Victor E A
VE
Arias
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D016371:Cranial Irradiation; D003520:Cyclophosphamide / Q000008:administration & dosage; D018450:Disease Progression; D005047:Etoposide / Q000008:administration & dosage; D015353:Eye Enucleation; D005134:Eye Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D015255:Idarubicin / Q000008:administration & dosage; D007069:Ifosfamide / Q000008:administration & dosage; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D009378:Neoplasms, Multiple Primary / Q000188:drug therapy / Q000235:genetics / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery; D016609:Neoplasms, Second Primary / Q000188:drug therapy / Q000235:genetics / Q000473:pathology* / Q000532:radiotherapy; D009386:Neoplastic Syndromes, Hereditary / Q000235:genetics / Q000473:pathology; D010871:Pinealoma / Q000188:drug therapy / Q000235:genetics / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery; D011379:Prognosis; D012175:Retinoblastoma / Q000188:drug therapy / Q000235:genetics / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery; D014750:Vincristine / Q000008:administration & dosage
D014750:Vincristine; D005047:Etoposide; D003520:Cyclophosphamide; D002945:Cisplatin; D007069:Ifosfamide; D008727:Methotrexate; D015255:Idarubicin