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Trilateral retinoblastoma
Author(s) -
Antoneli Célia B.G.,
Ribeiro Karina de Cássia B.,
Sakamoto Luis Henrique,
Chojniak Martha M.,
Novaes Paulo Eduardo R.S.,
Arias Victor E.A.
Publication year - 2007
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.20793
Subject(s) - medicine , retinoblastoma , enucleation , pediatrics , blood cancer , disease , incidence (geometry) , radiation therapy , chemotherapy , pediatric cancer , cancer , surgery , biochemistry , chemistry , physics , optics , gene
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. Procedure 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. Pediatr Blood Cancer 2007;48:306–310. © 2006 Wiley‐Liss, Inc.