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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
medicine
Familial retinoblastoma in developing countries
Pediatric Blood and Cancer, Volume 53, No. 3, Year 2009
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Description
Background. Although screening for familial retinoblastoma has been shown to be beneficial we suspected that such screening programs may be less than optimal in developing countries (DC). Methods. Retrospective cohort study comparing patients with familial retinoblastoma from five centers in DC (Argentina, Brazil, Turkey, Jordan, and Venezuela) versus a reference center in the USA. Results. Ninety-two (32 from the USA and 60 from DC) patients were included. Forty-one (44.6%) patients avoided enucleation, 42 (45.7%) had 1 eye removed, and 9 (9.8%) underwent bilateral enucleation. Eleven (11.9%) had major pathology risk factors at enucleation. There were no cases of metastatic disease at diagnosis. Detection via screening was significantly less common in DC than in the USA (23.3% vs. 71.8%, P<0.0001). Patients in DC were diagnosed at a significantly later age and with more advanced intraocular disease that led to increased risk of bilateral enucleation. Patients detected by screening in DC were significantly younger at diagnosis, had less advanced intraocular disease, better ocular preservation rates and survival results than those whose retinoblastoma was not detected via early screening. Five-year pEFS was 0.92 for the patients treated in the USA and 0.81 for the patients in DC (P=0.42). Seven events occurred (extraocular relapse four in patients from DC and second malignancies in three). Conclusions. Patients with familial retinoblastoma are less likely to be diagnosed by screening in DC and had higher morbidity and mortality caused by recurrent extraocular retinoblastoma. © 2009 Wiley-Liss, Inc.
Authors & Co-Authors
Chantada, Guillermo Luis
Argentina, Buenos Aires
Fundacion Hospital de Pediatria Professor Dr. Juan P. Garrahan
Dunkel, Ira J.
United States, New York
Memorial Sloan-kettering Cancer Center
Qaddoumi, Ibrahim A.
Jordan, Amman
King Hussein Cancer Center
Antoneli, Celia B.G.
Brazil, Sao Paulo
A.c.camargo Cancer Center
Totah, Alegria
Venezuela, Caracas
Universidad Central de Venezuela
Canturk, Serife
Turkey, Istanbul
İstanbul Tıp Fakültesi
Nawaiseh, Ibrahim
Jordan, Amman
King Hussein Cancer Center
Fandiño, Adriana C.
Argentina, Buenos Aires
Fundacion Hospital de Pediatria Professor Dr. Juan P. Garrahan
Pífano, Imelda
Venezuela, Caracas
Universidad Central de Venezuela
Peksayar, Gonul
Turkey, Istanbul
İstanbul Tıp Fakültesi
Ribeiro, Karina Braga
Brazil, Sao Paulo
Faculdade de Ciencias Medicas da Santa Casa de Sao Paulo
Abramson, David Harold
United States, New York
Memorial Sloan-kettering Cancer Center
Statistics
Citations: 40
Authors: 12
Affiliations: 7
Identifiers
Doi:
10.1002/pbc.21970
ISSN:
15455009
e-ISSN:
15455017
Research Areas
Health System And Policy
Study Design
Cohort Study
Study Approach
Quantitative