Aim: This paper presents a 20-year review of retinoblastoma in Johannesburg, South Africa, aiming to better characterize the disease in this sub-Saharan setting. Methods: The study represents a retrospective case series of retinoblastoma patients presenting to Charlotte Maxeke Johannesburg Academic Hospital and Chris Hani Baragwanath Academic Hospital between January 1, 1992, and December 31, 2011. Results: The total number of cases identified was 282, with 245 meeting the study inclusion criteria. Retinoblastoma comprised 6.9% of the total pediatric oncology presentations; 65.3% were unilateral, 34.3% bilateral, and 0.4% trilateral. The overall male-to-female ratio was 1.08. The mean age at presentation overall was 32.6 months (median 28.0), in the unilateral group 39.4 months (median 33.0), and in the bilateral group 19.7 months (median 17.0). The mean delay to presentation overall was 7.0 months (median 4.0). The most frequent presenting symptoms were leukocoria (37.1%) and proptosis (34.7%). The distribution of disease stages at presentation (International Retinoblastoma Staging System) was 1.6% stage 0, 24.1% stage I, 27.8% stage II, 16.3% stage III, and 25.3% stage IV. 26.5% defaulted care. The 5-year Kaplan-Meier survival estimate was 57.7% overall. Conclusion: This study shows that delayed presentation and refusal of therapy remains a significant barrier to effective treatment in this African setting.

Keywords:
Retinoblastoma, Ocular oncology, South Africa, Mortality
1.
Shields CL, Shields JA: Basic understanding of current classification and management of retinoblastoma. Curr Opin Ophthalmol 2006;17:228-234.
2.
Kaimbo WK, Mvitu MM, Missotten L: Presenting signs of retinoblastoma in Congolese patients. Bull Soc Belge Ophtalmol 2002;283:37-41.
3.
Yeole BB, Advani S: Retinoblastoma: an epidemiological appraisal with reference to a population in Mumbai, India. Asian Pac J Cancer Prev 2002;3:17-21.
4.
Houston SK, Murray TG, Wolfe SQ, Fernandes CE: Current update on retinoblastoma. Int Ophthalmol Clin 2011;51:77-91.
5.
Dimaras H, White A, Gallie B: The Kenyan National Retinoblastoma Strategy: building local capacity in the diagnosis and management of pediatric eye cancer in Kenya. Ophthalmology Rounds, July/August 2008, vol 6, issue 4.
6.
Kazadi Lukusa A, Aloni MN, Kadima-Tshimanga B, Mvitu-Muaka M, Gini Ehungu JL, Ngiyulu R, Ekulu Mfutu P, Budiongo Nzazi A: Retinoblastoma in the Democratic Republic of Congo: 20-year review from a tertiary hospital in Kinshasa. J Cancer Epidemiol 2012;2012:920468.
7.
Bekibele CO, Ayede AI, Asaolu OO, Brown BJ: Retinoblastoma: the challenges of management in Ibadan, Nigeria. J Pediatr Hematol Oncol 2009;31:552-555.
8.
Canturk S, Qaddoumi I, Khetan V, Ma Z, Furmanchuk A, Antoneli CB, Sultan I, Kebudi R, Sharma T, Rodríguez-Galindo C, Abramson DH, Chantada GL: Survival of retinoblastoma in less-developed countries impact of socioeconomic and health-related indicators. Br J Ophthalmol 2010;94:1432-1436.
9.
Schefler AC, Abramson DH: Retinoblastoma: what is new in 2007-2008. Curr Opin Ophthalmol 2008;19:526-534.
10.
Chantada G, Doz F, Antoneli CB, Grundy R, Clare Stannard FF, Dunkel IJ, Grabowski E, Leal-Leal C, Rodríguez-Galindo C, Schvartzman E, Popovic MB, Kremens B, Meadows AT, Zucker JM: A proposal for an international retinoblastoma staging system. Pediatr Blood Cancer 2006;47:801-805.
11.
Boubacar T, Fatou S, Fousseyni T, Mariam S, Fatoumata DT, Toumani S, Abdoul-Aziz D, Marouf KM: A 30-month prospective study on the treatment of retinoblastoma in the Gabriel Touré Teaching Hospital, Bamako, Mali. Br J Ophthalmol 2010;94:467-469.
12.
Kruger M, Reynders D, Omar F, Schoeman J, Wedi O, Harvey J: Retinoblastoma outcome at a single institution in South Africa. S Afr Med J 2014;104:859-863.
13.
Nyamori JM, Kimani K, Njuguna MW, Dimaras H: The incidence and distribution of retinoblastoma in Kenya. Br J Ophthalmol 2012;96:141-143.
14.
Bowman RJ, Mafwiri M, Luthert P, Luande J, Wood M: Outcome of retinoblastoma in east Africa. Pediatr Blood Cancer 2008;50:160-162.
15.
Freedman J, Goldberg L: Incidence of retinoblastoma in the Bantu of South Africa. Br J Ophthalmol 1976;60:655-656.
16.
Poyiadjis S, Wainwright L, Naidu G, Mackinnon D, Poole J: The Saint Siluan warning signs of cancer in children: impact of education in rural South Africa. Pediatr Blood Cancer 2011;56:314-316.
17.
Chantada GL, Dunkel IJ, Qaddoumi I, Antoneli CB, Totah A, Canturk S, Nawaiseh I, Fandiño A, Pífano I, Peksayar G, Ribeiro KB, Abramson DH: Familial retinoblastoma in developing countries. Pediatr Blood Cancer 2009;53:338-342.
18.
Dimaras H, Dimba EA, Gallie BL: Challenging the global retinoblastoma survival disparity through a collaborative research effort. Br J Ophthalmol 2010;94:1415-1416.
19.
Essuman V, Ntim-Amponsah CT, Akafo S, Renner L, Edusei L: Presentation of retinoblastoma at a paediatric eye clinic in Ghana. Ghana Med J 2010;44:10-15.
20.
Wiysonge CS, Ngcobo NJ, Jeena PM, Madhi SA, Schoub BD, Hawkridge A, Shey MS, Hussey GD: Advances in childhood immunisation in South Africa: where to now? Programme managers' views and evidence from systematic reviews. BMC Public Health 2012;12:578.
© 2017 S. Karger AG, Basel
2017
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.