PAEDIATRIC RETINOBLASTOMA PRESENTATION IN A REGIONAL CANCER CENTRE IN PAKISTAN
Abstract
Purpose: Retinoblastoma (RB) is a common paediatric intraocular tumour. In the developed world, RB accounts for <5% of all paediatric cancers. In the developing world, RB may comprise about 10–15% of all diagnosed paediatric
cancers according to hospital-based retrospective study in India.
Methods: Medical records for all diagnoses of RB at our regional cancer centre were retrospectively reviewed from January 2005 to September 2015 after IRB approval.
Results: A total of 258 (100%) patient charts were reviewed. More male n = 163 (63.2%) patients were seen than females n = 95 (36.8%). Most patients were diagnosed before the age of 5 years. Positive family history was seen
in 20 (7.8%) patients. The total number of bilateral disease was seen in 75 (29.1%) patients and 183 (70.9%) had unilateral diagnoses. Median age at presentation was 24 months for bilateral and 36 months for unilateral disease.
Conclusions: The median age at presentation shows a significant delay in the establishment of diagnosis for both unilateral and bilateral RB. Compared to western data, there were a higher number of male patients seen. Financial
constraints, access to primary health care, lack of trained personnel and cultural practices are likely factors, leading to delayed presentation and advanced disease state.
Key words: Retinoblastoma, ocular tumours, leukocoria
References
Ries LA, Smith MA, Gurney JG. Cancer incidence and survival among children and adolescents: United States SEER Program 1975-1995. Bethesda, MD: National Cancer Institute, SEER Program; 1999. NIH Pub. No. 99-4649.
Kivelä T. The epidemiological challenge of the most frequent eye cancer: Retinoblastoma, an issue of birth and death. Br J Ophthalmol 2009;93:1129-31.
Shields JA, Shields CL. Management of retinoblastoma. In: Shields JA, Shields CL. Intraocular Tumours. An Atlas and Textbook. Philadelphia, PA: Lippincott Williams Wilkins; 2008. p. 334-51.
Berman EL, Donaldson CE, Giblin M, et al. Outcomes in retinoblastoma, 1974-2005: The children’s hospital, westmead. Clin Exp Ophthalmol 2007;35:5-12.
MacCarthy A, Birch JM, Draper GJ, et al. Retinoblastoma:Treatment and survival in great Britain 1963 to 2002. Br J Ophthalmol 2009;93:38-9.
Bowman RJ, Mafwiri M, Luthert P, et al. Outcome of retinoblastoma in East Africa. Pediatr Blood Cancer 2008;50:160-2.
Bhurgri Y, Muzaffar S, Ahmed R, et al. Retinoblastoma in
Karachi, Pakistan. Asian Pac J Cancer Prev 2004;5:159-63.
Meel R, Radhakrishnan V, Bakhshi S. Current therapy and recent advances in the management of retinoblastoma. Indian J Med Paediatr Oncol 2012;33:80-8.
Aerts I, Lumbroso-Le Rouic L, Gauthier-Villars M, et al. Retinoblastoma. Orphanet J Rare Dis 2006;1:31.
Abramson DH, Frank CM, Susman M, et al. Presenting signs of retinoblastoma. J Pediatr 1998;132:505-8.
Chantada G, Fandiño A, Manzitti J, et al. Late diagnosis of retinoblastoma in a developing country. Arch Dis Child 1999;80:171-4.
Rodriguez-Galindo C, Wilson MW, Chantada G, et al. Retinoblastoma: One world, one vision. Pediatrics2008;122:e763-70.
Leander C, Fu LC, Peña A, et al. Impact of an education program on late diagnosis of retinoblastoma in Honduras. Pediatr Blood Cancer 2007;49:817-9.
Selistre SG, Maestri MK, Santos-Silva P, et al. Retinoblastoma in a pediatric oncology reference center in Southern Brazil. BMC Pediatr 2016;16:48.
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