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M. E. Orellana, B. F. Fernandes, C. Arean, I. Pifano, C. Abreu, M. N. Burnier, Jr.; Clinical Pathological Study of a Cohort of Retinoblastoma Patients From a Developing Country. Invest. Ophthalmol. Vis. Sci. 2007;48(13):1603. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
Retinoblastoma is the most common intraocular tumor in childhood. In underdeveloped countries, advanced retinoblastoma accounts for the majority of cases due to late diagnosis and absence of specialized centers. The objective of our study was to describe the histopathological characteristics of all cases of retinoblastoma received at the Instituto Anatomopatologico, Universidad Central de Venezuela, during a six-year period.
All retinoblastoma cases received in our laboratory between January 2000 and December 2005 were studied. The histological parameters included: degree of differentiation, growth pattern, anterior chamber invasion, choroidal invasion, optic nerve invasion, vitreous seeding and basophilic staining of the vessel wall, vitreous or zonulae. Patient information was collected from the requested biopsy forms and included age, gender and involved eye. The data obtained were entered in a Microsoft Excel spreadsheet and analyzed by means of percentages.
During the study period, a total of 109 eyes from 99 different patients were received. Fifty-five (55.6%) patients were male and 44 (44.4%) were females. There were 9 (8.3%) well differentiated, 25 (23%) moderately differentiated and 66 (60.5%) poorly differentiated retinoblastomas. In 9 (8.3%) of the cases, the degree of differentiation could not be defined due to necrosis. The most common growth pattern was mixed, endo and exophytic comprising 81 (74.4%) of the cases. Vitreous seeding, invasion of the anterior chamber and of the optic nerve beyond the lamina cribosa was present in 79 (72.5%), 24 (22%) and 44 (40.4%) of eyes, respectively. The tumor was confined to the retina in only 26 (23.9%) cases, while 16 (14.8%) had minimal choroidal invasion, 45 (41.3%) had massive choroidal involvement, 10 had scleral invasion and 11 (10.1%) had extraocular extension.
In this study, the percentage of cases with choroidal and optic nerve invasion reflecting poor prognosis, was much higher than previously reported North-American series. However, similar findings have been described in other Latin-American developing countries, where late diagnosis in cases of retinoblastoma is frequent. This study emphasized the need for early diagnosis and treatment in order to avoid optic nerve and extraocular invasion.
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