Abstract
A study was conducted to see the association of retinoblastoma with age, bilaterism, Tumor size, site, grades, involvement of anterior chamber, iris, ciliary body, choroid, sclera and optic nerve, and extraocular extension. This was a retrospective descriptive analytical study. The records of the Institute of Ophthalmology, King Edward Medical University/Mayo hospital, Lahore: Pakistan January 2006-Dec 2011 was analyzed. Five years biopsies (June 2006 to June 2012) of the retinoblastoma, from the Pathology department, was retrieved to see optic nerve involvement in all the retrieved specimens. The mean age was 3.54 ± 1.686 years. There were 15 cases of bilateral retinoblastoma and 37 cases with unilateral neoplasms. The age range of bilateral tumors was 2 - 6 years while the range in unilateral tumor was 1 - 10 years male to female ratio was 1:1.5. Among 52 cases, 37 (71.2%) patients were unilateral retinoblastoma and 15 (28.28%) patients were with bilateral disease. On radiological examination of patients suffering from retinoblastoma by B-Scan (Ultrasound), there were 33 (63.5%) cases with exophytic presentation while 18 (34.6%) cases showed endophytic morphology. On clinical examinations, 28 patients presented with proptosis of (it was the most common presenting sign in our patients, accounting for about 53.8% of cases), 20 cases presented with leucocoria eye with strabismus. 29/52 patients showed involvement with optic nerve, 17 were from 1 - 3 years age and 12 were from 4 - 6 years. Statistically there was no difference in the involvement of optic nerve, disc involvement and extra ocular extension with the age of patients (p = 0.217). There was also no statistical difference seen in age and gender for but a strong association was seen with patient’s presentation, symptoms and size of the tumors and anterior chamber, iris, ciliary body, choroid, sclera and optic nerve, and extra ocular extension. We concluded that a positive association was seen with tumor size and tumor extension (optic nerve, and extraocular extension). No Association was seen with age, gender and laterality of the tumors.
Highlights
Retinoblastoma, a rapidly developing cancer and which be inherited, arises from the immature neural retinal cells of retina
The maximum numbers of cases with retinoblastoma eye were of 3 years 18/52 (34.6%) while one case was found with seven years and years group (Table 1)
Our findings about the ages of our patients are not consistent with data of UK presented by Yun et al (2011) and age range of retinoblastoma in Iran presented by Nabie et al
Summary
Retinoblastoma, a rapidly developing cancer and which be inherited, arises from the immature neural retinal cells of retina. Genetic mutation involving “Retinoblastoma gene 1 (Rb1) at chromosome 13 affects has a significant role in its etiology [1]. RB inherited as autosomal dominant trait, with 40% of worldwide cases belonging to this category, needs mutation in both the alleles for the disease to occur [2]. Irrespective of the gender and race the mean age-adjusted incidence of RB in the USA is 11.8 per million children aged 0 - 4 years [3]. It is the commonest paediatric intraocular tumor in children under 5 years of age, occuring in approximately in every 20,000 live birth, was first reported by Benedict [4]. A heritable form where both eye are involved (bilateral retinoblastoma) or sometimes only in one eye, and a non-heritable form where only one eye is involved (unilateral retinoblastoma) [5,6,7]
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