Incidence and Management of Retinoblastoma

Abstract

This was a retrospective as well as prospective study of fifty (50) cases of retinoblastoma in series during the study period from January 2005 to December 2015, conducted at Liaquat Medical College Eye hospital and Indus Medical College Hospital, Pakistan. The average incidence of retinoblastoma in cases presented in our department was 4.5 cases per year with 0.8 cases per year bilateral and 3.7 cases per year with unilateral involvement respectively. The minimum age in this series was 15 days and maximum age was 8 years, mean age being 3.46 years. Out of these 50 cases, 27(54%) were male and 23(46%) were females. In most of the cases diagnosis was made on clinical grounds and histopathological reports of the biopsy specimens. Other measures like plain X-ray orbit, CT scan, ultrasonography (A and B Scan) and studies of blood chemistry were used where ever needed. Majority of our cases were found to be in a fairly advanced stage (an aplasia grade severe) that is extending beyond the sclera in the orbital tissues. In 4 cases cat’s eye reflex noted. Out of these cases 3 were having involvement of the other eye extensively while in 4th case the other eye was not involved at all. Of the remaining cases 11(22%) were having vitreous seeding, 16(32%) showed extension up to the sclera and 22(44%) extended to involve the orbit. Only one case presented with the involvement of regional lymph nodes. All those cases that underwent modified exonerations were sent to radio isotope center for further management with radiation and/or chemotherapy depending upon the nature of spread. One patient who had the regional lymph nodes involvement, though did not show extra ocular spread in the orbit, underwent chemotherapy following enucleations.2 cases which were diagnosed in very early stage involving the posterior retina behind equator were managed by 60 Cobalt radioactive plaques insertion over the sclera at the site of lesion, left in place till such time as advised by the oncologist of the radio isotope center. A total of 28 enucleations and 19 modified exentrations were carried out. Recurrences were noted in 13 cases, no surgery was done in these cases and they were referred to radio isotope center at Jamshoro, Pakistan for further management with radiation or chemotherapy. In this series no case was found to have distant metastasis anywhere including long bones and other viscera of the body. 12 cases were lost to follow up at different stages of management and 6 of the cases died of unknown cause.