Histopathologic risk factors in retinoblastoma: A retrospective study of 172 patients treated in a single institution

Orbital recurrence of retinoblastoma following enucleation

“…In 1987, Hungerford et al reported an incidence of 5% for orbital retinoblastoma in a group of 317 children referred for retinoblastoma, although they acknowledged that some of these cases already had extraocular disease at presentation 8. Khelfaoui et al reported a slightly higher incidence of 7.6% in 172 enucleated eyes, although again, some of these cases were referred for extraocular disease and had evidence of metastasis at presentation 9. The analysis of 1674 patients enucleated by our service from 1914 to 2006 showed an orbital relapse rate of 4.2%, which may be more accurate than previous reports due to the large number of patients in our series and the inclusion of only biopsy-confirmed cases of orbital recurrence following enucleation.…”

“…There appears to be universal agreement that optic-nerve invasion with tumour involvement of the resection margin and extrascleral spread are highly predictive markers for extraocular relapse 26 27. The prognostic value of other extraretinal features, such as choroidal invasion and retrolaminar optic-nerve invasion (with a negative margin), has been debated, although there is general agreement that these features increase the risk for systemic disease 9 18. In our series of orbital recurrence cases, 35 of the 70 globes (with data available) demonstrated evidence of optic nerve invasion, and 32 of 66 globes (with data available) had choroidal invasion, indicating a higher risk of metastatic disease in this cohort of patients than other published series of retinoblastoma patients undergoing enucleations 15 16 26.…”

“…Orbital tumour recurrence following enucleation is considered a relatively rare complication, although its true incidence has only been estimated 8 16. There have also been efforts to demonstrate an association between certain histopathological features of enucleated eyes and the risk for local and systemic relapse, although a consensus on these putative risk factors is lacking 9 15 – 19. Other than isolated case reports, few studies have described the clinical profiles of patients who present with an orbital tumour recurrence after an uncomplicated enucleation 20 21.…”

Orbital retinoblastoma: Where do we go from here?

“…[13][14][15][16][17] The presence of orbital invasion is associated with 10-27 times higher risk of metastasis when compared to cases without orbital extension. [18][19][20] The 5-year survival rates of orbital retinoblastoma has been reported to be 88% from the United Kingdom, [21] 91% from Japan [22] and 93% from the United States. [23,24] However, the mortality in developing countries is still high owing to late presentations compounded by socioeconomic factors, with the mortality reported as high as 50-90%.…”

High Risk Retinoblastoma: Prevalence and Success of Treatment in Developing Countries

“…24 However, there are standing disparities in these risk definitions. 5,13 Choroidal invasion represents a risk for metastasis due to facilitation of tumor invasion through emissary vessels, and although it's considered a risk for metastasis, administering adjuvant chemotherapy for independent choroidal invasion is still a controversial issue. 25 Finding out that about 40% of our unilateral patients have risk features alerts physicians in developing countries to the fact that almost half of their patients are liable to tumor dissemination.…”