Updates in the Management of Retinoblastoma

Abstract

Retinoblastoma is the commonest childhood intraocular
malignant tumour, with an approximate
incidence of 1 in 15,000–20,000 live births worldwide.
Advances in treatment over the last quarter century
have led to a survival rate that is over 90% in
developed countries1
. While paradigm shifts have
occurred in conservative treatment, enucleation
(removal of the eye) remains the mainstay for
treatment for advanced disease. With improved
survival rates, there has been an impetus to treat
retinoblastoma without removal of the eye and to
preserve vision.
Previously external beam radiotherapy (EBR) was
extensively used to avoid enucleation. However well
recognised side effects such as second cancers in the
field of radiation, particularly if given in the first year
of life, have limited its use to salvage treatment in
order to avoid enucleation.
2