Posterior Segment Oncology Feature Story

JANUARY/FEBRUARY 2008 I RETINA TODAY I 41 T he management of retinoblastoma has gradually evolved over the years from enucleation to radiotherapy to current techniques of chemotherapy. Eyes with massive retinoblastoma filling the globe are still managed with enucleation, whereas those with small, medium, or even large tumors can be managed with chemoreduction followed by tumor consolidation with thermotherapy or cryotherapy. Despite multiple or large tumors, visual acuity can reach ≥20/40 in many cases, particularly in eyes with extrafoveal retinopathy, and facial deformities that have been found following external beam radiotherapy are not anticipated following chemoreduction. Recurrence from subretinal and vitreous seeds can be problematic. Longterm follow-up for second cancers is advised. Most of us can only remember a few interesting points from a lecture, even if was delivered by an outstanding, colorful speaker. Likewise, we generally retain only a small percentage of the information that we read, even if written by the most descriptive or lucent author. We usually remember the “three pearls” that are emphasized or the “five forget-me-nots” that are listed. In this article, the important items on systemic care are highlighted as pearls and critical information on ocular care will be listed as forget-me-nots. What is the origin of the name forget-me-not? There are numerous tales associated with this beautiful dusky blue flower. A tragic legend states that a knight and his lady were walking near a river and, upon picking the flower, he tripped into the river with his armour and chanted “Forget me not.” So forget not the legend. Now let’s learn and remember the unforgettables about retinoblastoma. Retinoblastoma represents approximately 4% of all pediatric malignancies and is the most common intraocular malignancy in children.1-3 It is estimated that 250 to 300 new cases of retinoblastoma are diagnosed in the United States each year, and 5,000 cases are found worldPearls and Forget-Me-Nots in the Management of Retinoblastoma