FORT LAUDERDALE, Florida—Current conservative management of pediatric intraocular retinoblastoma is very effective, especially with chemotherapy plus focal ablation of tumors. But enucleation remains the primary therapeutic choice in children, undertaken in almost three quarters of cases, investigators reported at the Association for Research in Vision and Ophthalmology (ARVO) annual meeting.
Timely diagnosis and treatment of retinoblastoma, which occurs in one in 23,000 live births, have contributed to a marked improvement in patient survival. Therapeutic strategies have evolved considerably, and current modalities include enucleation, external beam radiotherapy, scleral plaque radiotherapy, transpupillary thermoablation, cryotherapy, laser ablation, and chemotherapy.
A series of 461 cases retrospectively evaluated over 3 decades shows that enucleation rates have declined considerably, from 96% in the 1970s to 73% today, due to earlier diagnosis and effective conservative therapy, reported John A. Epstein, MD, of the Wills Eye Hospital, Philadelphia (abstract 2588).
According to the Reese-Ellsworth classification of disease severity (size, location, and number of tumors; presence of seeding), enucleation in Dr. Epstein’s series was performed on 84% of stage V patients, but only 38% of stage IV, 20% of stage III, 6% of stage II, and 0% of stage I patients. Eyes with vitreous seeding only were commonly enucleated (76%), while those with subretinal seeding were less commonly enucleated (49%) and often treated with chemoreduction (36%), Dr. Epstein reported.
The management of unilateral retinoblastoma, therefore, varies considerably depending on many factors. To prevent metastasis, aggressive treatment is often necessary in patients whose disease is obviously severe and threatening, but a skilled and insightful treatment team can often spare the globe in some patients with advanced disease, Dr. Epstein’s and other reports from the ARVO meeting suggested.
"These days you can have good results even in advanced stages without enucleation," he said. "But at some point, you reach a threshold. You can’t treat all patients conservatively."
Chemoreduction vs Radiotherapy
