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Globe-Sparing Therapy for Retinoblastoma Is Increasing

Globe-Sparing Therapy for Retinoblastoma Is Increasing

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

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


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