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Study: Enucleation preferred treatment for advanced unilateral retinoblastoma
J AAPOS. 2009;13(6):546-550.
Chemotherapy and focal therapy are suitable for treating less severe retinoblastoma, but enucleation is appropriate for advanced unilateral cases, according to a study.
"'Conservative' treatment is an option when there is good potential for useful vision without prolonged, costly therapy with potential side effects," the study authors said. "Simple enucleation reduces the risk of masking high-risk pathology and promotes early return to normal life."
The retrospective study included 130 patients with unilateral retinoblastoma. Mean patient age at disease presentation was 26 months. Investigators reviewed presentation, age at diagnosis, classification of disease severity, genetic status, mode of treatment and long-term outcomes.
Enucleation was the primary treatment for 106 patients.
Study data showed a statistically significant correlation between severe disease at presentation and adverse histopathological risk factors (P < .001).
Results showed that treatment was successful in nine of 16 patients who underwent eye-conserving therapy. Two patients with a relevant family history of retinoblastoma were diagnosed early and underwent focal therapy.
Three patients regained visual acuity of 6/18 or better in the treated eye. Enucleation was later performed in seven patients. One patient died of metastasis after parents delayed consent for enucleation and refused prophylactic chemotherapy for high-risk histopathology, the authors said.
The authors review retrospectively the presentation and outcome of 130 pediatric patients with unilateral retinoblastoma. Based on their results, they emphasize the importance of careful selection of patients for eye-preserving treatments. Even with patient categorization, based on the International Intraocular Retinoblastoma Classification, seven of 16 patients initially treated with eye-preserving treatment ultimately required enucleation for recurrence or worsening of tumor activity. The authors point out that while current chemotherapy and focal therapy may be appropriate for very select patients, it should not replace primary enucleation as the treatment of choice.
– Jean E. Ramsey, MD, MPH
Director,
Pediatric Ophthalmology Service, Boston Medical Center, Associate Professor of
Ophthalmology and Pediatrics, Boston University School of Medicine