Chemo Can Save Lives in High-Risk Unilateral Retinoblastoma

TOPLINE: 

Adjuvant chemotherapy after removal of the affected eye improves outcomes in children with unilateral retinoblastoma who have high-risk histopathologic features that predispose them to metastases and death. 

METHODOLOGY:

• A retrospective study compared outcomes in children with unilateral retinoblastoma with high-risk histopathologic features who received adjuvant chemotherapy following enucleation vs those who did not adjuvant chemotherapy.
• Data were obtained from 600 children with unilateral retinoblastoma (mean age at diagnosis, 34.8 months; 55.0% boys) from 14 centers in nine countries who underwent removal of the affected eye between 2011 and 2020.
• Among the participants, 505 (84.2%) were considered to have high-risk histopathologic features (HPHF) such as retrolaminar optic nerve invasion, massive choroidal invasion, scleral or extrascleral invasion, anterior segment involvement, or combined nonmassive choroidal and prelaminar or laminar optic nerve invasion; they received adjuvant chemotherapy, with a median follow-up period of 39.2 months.
• Overall, 63 (10.5%) children were initially identified as having non–high-risk disease and did not receive adjuvant chemotherapy but were later classified as having high-risk histopathologic features.
• The main outcomes were recurrence of an orbital tumor, metastasis, death, and combined outcomes (defined as at least one of the three abovementioned outcomes).

TAKEAWAY:

• Massive choroidal invasion and postlaminar optic nerve invasion increased the risk for systemic metastasis and mortality by threefold to fourfold in children with retinoblastoma (P
• Adjuvant chemotherapy significantly reduced the risk for tumor recurrence , systemic metastasis, mortality, and combined outcomes in children with high-risk disease (P
• Among the 63 children who had a delayed conversion to the high-risk group, six experienced tumor recurrence, five developed systemic metastasis (all of whom died) and six died during a follow-up period of 5 years.
• Isolated minor choroidal invasion with prelaminar or laminar optic nerve invasion was reported in 114 children; however, this was considered a high-risk feature and thus was eligible for chemotherapy in only 59.6% of the cases. Six of 114 children developed metastasis and died, four of whom did not receive adjuvant chemotherapy.

IN PRACTICE:

“The implications of not standardizing HRHF and defining protocols for adjuvant therapy result in adverse patient outcomes. As efforts to address Rb [retinoblastoma] on a global scale become evident, corresponding efforts to reduce disparities in Rb outcomes through defined management strategies are warranted, especially among lower income countries,” the study authors wrote.

SOURCE:

This study was led by Mattan Arazi, MD, of the Goldschleger Eye Institute at Sheba Medical Center in Ramat Gan, Israel. It was published online on September 25, 2024, in the American Journal of Ophthalmology.

LIMITATIONS: 

The study was its retrospective nature, which may have introduced biases in the collection and interpretation of the data. The chemotherapy agents were not uniform across all centers, potentially affecting outcomes. The study did not include local guidelines for high-risk histopathologic feature definitions, leading to discrepancies in classification.

DISCLOSURES: 

This study did not receive any funding. The authors declared no conflicts of interests.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.