Risk of metastatic disease after retinoblastoma warrants careful, long-term follow-up

Br J Ophthalmol. 2009;93(4):463-467.

The risk of orbital recurrence after enucleation for retinoblastoma is modest, but careful monitoring is warranted given the risk of metastatic disease, according to a study.

Looking at a historical cohort of 1,674 consecutive patients undergoing enucleation between 1914 and 2006, researchers identified 71 cases of orbital recurrence, an incidence of 4.2%. However, in follow-up to a maximum of 208 months (mean, 34.8 months), 60 of those 71 patients (85%) developed metastatic disease and 53 (75%) died as a result of metastatic retinoblastoma.

Subgroup analysis of the 71 cases showed that orbital tumor recurrence occurred within 2 years of initial surgery, and 69 (97%) occurred in the first 12 months postoperatively. Additionally, 10 of 11 patients diagnosed since 1984 with a recurrence were still living as of the last follow-up, suggesting improvements in management techniques in the modern age, according to the study.

"Histopathological risk factors are not always reliable in predicting which patients will ultimately develop orbital tumor recurrence," the study authors said. "Therefore, all patients undergoing enucleations for intraocular retinoblastoma require careful follow-up during the first 2 years after surgery, and clinicians should be aware that patients may be asymptomatic or present with subtle, non-localizing or even contralateral symptoms."