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Talia Rosenberg Kaden, Jasmine H Francis, Brian Marr, Scott E Brodie, Y. Pierre Gobin, David H Abramson; (Neonatal) Retinoblastoma in the First Month of Life: Comparison of the Pre- and Post-Intraarterial Chemotherapy Era. Invest. Ophthalmol. Vis. Sci. 2014;55(13):6017. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
This project evaluated the clinical features, treatment and outcomes of children diagnosed with retinoblastoma (Rb) before 1 month of age. We assessed how intra-arterial chemotherapy (IAC) impacted outcomes when compared with a similar, pre-IAC cohort in the literature.
This is a retrospective review of patients diagnosed with Rb before 31 days between 2002 and 2013. We recorded patients’ sex, family history, age at diagnosis, manifesting symptom, laterality, stage of ocular disease at diagnosis, number of and location of tumors, electroretinograms pre-and post treatment, development of new ocular or extraocular tumors, treatment, length of follow-up, ocular and patient survival. Each eye was classified at diagnosis by Reese-Ellsworth (RE) and International Classification (ICRB) systems.
We identified 22 patients with a median follow-up of 4.3 years. The patients in the pre- and post-IAC era were comparable in mean age at diagnosis, gender distribution, reason for diagnosis, laterality on presentation, location of presenting and recurrent tumors and initial tumor classification. Only 1 patient in our post-IAC cohort received external beam radiotherapy (EBRT) as compared to 21 of 46 patients in the pre-IAC cohort (p<0.05). All eyes were treated with laser therapy and 70% of our patients underwent IAC after receiving bridge intravenous chemotherapy. 30-Hz flicker ERG responses remained stable or improved by 6 months after the first IAC. Most notable were the increased patient and ocular survival in our cohort. 100% of our patients are alive while 17% of the pre-IAC cohort died during follow-up (p<0.05). We retained all but one RE Va/ICRB D eye. In contrast, 10 out of 11 stage V eyes were enucleated in the pre-IAC era (p<0.05). 4 patients developed second nonocular cancers within 5 years of diagnosis in the pre-IAC cohort while we have no extraocular Rb or second tumors in our cohort to date.
Our study is consistent with previous literature in terms of demographics, but our data differs substantially from previous reports in ocular survival, patient survival and incidence of nonocular tumors. These results suggest that the use of IAC in patients with very early diagnoses of Rb has meaningful benefits for patients, particularly for those who present initially with extensive intraocular disease.
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