This paper reports the use of a modified staging criteria for optic nerve invasion in extraocular retinoblastoma and its correlation with treatment outcomes in 21 patients. The average age at presentation was 41 months (7–120) and there were 14 unilateral and seven bilateral cases. Presenting history included leukocoria (n=17), proptosis (n=3) and strabismus (n=1). Eleven had optic nerve enhancement only with no optic nerve thickening with length involvement of 2mm to the orbital apex. Ten had optic nerve thickening with enhancement. Staging results included 11 at stage 0, two at stage 1, one at stage 2 and seven at stage 3, at presentation. Post neo-adjuvant chemotherapy of 10 patients at three cycles, there were three complete responses, five partial and two stable responses. There were no cases of progression. All with presentation stages of 1 and 2 had complete resolution (n=3). Of seven with stage 3 presentation, three were stable and four had partial responses. Average event-free survival was 27.71 months. Five patients died of central nervous system metastasis at a mean follow-up of 14 months. All stage 0 and 1 presentations were alive and free of disease. Treatment complete responses had better survival rates. Survival outcomes were significantly correlated with baseline staging. However, the authors acknowledge their small patient numbers and a larger study is needed.
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Using modified staging criteria to determine optic nerve invasion in retinoblastoma
Reviewed by Fiona Rowe
CONTRIBUTOR
Fiona Rowe (Prof)
Institute of Population Health, University of Liverpool, UK.
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