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A. Mataftsi, D. Hadjistilianou, A. Balmer, L. Zografos, F.L. Munier; Malignant Transformation in Two Retinoma Cases . Invest. Ophthalmol. Vis. Sci. 2003;44(13):4984.
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Purpose: To describe spontaneous malignant transformation in two retinoma patients. Methods: Prospective follow-up of 2 patients with sporadic unifocal retinoma was performed by monthly examination under narcosis. Retinal tumors were documented both by photography (Retcam) and b-mode ultrasonography. Results: Age at diagnosis was 22 months in a girl presenting with leucocoria and strabismus of the right eye (case 1), and 14 months in a boy with leucocoria of the right eye (case 2). Neither of them had received previous treatment. Both tumors were consistent with a type III regression pattern, with a macular epicenter. First evidence of growth occurred 11 months and 13 months after diagnosis in case 1 and 2 respectively. Growth rate of these 2 tumors was estimated. Conservative treatment was initiated in both cases. Conclusions: To the best of our knowledge this report is the first to document in vivo the birth of retinoblastoma arising from a premalignant tumor. Such a secondary tumorigenesis would imply at least 3 mutational events to occur, which is in contrast with the 2 mutations responsible for retinoblast transformation.
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