Bilateral retinoblastoma: clinical presentation, management and treatment
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CASE REPORT
Bilateral retinoblastoma: clinical presentation, management and treatment Francesco Pichi • Andrea Lembo • Mariacarla De Luca • Theodora Hadjistilianou Paolo Nucci
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Received: 27 November 2012 / Accepted: 19 December 2012 Ó Springer Science+Business Media Dordrecht 2012
Abstract Management of retinoblastoma (Rb), the most common intraocular malignant tumor in childhood, is tailored to each individual case and based on the overall situation. We present a case of bilateral Rbs in a 4-month-old girl, referred to our center for bilateral leukocoria. In the right eye, the optic disc was partially visible, and three large foci of retinoblastoma were noted adjective in the vitreous cavity with satellite retinal detachment. The macula was obscured by the tumors. The tumor was therefore classified as group D (International classification of retinoblastoma), and thus underwent intravenous chemotherapy with the standard three-agent protocol of ifosfamide, carboplatin and etoposide (ICE protocol) delivered monthly for six cycles, regressing with a type I calcified pattern. The left eye presented three multifocal yellow-white retinal masses, with a total retinal detachment, and secondary glaucoma. The lesions were classified as group E and, therefore, taking the results obtained by Shields et al. in group E tumors into consideration, underwent enucleation. This case clearly shows that this cancer is curable if detected at a stage in which it is still contained within the retina, F. Pichi (&) A. Lembo P. Nucci San Giuseppe Hospital, University Eye Clinic, Via San Vittore 12, 20123 Milan, Italy e-mail: [email protected] M. De Luca T. Hadjistilianou Department of Ophthalmology, Azienda Ospedaliera e Universitaria Santa Maria alle Scotte, Siena, Italy
subretinal space or vitreous, and that the management of Rb relies on an experienced team of ocular oncologists, pediatricians and pediatric ophthalmologists working together for the single goal of saving the child’s life. Keywords Retinoblastoma Enucleation Intravenous chemotherapy
Introduction Retinoblastoma (Rb) represents approximately 4 % of all pediatric malignancies and is the most common intraocular malignancy in children [1–3], with an estimated 5,000 cases worldwide each year. Most ([95 %) children with Rb in medically developed countries survive their malignancy, whereas approximately only 50 % survive worldwide. The reason for the poor survival in undeveloped countries relates to late detection of advanced Rb, often presenting with orbital invasion or metastatic disease. About two-thirds of all cases are unilateral and onethird of cases are bilateral. Genetically, it is simpler to discuss Rb with the classification of somatic or germline mutation. Germline mutation implies that the mutation is present in all cells of the body whereas somatic mutation means that only the tissue of concern, the Rb, has the mutation.
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Int Ophthalmol Fig. 1 Bilateral leukocoria and esotropia (a) in a 4 month-old female; external examination reveals a w
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