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, 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.
References
Shields CL, Shields JA (2006) Basic understanding of current classification and management of retinoblastoma. Curr Opin Ophthalmol 17:228–234
Shields JA, Shields CL (2008) Retinoblastoma. In: Shields JA, Shields CL (eds) Atlas of Intraocular Tumors. Lipincott Williams Wilkins, Philadelphia, pp 293–365
Shields JA, Shields CL (1992) Management and prognosis of retinoblastoma. Intraocular tumors: a text and atlas. WB Saunders, Philadelphia, pp 377–392
Epstein J, Shields CL, Shields JA (2003) Trends in the management of retinoblastoma; Evaluation of 1,196 consecutive eyes during 1974–2001. J Pediatr Ophthalmol Strab 40:196–203
Shields CL, Meadows AT, Leahey AM, Shields JA (2004) Continuing challenges in the management of retinoblastoma with chemoreduction. Retina 24:849–862
Shields CL, Honavar SG, Shields JA, Demirci H, Meadows AT, Naduvilath TJ (2002) Factors predictive of recurrence of retinal tumor, vitreous seeds and subretinal seeds following chemoreduction for retinoblastoma. Arch Ophthalmol 120:460–464
Shields CL, Honavar SG, Meadows AT, Shields JA, Demirci H, Singh AD, Friedman D, Naduvilaths TJ (2002) Chemoreduction plus focal therapy for retinoblastoma: factors predictive of need for treatment with external beam radiotherapy or enucleation. Am J Ophthalmol 133:657–664
Shields CL, Mashayekhi A, Cater J, Shelil A, Meadows AT, Shields JA (2004) Chemoreduction for retinoblastoma analysis of tumor control and risks for recurrence in 457 tumors. Am J Ophthalmol 138:329–337
Murphree AL (2005) Intraocular retinoblastoma: the case for a new group classification. Ophthalmol Clin North Am 18(1):41–53
Shields CL, Mashayekhi A, Au AK, Czyz C, Leahey A, Meadows AT, Shields JA (2006) The international classification of retinoblastoma predicts chemoreduction success. Ophthalmology 113:2276–2280
Zigiotti GL, Cavarretta S, Morara M, Nam SM, Ranno S, Pichi F, Lembo A, Lupo S, Nucci P, Meduri A (2012) Standard enucleation with aluminium oxide implant (bioceramic) covered with patient’s sclera. Sci World J 2012:481584. doi:10.1100/2012/481584
Karcioglu ZA, Hadjistilianou T (2004) Ocular tumors. In: Karcioglu ZA (ed) Orbital tumors—diagnosis and treatment. Springer, New York, pp 257–265
Knudson AG Jr (1974) Heredity and human cancer. Am J Pathol 77(1):77–84
DePotter P, Shields CL, Shields JA (1994) Clinical variations of trilateral retinoblastoma: a report of 13 cases. J Pediatr Ophthalmol Strabismus 31:26–31
Shields CL, Meadows AT, Shields JA et al (2001) Chemoreduction for retinoblastoma may prevent intracranial neuroblastic malignancy (trilateral retinoblastoma). Arch Ophthalmol 119:1269–1272
Moll AC, Imhof SM, Schouten-Van Meeteren AY et al (2003) Chemoreduction for retinoblastoma. Arch Ophthalmol 121:1513
Uusitalo MS, Van Quill KR, Scott IU et al (2001) Evaluation of chemoprophylaxis in patients with unilateral retinoblastoma with high-risk features on histopathologic examination. Arch Ophthalmol 119:41–48
Honavar SG, Singh AD, Shields CL et al (2002) Postenucleation adjuvant therapy in high-risk retinoblastoma. Arch Ophthalmol 120:923–931
Eagle RC Jr (2009) High-risk features and tumor differentiation in retinoblastoma: a retrospective histopathologic study. Arch Pathol Lab Med 133:1203–1209
Kingston JE, Hungerford JL, Madreperla SA, Plowman PN (1996) Results of combined chemotherapy and radiotherapy for advanced intraocular retinoblastoma. Arch Ophthalmol 114:1339–1343
Gallie BL, Budning A, DeBoer G et al (1996) Chemotherapy with focal therapy can cure intraocular retinoblastoma without radiotherapy. Arch Ophthalmol 114:1321–1328
Murphree AL, Villablanca JG, Deegan WF III et al (1996) Chemotherapy plus local treatment in the management of intraocular retinoblastoma. Arch Ophthalmol 114:1348–1356
Shields CL, De Potter P, Himelstein BP et al (1996) Chemoreduction in the initial management of intraocular retinoblastoma. Arch Ophthalmol 114:1330–1338
Shields CL, Shields JA (2002) Chemotherapy for retinoblastoma. Editor Med Ped Oncol 38:377–378
Shields CL, Shields JA (2004) Changing management of retinoblastoma. Clin Expiment Ophthalmol 32:345–346
Shields JA, Shields CL, Meadows AT (2005) Chemoreduction in the management of retinoblastoma. Am J Ophthalmol 140:505–506
Antoneli CB, Ribeiro KB, Rodriguez-Galindo C et al (2007) The addition of ifosfamide/etoposide to cisplatin/teniposide improves the survival of children with retinoblastoma and orbital involvement. J Pediatr Hematol Oncol 29(10):700–704
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Pichi, F., Lembo, A., De Luca, M. et al. Bilateral retinoblastoma: clinical presentation, management and treatment. Int Ophthalmol 33, 589–593 (2013). https://doi.org/10.1007/s10792-012-9703-5
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DOI: https://doi.org/10.1007/s10792-012-9703-5