Abstract
Retinoblastoma is a relatively uncommon tumor of childhood that arises from the retina and accounts for about 3% of the cancers occurring in children younger than 15 years. The treatment of retinoblastoma is complex and requires a multidisciplinary team that includes pediatric oncologists, ophthalmologists, ocular pathologists, geneticists, and radiation oncologists, all of whom have expertise in retinoblastoma management. This chapter includes a brief discussion of the presentation, classification, and pathologic features of the disease. Available treatment options are also discussed, including enucleation, chemoreduction, systemic or local chemotherapy, focal therapy with cryotherapy, laser photocoagulation, or plaque brachytherapy, and external-beam radiation therapy.
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsReferences
Reese AB, Ellsworth RM. The evaluation and current concept of retinoblastoma therapy. Trans Am Acad Ophthalmol Otolaryngol 1963;67:164–72.
Murphree AL. Intraocular retinoblastoma: the case for a new group classification. Ophthalmol Clin North Am 2005;18(1):41–53, viii.
Shields CL, Mashayekhi A, Au AK, et al. The International Classification of Retinoblastoma predicts chemoreduction success. Ophthalmology 2006;113(12):2276–80.
Dryja TP, Cavenee W, White R, et al. Homozygosity of chromosome 13 in retinoblastoma. N Engl J Med 1984;310:550–3.
Classon M, Harlow E. The retinoblastoma tumour suppressor in development and cancer. Nat Rev Cancer 2002;2:910–7.
Hagstrom SA, Dryja TP. Mitotic recombination map of 13cen-13q14 derived from an investigation of loss of heterozygosity in retinoblastomas. Proc Natl Acad Sci U S A 1999;96:2952–7.
Sherr CJ. Cancer cell cycles. Science 1996;274:1672–7.
Wong FL, Boice JD Jr, Abramson DH, et al. Cancer incidence after retinoblastoma: radiation dose and sarcoma risk. JAMA 1997;278:1262–7.
Rubenfeld M, Abramson DH, Ellsworth RM, et al. Unilateral vs. bilateral retinoblastoma: correlations between age at diagnosis and stage of ocular disease. Ophthalmology 1986;93:1016–9.
Noorani HZ, Khan HN, Gallie BL, et al. Cost comparison of molecular versus conventional screening of relatives at risk for retinoblastoma. Am J Hum Genet 1996;59:301–7.
Cohen JG, Dryja TP, Nicholls KB, et al. RB1 genetic testing as a clinical service: a follow-up study. Med Pediatr Oncol 2001;37:372–8.
McLean IW, Burnier M, Zimmerman L, et al. Tumors of the retina. In: McLean IW, Burnier MN, Zimmerman LE, Fa J, editors. Atlas of Tumor Pathology. Tumors of the Eye and Ocular Adnexa. Washington, DC: Armed Forces Institute of Pathology, 1994:100–35.
Hurwitz RL, Chévez-Barrios P, Chintagumpala M, et al. Retinoblastoma. In: Pizzo PA, Poplack D, editors. Principles and Practice of Pediatric Oncology, 6th edition. Philadelphia, PA: Lippincott-Raven, 2002:825–46.
Chévez-Barrios P, Eagle RC, Marbach P. Histological features and risks for metastatic spread of retinoblastoma. In: Singh AD, Damato B, Pe’er J, Murphree AL, Perry JD, editors. Clinical Ocular Oncology, first edition. London: Elsevier Saunders, 2007:468–76.
Karcioglu ZA, al Mesfer SA, Abboud E, et al. Workup for metastatic retinoblastoma. A review of 261 patients. Ophthalmology 1997;104:307–12.
Friedman DL, Himelstein B, Shields CL, et al. Chemoreduction and local ophthalmic therapy for intraocular retinoblastoma. J Clin Oncol 2000;18(1):12–7.
Brichard B, De Bruycker JJ, De Potter P, et al. Combined chemotherapy and local treatment in the management of intraocular retinoblastoma. Med Pediatr Oncol 2002;38(6):411–5.
Antoneli CB, Ribeiro KC, Steinhorst F, et al. Treatment of retinoblastoma patients with chemoreduction plus local therapy: experience of the AC Camargo Hospital, Brazil. J Pediatr Hematol Oncol 2006;28(6):342–5.
Rodriguez-Galindo C, Wilson MW, Haik BG, et al. Treatment of intraocular retinoblastoma with vincristine and carboplatin. J Clin Oncol 2003;21(10):2019–25.
Schiavetti A, Hadjistillianou T, Clerico A, et al. Conservative therapy in intraocular retinoblastoma: response/recurrence rate. J Pediatr Hematol Oncol 2005;27(1):3–6.
Zage PE, Reitman AJ, Seshadri R, et al. Outcomes of a two-drug chemotherapy regimen for intraocular retinoblastoma. Pediatr Blood Cancer 2008;50(3):567–72.
Williams PC, Hemer WD, Roman-Goldstein S, et al. Toxicity and efficacy of carboplatin and etoposide in conjunction with disruption of the blood-brain tumor barrier in the treatment of intracranial neoplasms. Neurosurgery 1995;37(1):17–27; discussion 27–8.
Chantada GL, Fandino AC, Raslawski EC, et al. Experience with chemoreduction and focal therapy for intraocular retinoblastoma in a developing country. Pediatr Blood Cancer 2005;44(5):455–60.
Hadjistilianou T, Mastrangelo D, De Francesco S, et al. Brief report: conservative treatment in unilateral retinoblastoma: a preliminary report. Med Pediatr Oncol 2002;38(6):439–41.
Shields CL, Shelil A, Cater J, et al. Development of new retinoblastomas after 6 cycles of chemoreduction for retinoblastoma in 162 eyes of 106 consecutive patients. Arch Ophthalmol 2003;121(11):1571–6.
Beck MN, Balmer A, Dessing C, et al. First-line chemotherapy with local treatment can prevent external-beam irradiation and enucleation in low-stage intraocular retinoblastoma. J Clin Oncol 2000;18(15):2881–7.
Levy C, Doz F, Quintana E, et al. Role of chemotherapy alone or in combination with hyperthermia in the primary treatment of intraocular retinoblastoma: preliminary results. Br J Ophthalmol 1998;82(10):1154–8.
Hungerford JL, Toma NM, Plowman PN, et al. External beam radiotherapy for retinoblastoma: I. Whole eye technique. Br J Ophthalmol 1995;79(2):109–11.
Shields CL, Shields JA, DePotter P, et al. The effect of chemoreduction on retinoblastoma-induced retinal detachment. J Pediatr Ophthalmol Strabismus 1997;34(3):165–9.
Wilson MW, Rodriguez-Galindo C, Haik BG, et al. Multiagent chemotherapy as neoadjuvant treatment for multifocal intraocular retinoblastoma. Ophthalmology 2001;108(11):2106–14; discussion 2114–5.
Abramson DH, Ellsworth RM, Rozakis GW. Cryotherapy for retinoblastoma. Arch Ophthalmol 1982;100(8):1253–6.
Shields JA, Shields CL, Donoso LA, et al. Current treatment of retinoblastoma. Trans Pa Acad Ophthalmol Otolaryngol 1989;41:818–22.
Shields CL, Shields JA, Cater J, et al. Plaque radiotherapy for retinoblastoma: long-term tumor control and treatment complications in 208 tumors. Ophthalmology 2001;108(11):2116–21.
Abramson DH, Schefler AC. Transpupillary thermotherapy as initial treatment for small intraocular retinoblastoma: technique and predictors of success. Ophthalmology 2004;111(5):984–91.
Lee SY, Kim DK, Cho JH, et al. Inhibitory effect of bevacizumab on the angiogenesis and growth of retinoblastoma. Arch Ophthalmol 2008;126(7):953–8.
Dalgard CL, Van Quill KR, O’Brien JM. Evaluation of the in vitro and in vivo antitumor activity of histone deacetylase inhibitors for the therapy of retinoblastoma. Clin Cancer Res 2008;14(10):3113–23.
Laurie NA, Donovan SL, Shih CS, et al. Inactivation of the p53 pathway in retinoblastoma. Nature 2006;444:61–6.
Wadhwa L, Hurwitz MY, Chevez-Barrios P, et al. Treatment of invasive retinoblastoma in a murine model using an oncolytic picornavirus. Cancer Res 2007;67(22):10653–6.
Kleinerman RA, Tucker MA, Tarone RE, et al. Risk of new cancers after radiotherapy in long-term survivors of retinoblastoma: an extended follow-up. J Clin Oncol 2005;23(10):2272–9.
Dunkel IJ, Aledo A, Kernan NA, et al. Successful treatment of metastatic retinoblastoma. Cancer 2000;89(10):2117–21.
Kremens B, Wieland R, Reinhard H, et al. High-dose chemotherapy with autologous stem cell rescue in children with retinoblastoma. Bone Marrow Transplant 2003;31(4):281–4.
Lee SH, Yoo KH, Sung KW, et al. Tandem high-dose chemotherapy and autologous stem cell rescue in children with bilateral advanced retinoblastoma. Bone Marrow Transplant 2008;42(6):385–91.
Matsubara H, Makimoto A, Higa T, et al. A multidisciplinary treatment strategy that includes high-dose chemotherapy for metastatic retinoblastoma without CNS involvement. Bone Marrow Transplant 2005;35(8):763–6.
Namouni F, Doz F, Tanguy ML, et al. High-dose chemotherapy with carboplatin, etoposide, and cyclophosphamide followed by a hematopoietic stem cell rescue in patients with high-risk retinoblastoma: a SFOP and SFGM study. Eur J Cancer 1997;33(14):2368–75.
Gombos DS, Hungerford JH, Abramson DH, et al. Secondary acute myelogenous leukemia in patients with retinoblastoma: is chemotherapy a factor? Ophthalmology 2007;114(7):1378–83.
Gunduz K, Shields CL, Shields JA, et al. The outcome of chemoreduction treatment in patients with Reese-Ellsworth group V retinoblastoma. Arch Ophthalmol 1998;116(12):1613–7.
Chevez-Barrios P, Hurwitz MY, Louie K, et al. Metastatic and nonmetastatic models of retinoblastoma. Am J Pathol 2000;157:1405–12.
Hurwitz MY, Marcus KT, Chevez-Barrios P, et al. Suicide gene therapy for treatment of retinoblastoma in a murine model. Hum Gene Ther 1999;10: 441–8.
Wadhwa L, Hurwitz MY, Chevez-Barrios P, et al. Treatment of invasive retinoblastoma in a murine model using an oncolytic picornavirus. Cancer Res 2007;67:10653–6.
Mallam JN, Hurwitz MY, Mahoney T, et al. Efficient gene transfer into retinal cells using adenoviral vectors: dependence on receptor expression. Invest Ophthalmol Vis Sci 2004;45:1680–7.
Chevez-Barrios P, Chintagumpala M, Mieler W, et al. Response of retinoblastoma with vitreous tumor seeding to adenovirus-mediated delivery of thymidine kinase followed by ganciclovir. J Clin Oncol 2005;23:7927–35.
Lee V, Hungerford JL, Bunce C, et al. Globe conserving treatment of the only eye in bilateral retinoblastoma. Br J Ophthalmol 2003;87(11):1374–80.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2010 Springer Science+Business Media, LLC
About this chapter
Cite this chapter
Ghisoli, M.L. et al. (2010). Multidisciplinary Management of Retinoblastoma: Diagnosis, Treatment, and Future Direction. In: Esmaeli, B. (eds) Ophthalmic Oncology. M.D. Anderson Solid Tumor Oncology Series, vol 6. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-0374-7_14
Download citation
DOI: https://doi.org/10.1007/978-1-4419-0374-7_14
Published:
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4419-0373-0
Online ISBN: 978-1-4419-0374-7
eBook Packages: MedicineMedicine (R0)