Retinoblastoma

Review Article

Abstract

Retinoblastoma represents 3% of all childhood cancers, and is the most common intraocular malignancy of childhood. It is fatal, if untreated. White eye reflex, also known as leukocoria, is the commonest sign, followed by strabismus. The pediatricians have a very important role to play in the diagnosis of this relatively rare, but easily detectable tumor. Early diagnosis yields better results. The management of retinoblastoma has gradually evolved over the past few decades, with an aim to not only preserve life and eye, but also optimize residual vision. The treatment of retinoblastoma is multimodal, with chemotherapy, focal treatment including trans-pupillary thermotherapy, cryotherapy and laser photocoagulation, radiation therapy and surgery, all playing a vital role. Intravenous chemotherapy has been the mainstay of treatment for the past two decades, and still continues to be the most extensively used eye-saving modality of treatment. Periocular and intravitreal chemotherapy have specific indications in the management of retinoblastoma. Intra-arterial chemotherapy has emerged as a promising alternative for advanced and refractory retinoblastoma, both as a primary and secondary therapy. Recent advances in genetics of retinoblastoma have also helped in improving the overall clinical management of this malignancy.

Keywords

Eye Retina Malignant tumor Retinoblastoma Chemotherapy Radiotherapy 

References

  1. 1.
    Albert DM. Historic review of retinoblastoma. Ophthalmology. 1987;94:654–62.CrossRefPubMedGoogle Scholar
  2. 2.
    Shields J, Shields C. Retinoblastoma: introduction, genetics, clinical features, classification. In: Shields J, Shields C, editors. Atlas of Intraocular Tumors, 3rd ed. Philadelphia: Lippinocott, Wolters Kluwer; 2016. p. 311–4.Google Scholar
  3. 3.
    Satyanarayana L, Asthana S, Labani SP. Childhood cancer incidence in India: a review of population-based cancer registries. Indian Pediatr. 2014;51:218–20.CrossRefPubMedGoogle Scholar
  4. 4.
    Nichols KE, Walther S, Chao E, Shields C, Ganguly A. Recent advances in retinoblastoma genetic research. Curr Opin Ophthalmol. 2009;20:351–5.CrossRefPubMedGoogle Scholar
  5. 5.
    Jijelava KP, Grossniklaus HE. Diffuse anterior retinoblastoma: a review. Saudi J Ophthalmol. 2013;27:135–9.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Shields CL, Schoenberg E, Kocher K, et al. Lesions simulating retinoblastoma (pseudoretinoblastoma) in 604 cases: results based on age at presentation. Ophthalmology. 2013;120:311–6.CrossRefPubMedGoogle Scholar
  7. 7.
    Rootman DB, Gonzalez E, Mallipatna A, et al. Hand-held high-resolution spectral domain optical coherence tomography in retinoblastoma: clinical and morphologic considerations. Br J Ophthalmol. 2013;97:59–65.CrossRefPubMedGoogle Scholar
  8. 8.
    Shields J, Shields C. Retinoblastoma: diagnostic approaches. In: Shields J, Shields C, editors. Atlas of Intraocular Tumors, 3rd ed. Philadelphia: Lippinoctt, Wolters Kluwer; 2016. p. 335–42.Google Scholar
  9. 9.
    Chantada G, Doz F, Antoneli CB, et al. A proposal for an international retinoblastoma staging system. Pediatr Blood Cancer. 2006;47:801–5.CrossRefPubMedGoogle Scholar
  10. 10.
    Shields CL, Mashayekhi A, Au AK, et al. The international classification of retinoblastoma predicts chemoreduction success. Ophthalmology. 2006;113:2276–80.CrossRefPubMedGoogle Scholar
  11. 11.
    Shields CL, Fulco EM, Arias JD, et al. Retinoblastoma frontiers with intravenous, intra-arterial, periocular, and intravitreal chemotherapy. Eye. 2012;27:253–64.CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Shields CL, Shields JA. Retinoblastoma management: advances in enucleation, intravenous chemoreduction, and intra-arterial chemotherapy. Curr Opin Ophthalmol. 2010;21:203–12.CrossRefPubMedGoogle Scholar
  13. 13.
    Kingston JE, Hungerford JL, Madreperla SA, Plowman PN. Results of combined chemotherapy and radiotherapy for advanced intraocular retinoblastoma. Arch Ophthalmol. 1996;114:1339–43.CrossRefPubMedGoogle Scholar
  14. 14.
    Manjandavida FP, Honavar SG, Reddy V, Khanna R. Management and outcome of retinoblastoma with vitreous seeds. Ophthalmology. 2014;121:517–24.CrossRefPubMedGoogle Scholar
  15. 15.
    Suzuki S, Yamane T, Mohri M, Kaneko A. Selective ophthalmic arterial injection therapy for intraocular retinoblastoma: the long-term prognosis. Ophthalmology. 2011;118:2081–7.CrossRefPubMedGoogle Scholar
  16. 16.
    Abramson DH, Marr BP, Dunkel IJ, et al. Intra-arterial chemotherapy for retinoblastoma in eyes with vitreous and/or subretinal seeding: 2-year results. Br J Ophthalmol. 2012;96:499–502.CrossRefPubMedGoogle Scholar
  17. 17.
    Shields CL, Manjandavida FP, Lally SE. Intra-arterial chemotherapy for retinoblastoma in 70 eyes: outcomes based on the international classification of retinoblastoma. Ophthalmology. 2014;121:1453–60.CrossRefPubMedGoogle Scholar
  18. 18.
    Shields CL, Jorge R, Say EA, et al. Unilateral retinoblastoma managed with intravenous chemotherapy versus intra-arterial chemotherapy: outcomes based on the international classification of retinoblastoma. Asia Pac J Ophthalmol. 2016;5:97–103.CrossRefGoogle Scholar
  19. 19.
    Shields CL, Kaliki S, Al-Dahmash S, et al. Management of advanced retinoblastoma with intravenous chemotherapy then intra-arterial chemotherapy as alternative to enucleation. Retina. 2013;33:2103–9.CrossRefPubMedGoogle Scholar
  20. 20.
    Munier FL, Gaillard MC, Balmer A, et al. Intravitreal chemotherapy for vitreous disease in retinoblastoma revisited: from prohibition to conditional indications. Br J Ophthalmol. 2012;96:1078–83.CrossRefPubMedGoogle Scholar
  21. 21.
    Munier FL, Soliman S, Moulin AP, Gaillard MC, Balmer A, Beck-Popovic M. Profiling safety of intravitreal injections for retinoblastoma using an anti-reflux procedure and sterilisation of the needle track. Br J Ophthalmol. 2012;96:1084–7.CrossRefPubMedGoogle Scholar
  22. 22.
    Ghassemi F, Shields CL. Intravitreal melphalan for refractory or recurrent vitreous seeding from retinoblastoma. Arch Ophthalmol. 2012;130:1268–71.CrossRefPubMedGoogle Scholar
  23. 23.
    Francis JH, Abramson DH, Gaillard MC, Marr BP, Beck-Popovic M, Munier FL. The classification of vitreous seeds in retinoblastoma and response to intravitreal melphalan. Ophthalmology. 2015;122:1173–9.CrossRefPubMedGoogle Scholar
  24. 24.
    Shields CL, Manjandavida FP, Arepalli S, et al. Intravitreal melphalan for persistent or recurrent retinoblastoma vitreous seeds: preliminary results. JAMA Ophthalmol. 2014;132:319–25.CrossRefPubMedGoogle Scholar
  25. 25.
    Shields CL, Shields JA, Cater J, et al. Plaque radiotherapy for retinoblastoma: long-term tumor control and treatment complications in 208 tumors. Ophthalmology. 2001;108:2116–21.CrossRefPubMedGoogle Scholar
  26. 26.
    Shields CL, Honavar SG, Meadows AT, Shields JA, Demirci H, Naduvilath TJ. Chemoreduction for unilateral retinoblastoma. Arch Ophthalmol. 2002;120:1653–8.CrossRefPubMedGoogle Scholar
  27. 27.
    Shields CL, Honavar SG, Meadows AT, et al. Chemoreduction plus focal therapy for retinoblastoma: factors predictive of need for treatment with external beam radiotherapy or enucleation. Am J Ophthalmol. 2002;133:657–64.CrossRefPubMedGoogle Scholar
  28. 28.
    Murphree AL, Villablanca JG, Deegan WF 3rd, et al. Chemotherapy plus local treatment in the management of intraocular retinoblastoma. Arch Ophthalmol. 1996;114:1348–56.CrossRefPubMedGoogle Scholar
  29. 29.
    Honavar SG, Singh AD, Shields CL, Demirci H, Smith AF, Shields JA. Post-enucleation prophylactic chemotherapy in high-risk retinoblastoma. Arch Ophthalmol. 2002;120:923–31.CrossRefPubMedGoogle Scholar
  30. 30.
    Honavar SG. Orbital retinoblastoma. In: Singh AD, Murphee LA, Damato BE, editors. Clinical ophthalmic Oncology-retinoblastoma. 2nd ed. NY: Springer; 2015. p. 185–94.Google Scholar
  31. 31.
    Dunkel IJ, Khakoo Y, Kernan NA, et al. Intensive multimodality therapy for patients with stage 4a metastatic retinoblastoma. Pediatr Blood Cancer. 2010;55:55–9.PubMedGoogle Scholar
  32. 32.
    Rao R, Honavar SG. Recent developments in retinoblastoma. Delhi J Ophthalmol. 2016;27:50–61.CrossRefGoogle Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 2017

Authors and Affiliations

  1. 1.Orbit, Oculoplasty and Ocular OncologyChaithanya Eye Hospital and Research InstituteTrivandrumIndia
  2. 2.Ocular Oncology Service, National Retinoblastoma FoundationCentre for SightHyderabadIndia

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