The Indian Journal of Pediatrics

, Volume 84, Issue 12, pp 937–944 | Cite as


  • Raksha Rao
  • Santosh G. Honavar
Review Article


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.


Eye Retina Malignant tumor Retinoblastoma Chemotherapy Radiotherapy 



RR: Drafting of the manuscript and figure compilation; SGH: Concept, critical revision and will act as guarantor for the paper.

Compliance with Ethical Standards

Conflict of Interest


Source of Funding



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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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