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Retinoblastoma: ophthalmic problems in survivors. Still a challenge?
  1. George F von Arx,
  2. Nicole Fichter
  1. Ophthalmic Plastic and Orbital Surgeons, ADMEDICO Eye Care and Orbital Centre, Faehrweg 10, CH-4600 Olten, Switzerland
  1. Correspondence to Dr George F von Arx, Faehrweg 10, CH-4600 Olten, Switzerland; georg.vonarx{at}

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Nowadays, retinoblastoma is a curable cancer if it is treated appropriately and if it is detected as early as possible, while still limited to the globe and without optic nerve, choroid and scleral invasion.1 2 In the editorial of the Br J Ophthalmol 2010 April issue, Abramson3 presented a synopsis of the non-surgical treatment modalities with encouraging results of super selective ophthalmic artery delivery of chemotherapy for intraocular retinoblastoma. In the same issue, Boubacar et al4 reported on combined treatment modalities according to their 30-month prospective study on retinoblastoma in Mali, Africa. They conclude that mortality as a result of retinoblastoma remains high in their region owing to late diagnosis and difficulties in following the patients. Hence, the chapters are not yet closed on this important subject. In this edition of Br J Ophthalmol, Lin et al5 report on the orbital development in survivors of retinoblastoma treated by enucleation and volume replacement by hydroxyapatite implant.

Early in 1809, Wardrop6 established retinoblastoma as an entity and proposed enucleation as its primary treatment. Virchow7 considered the tumour to be a glioma of the retina, whereas Flexner8 in 1881 proposed the term ‘neuroepithelioma of the retina’ based on the characteristic rosettes on histological evaluation. Many more names have been suggested ever since, until Verhoeff9 suggested ‘retinoblastoma’ in 1922. This name was adopted consecutively by the American Ophthalmic Society and the WHO.10–15

Retinoblastoma is the most common primary intraocular tumour of early childhood, arising from primitive embryonal cells, either photoreceptors or neuronal cell types of the sensory retina (tables 1, 2). Accordingly it may either display evidence of photoreceptor differentiation or show areas of glial differentiation. Retinoblastoma primarily presents as intraocular malignant tumour but also spreads through the sclera into the orbit and …

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