Rare tumors: an eye on the future

Cancers of the eye are rare, but have become the paradigm for the study of rare malignancies. Retinoblastoma, the commonest intraocular malignancy of childhood occurs in one in 18,000 live births. It was one of the first cancers to be assigned a genetic basis, with Knudson’s double hit hypothesis [1]. Whereas treatment was once limited to radiotherapy and/or surgical eye removal (enucleation), advances in chemotherapy have changed the treatment and prognosis of this tumor [2]. Systemic chemotherapy supplanted other modalities in the 1980s and 1990s, with impressive results for preservation of life, globe and vision retention. Often local consolidation with direct laser or cryotherapy was undertaken to prevent recurrence [3]. Further advances occurred to try to save eyes that were otherwise destined for enucleation with chemotherapy infusions directly into the ophthalmic artery (intra-arterial chemotherapy) initially of melphalan but now also with topotecan [4]. Though there are risks associated with this [5], vision can be spared in many cases [6].

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