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Advancing the Treatments of Retinoblastoma: Stuck in the 1950s
Author(s) -
Matthew W. Wilson
Publication year - 2016
Publication title -
ocular oncology and pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.444
H-Index - 10
eISSN - 2296-4681
pISSN - 2296-4657
DOI - 10.1159/000453361
Subject(s) - medicine , retinoblastoma , genetics , biology , gene
and involvement of the trabecular meshwork for 270 degrees. The visual acuity was “normal OU”. Initial treatment consisted of a multitier approach using intra-arterial, intravitreal, and intracameral melphalan. This was followed by 4 monthly cycles of neoadjuvant systemic carboplatin and etoposide. An anterior chamber recurrence was noted 3.5 months later and further intracameral and intravitreal melphalan were delivered. Reported complications included heterochromia and opacification of the lens cortex, necessitating cataract surgery. Endothelial cell density remained stable during therapy; however, following cataract surgery, a 10% endothelial cell loss was noted. At 5 years of follow-up, the child was disease free with 20/20 vision in both eyes. The second patient had bilateral retinoblastoma (ICRB Group D right eye and Group E left eye) diagnosed at 1 year of age. The left eye had been enucleated, and the right eye developed ciliary body and anterior chamber relapse. Intraophthalmic artery chemotherapy was ineffective, so in hopes of avoiding external radiation, the authors elected to couple 4 alternating courses of intravitreal and intracameral melphalan with a seventh cycle of systemic chemotherapy. At 3 years of follow-up, the patient had no recurrent disease and “useful vision” despite documented atrophic choroidal scarring and retinal vascular toxicity. With advancing age and wisdom, we are often apt to romance the simpler time of our youth. The world was less complicated. We were not hyperstimulated in an era devoid of email, cell phones, and texts. However, survival rates from pediatric cancers, notably retinoblastoma, were much lower. There was no Knudson two-hit hypothesis, RB1 gene, patient-derived orthotopic xenografts, or whole-genome sequencing. However, there was external beam radiation [1] , intra-arterial therapy [2] , intravitreal therapy [3] , and melphalan [4] . Fast-forward to the present, where advances in science and medicine are improving health care, but with the background force of social media. Our challenge as clinicians and scientists is to assure that science and medicine are kept in parallel with bench-to-bedside research as a feedback loop. The medical treatment we bring to our patients today must be thoroughly evaluated using the research tools that have been made available. In this issue of Ocular Oncology and Pathology , we are presented with 2 separate case reports, each documenting the novel use of intracameral melphalan for the treatment of anterior chamber seeding from advanced intraocular retinoblastoma [5, 6] . The first patient was an 11-year-old with unilateral International Classification of Retinoblastoma (ICRB) Group E eye with anterior chamber seeding Received: November 7, 2016 Accepted: November 10, 2016 Published online: December 24, 2016

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