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Abstract
Retinoblastoma in children and uveal melanoma in adults can pose a serious threat to both vision and life. For many decades, enucleation was often the only option to treat these intraocular malignancies. For retinoblastoma, intra-arterial chemotherapy is often utilized as the primary treatment at advanced academic centers and has dramatically improved local tumor control and eye salvage rates. For uveal melanoma, both plaque brachytherapy and proton beam irradiation have served as widely utilized therapies with a local failure rate of approximately 1–10%, depending on the series. Major recent advancements have allowed for a better understanding of the genomics of uveal melanoma and the impact of certain mutations on metastatic susceptibility. Gene expression profile stratifies uveal melanomas into two classes: low-risk (class 1) and high-risk (class 2). A loss-of-function mutation of BAP1 is associated with a class 2 gene expression profile and therefore confers worse prognosis due to elevated risk of metastasis. On the other hand, gain-of-function mutations of EIF1AX and SF3B1 correspond to a gene expression profile of class 1A and class 1B and confer a better prognosis. Preferentially expressed antigen in melanoma (PRAME) is an antigen that increases metastatic susceptibility when expressed in uveal melanoma cells. In addition to plaque brachytherapy and proton beam irradiation, both of which have demonstrated superb clinical outcomes, scientists are actively investigating newer therapeutic modalities as either primary therapy or adjuvant treatment, including a novel nanoparticle therapy and immunotherapy.
Highlights
Retinoblastoma and uveal melanoma, albeit rare, are the most commonly observed intraocular malignancies in pediatric and adult populations, respectively
Retinoblastoma occurs during early childhood in 1 per 16,000 people worldwide[1], whereas uveal melanoma occurs on average in Caucasians in their fifties and sixties[2]
Gobin et al reported that eyes that received intra-arterial chemotherapy (IAC) as primary treatment had an ocular event-free survival rate of 81.7% after 2 years, which was significantly higher than the rate of 58.4% for the eyes that had undergone intravenous chemotherapy (IVC) or EBR prior to IAC27
Summary
Retinoblastoma and uveal melanoma, albeit rare, are the most commonly observed intraocular malignancies in pediatric and adult populations, respectively. There have been published studies that demonstrated the efficacy of iodine-125 plaque brachytherapy as salvage treatment for retinoblastoma after both IAC and IVC39,40. Treatment outcomes of plaque brachytherapy, including 5-year mortality and local recurrence rates (4%), are comparable to those of proton beam radiotherapy in recent publications[88,89] Both plaque and proton beam therapy are known to cause ocular complications, including cataracts, radiation retinopathy, and radiation optic neuropathy. When activated by a 589 nm laser, the particles selectively break down the tumor cell membrane without affecting adjacent tissues This treatment modality, if proven successful in clinical trials, has the potential to preserve much of the patient’s vision and could be groundbreaking in patients with small tumors that are close to critical ocular structures such as the optic nerve and the macula. The effect on rates of metastatic disease are still unknown
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