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Abstract
Retinoblastoma and uveal melanoma are the most common intraocular malignancies observed in pediatric and adult populations, respectively. For retinoblastoma, intra-arterial chemotherapy has dramatically improved treatment outcomes and eye salvage rates compared with traditional salvage rates of systemic chemotherapy and external beam radiation therapy. Intravitreal injections of chemotherapy have also demonstrated excellent efficacy for vitreous seeds. Uveal melanoma, on the other hand, is treated predominantly with iodine-125 plaque brachytherapy or with proton beam therapy. Major strides in uveal melanoma genomics have been made since the early 2000s, allowing ocular oncologists to better understand the metastatic risks of the tumor on the basis of specific genetic signatures. Loss-of-function mutations of the BAP1 gene are associated with the highest metastatic risk, whereas gain-of-function mutations of SF3B1 and EIF1AX often confer a better prognosis. Expression of a cancer-testis antigen called PRAME (preferentially expressed antigen in melanoma) has been shown to increase metastatic risks in both low-risk and high-risk melanomas. New therapeutic approaches, including molecular therapies and nanoparticle phototherapy, are currently being investigated as alternative treatment modalities for uveal melanoma.
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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