Recent advancements in the management of retinoblastoma and uveal melanoma

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.

Piperlongumine promotes death of retinoblastoma cancer cells

“…Although the survival rate of patients with retinoblastoma is extremely high in developed countries, left untreated advanced tumors limit eye preservation and expose patients to risks of metastasis and death. Therefore, depending on the disease stage, the therapeutic approaches for retinoblastoma treatment have to be adjusted, including enucleation, intravenous or intraarterial chemotherapy, or local treatments such as laser therapy, cryotherapy, and radiation [2]. Renuméroter les références.…”

Recent advancements in the management of retinoblastoma and uveal melanoma

“…The patient underwent three IAC cycles and adjuvant therapy, including five sessions of laser ablation and two sessions of cryotherapy. This figure was reused from Schefler A and Kim R 49 under the terms of CC BY 4.0 ( https://creativecommons.org/licenses/by/4.0/ ).…”

Rare tumors: Retinoblastoma, nasopharyngeal cancer, and adrenocorticoid tumors

“…45 In a 2013 systematic review of 14 studies of > 1300 intravitreal injections, only one case of extraocular tumor spread was seen. 46 Intravitreal chemotherapy has been widely adopted for refractory or recurrent vitreal seeding after IAC or IVC 45 and provides nearly 100% two-year control in this setting. 44,47 IVC frequently was used with focal therapies to avoid EBRT and enucleation in the 1990s until the development of IAC in the 2000s.…”