The management of retinoblastoma

Abstract

Retinoblastoma (Rb) is the most common primary intraocular malignancy of childhood, but an uncommon paediatric cancer, with a constant incidence worldwide of 1:15,000-1:20,000 live births. Despite its rarity, Rb has served as a cornerstone in the field of oncology in many of the aspects that comprise cancer management, including classification schemes, treatment modalities, genetic testing and screening. Until just over half a century ago, the major treatment for Rb was eye removal, and prognosis was poor with outcome fatal for most children. The dramatic evolution, in a short period of time across all fields of Rb management, as well as the development of specialized centres, better infrastructure and introduction of awareness campaigns, has resulted in nearly 100% survival in developed countries and allowed eye salvage in many of the cases. External beam radiotherapy was used as the main treatment choice for four decades, but replaced by chemotherapy at the turn of the century. Initially, and still in many centres, chemotherapy is administered intravenously, but recently is targeted directly into the eye by means of intra-ophthalmic artery and intravitreal chemotherapy. To date, a range of treatments is available to the Rb expert, including enucleation, but there is lack of consensus in a number of scenarios as to what to use and when. In such a rare cancer, treatment outcomes are reported usually via retrospective analyses, with few prospective randomized controlled trials. Classification schemes have also evolved following the introduction of new treatment modalities, but discrepancies exist among centres with respect to the preferred schema and its interpretation. Retinoblastoma management is a remarkable success story, but the future will require a collaborative effort in the form of multicentre randomized controlled trials in order to further improve the quality of care for this subset of young children with ocular cancer.

Associations among Socioeconomic Factors, Lag Time, and High-Risk Histopathologic Features in Eyes Primarily Enucleated for Retinoblastoma

“…1,2 Despite great progresses in ocularsalvage treatment in the past decades, enucleation remains the only option to save the lives for children who present with advanced disease. 3 High-risk histopathologic features, representing extensive invasion of tumor cells, facilitate in predicting metastasis and mortality. For children with high-risk findings, postoperative adjuvant systemic chemotherapy is frequently administered to reduce the risk of metastasis.…”

Long noncoding RNA UCA1 facilitates cell proliferation and inhibits apoptosis via activating PI3K/Akt pathway in retinoblastoma

“…[18] In developing countries, the survival rate of RB patients is still below 70% . [19] It is urgent to investigate new and effective molecular targets for the treatment of RB. [20,21] In this study, we confirmed that lncRNA-UCA1 could facilitate cell proliferation and inhibit apoptosis through activating PI3K/Akt pathway in RB.…”

Long noncoding RNA UCA1 facilitates cell proliferation and inhibits apoptosis via activating PI3K/Akt pathway in retinoblastoma

“…18 In developing countries, the survival rate of RB patients is still below 70%. 19 It is urgent to investigate new and effective molecular targets for the treatment of RB. In this study, 16 ovarian cancer 22 and lung cancer.…”