RB is a retinal developmental tumor and the most frequent, intraocular malignancy in chidlren.1,2 Though, others may have done so before, Dr. James Wardrop is, often credited for the first RB enucleations with curative, intent. However, only later did enucleation of early-stage, RB become the standard of care.3 Along with progress in, pathology and the advent of ophthalmoscopy, Virchow, and subsequently Flexner and Wintersteiner described, the tumor’s retinal origin and histopathologic characteristics., 4 However, it wasn’t until 1926 that the consensus, term “retinoblastoma” was accepted based on its cytological, origin from retinoblasts.5, The scientific advances and increased RB awareness in, the last few decades have resulted in early detection,, diagnosis, and protocol-based treatment. This, in turn,, improved RB patient survival and globe salvage, often, with the preservation of useful vision.6-8 However, there, exists a disparity in RB outcomes globally.9 In lower-resource, nations, birth rates and RB incidence are higher,, and it is made worse by a lack of access to RB care.10, Therefore, it is imperative to focus on raising awareness,, subspecialty eye cancer training, and thereby employment, of effective treatment strategies for children in, low- and middle-resource countries (see Chapter 9).8,11, In this chapter, we will discuss the epidemiology, clinical, presentation, and socioeconomic aspects of RB.
Chapter Keyword: retinoblastoma
Treatment of Retinoblastoma
The aims of RB treatment in order of priority are to save, life, the eye, and vision. Early detection, prompt treatment,, and advanced treatment modalities have improved, survival leading to increased interest in globe salvage., All patients should be initially staged using the 8th edition, AJCC TNMH staging of RB as shown in Table 33-1.1, Many centers use the International Intraocular RB Classification, (IIRC) in addition to the AJCC.2 Staging can help, determine the type of treatment required and has been, shown to accurately predict both mortality from metastatic, disease and globe salvage rates.3,4 Table 33-1 includes, a column showing how the IIRC classification compares, to AJCC staging, as many studies discussed in this chapter, use the older, less robust classification. In contrast to IIRC, and other RB classification systems, the 8th edition AJCC, RB staging system is the only comprehensive classification, that addresses intraocular, orbital, and metastatic RB,, predicts metastatic death and local treatment outcomes,, accounts for sporadic and germline RB, and has been periodically, updated with new medical evidence.3,4, Treatment modalities have evolved from external radiation, in the 1960s, to systemic chemotherapy with sequentially, aggressive local treatments (SALT) in the 1990s.5 The, last decade has witnessed a growing interest in therapies, where treatment is delivered through regional arteries or, directly into the globe. In high resource countries, where, patients present early and more treatment options are readily, available, there exists a 3%–5% risk of metastasis-related, mortality.6-8 In contrast, children with RB from middle- and, lower-resource countries have a 10.3-fold and 9.3 to 10-fold, higher risk of metastasis-related mortality, respectively.9
International Outreach: Improving Global Retinoblastoma Outcomes
RB is the most common primary intraocular malignancy of childhood, and > 8,000 children are diagnosed each year worldwide.1,2 In high-resource settings with infra¬structure and support, the survival of children with RB can be nearly 100%.3,4 In contrast, in low- and middle-re¬source countries, eye cancer specialists and support are lacking, leading to survival rates approximating 10%.3-5 In that more than 80% of the children diagnosed with pedi¬atric cancer live in low- and middle-resource countries, many more children are dying of RB than surviving.6-11 Given this disparity, it is important to identify what can be done to save the lives of RB children in low- and mid¬dle-resource countries.
That said, the World Health Organization (WHO) has identified RB as one of the most curable pediatric index cancers, stating a goal of at least 60% survival by 2030.12 This will require cooperation between ophthalmic and pediatric oncology. Governments and nongovernmental organizations will need to work towards allocating highly specific resources, education/training, and infrastruc¬ture. Thus, RB can serve as a model for other pediatric cancers by implementing effective education, training, and capacity-building initiatives that are scalable and adaptable. Many strategies are already known to be effec¬tive: multidisciplinary care teams, well-defined referral networks, resource-adapted treatment guidelines, as well as early programs to promote early detection and awareness.