Challenging the global retinoblastoma survival disparity through a collaborative research effort

Dimaras, Helen; Dimba, Elizabeth A O; Gallie, Brenda L.

doi:10.1136/bjo.2009.174136

Cited by 54 publications

(44 citation statements)

References 21 publications

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“…Nyamori et al [13] found that in Kenya, even patients with a family history of retinoblastoma had a delayed presentation. Late presentation in developing countries is thought to be a result of poor awareness of retinoblastoma by both parents and healthcare professionals, difficulties accessing healthcare in remote areas, cultural beliefs, and regional differences in attitudes towards disease [1,6,17,18]. Patients are frequently diagnosed at a stage when extraocular spread of the disease has occurred, and thus, as in other African studies, proptosis was a common presentation in our population [2,6,11,14,19].…”

Section: Discussionmentioning

confidence: 99%

A 20-Year Retrospective Review of Retinoblastoma at Two Tertiary Academic Hospitals in Johannesburg, South Africa

et al. 2017

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Aim: This paper presents a 20-year review of retinoblastoma in Johannesburg, South Africa, aiming to better characterize the disease in this sub-Saharan setting. Methods: The study represents a retrospective case series of retinoblastoma patients presenting to Charlotte Maxeke Johannesburg Academic Hospital and Chris Hani Baragwanath Academic Hospital between January 1, 1992, and December 31, 2011. Results: The total number of cases identified was 282, with 245 meeting the study inclusion criteria. Retinoblastoma comprised 6.9% of the total pediatric oncology presentations; 65.3% were unilateral, 34.3% bilateral, and 0.4% trilateral. The overall male-to-female ratio was 1.08. The mean age at presentation overall was 32.6 months (median 28.0), in the unilateral group 39.4 months (median 33.0), and in the bilateral group 19.7 months (median 17.0). The mean delay to presentation overall was 7.0 months (median 4.0). The most frequent presenting symptoms were leukocoria (37.1%) and proptosis (34.7%). The distribution of disease stages at presentation (International Retinoblastoma Staging System) was 1.6% stage 0, 24.1% stage I, 27.8% stage II, 16.3% stage III, and 25.3% stage IV. 26.5% defaulted care. The 5-year Kaplan-Meier survival estimate was 57.7% overall. Conclusion: This study shows that delayed presentation and refusal of therapy remains a significant barrier to effective treatment in this African setting.

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Section: Discussionmentioning

confidence: 99%

A 20-Year Retrospective Review of Retinoblastoma at Two Tertiary Academic Hospitals in Johannesburg, South Africa

et al. 2017

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“…14,20,21 This has been attributed to a delay in diagnosis or presentation, absence of specialized centers, or a difference in biologic behavior of the tumor. 2,[6][7][8]15 Owing to the varied criteria used in different series, it is difficult to compare individual HRFs. The overall reported incidence of retrolaminar optic nerve involvement varies a great deal, from 7% to 30%.…”

Section: Commentmentioning

confidence: 99%

“…[2][3][4][5] This has been attributed to delay in presentation and diagnosis, lack of specialized centers, and/or differences in biologic behavior of the tumors. [6][7][8] Herein, we report the results of a retrospective histopathologic analysis conducted on a large series of eyes primarily enucleated for advanced intraocular retinoblastoma at a major tertiary referral hospital. The purpose of this study was to evaluate the frequency of HRFs in Indian children enucleated for advanced intraocular retinoblastoma and to analyze the relationship between tumor differentiation, age at diagnosis, and HRFs.…”

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confidence: 99%

A Histopathologic Analysis of Eyes Primarily Enucleated for Advanced Intraocular Retinoblastoma From a Developing Country

Kashyap¹,

Sethi²,

Meel³

et al. 2012

Archives of Pathology & Laboratory Medicine

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N Context.-In eyes enucleated for retinoblastoma, presence of histopathologic high-risk factors is associated with a higher risk of local recurrence and systemic metastasis.Objective.-To evaluate histopathologic features in children with retinoblastoma in our population and establish relationship between age, tumor differentiation, and high-risk features.Design.-Retrospective histopathologic analysis of 609 consecutively enucleated eyes for advanced intraocular retinoblastoma during a 10-year period. A nonparametric test was used to establish relationship between age, differentiation, and high-risk features.Results.-Poorly differentiated retinoblastoma presented in 80.3% and well-differentiated in 19.7% of eyes. Welldifferentiated tumors presented earlier (median 1.2 years) than poorly differentiated tumors (median 2.5 years) (P , .001). One hundred fourteen eyes (18.7%) had 1 and 138 (22.7%) had at least 2 high-risk histopathologic factors. Invasion of anterior chamber was found in 10.0%, iris in 10.7%, ciliary body in 6.7%, sclera in 13.7%, massive choroid in 24.6%, postlaminar optic nerve in 16.1%, resected margin of the optic nerve in 7.4%, and extrascleral tissue in 4.1% of eyes. Extensive necrosis was seen in 31.0% of eyes. Poorly differentiated tumors were significantly associated with presence of more than 1 highrisk histopathologic feature (P , .001) and extensive necrosis (P , .001).Conclusion.-Poorly differentiated tumors present at a later age and are associated with presence of multiple high-risk factors and extensive necrosis. In our population, high-risk histopathologic factors are present in a significant number of eyes. Because we have included only primarily enucleated eyes, this could truly represent the distribution of high-risk histopathologic factors in children with retinoblastoma. (Arch Pathol Lab Med. 2012;136:190-193; doi: 10.5858/ arpa.2010-0759-OA) R etinoblastoma is the most frequent primary malignant intraocular tumor in children and has an estimated annual incidence of 1 in 16 000 to 1 in 18 000 live births. 1With the present-day multidisciplinary therapeutic approach, ocular salvage is possible in group A through D intraocular tumors (International Retinoblastoma Classification). However, in group E tumors (and group D in unilateral cases), enucleation remains the modality of choice. Some histopathologic findings in enucleated eyes, designated as high-risk factors (HRFs), are associated with a greater risk of orbital recurrence and distant metastasis and need adjuvant therapy.There are few studies that have evaluated HRFs in eyes enucleated for advanced intraocular retinoblastoma from developing countries, and most of them have reported higher incidences compared with studies reported in the Western literature. [2][3][4][5] This has been attributed to delay in presentation and diagnosis, lack of specialized centers, and/or differences in biologic behavior of the tumors. 6-8Herein, we report the results of a retrospective histopathologic analysis conducted on a large series of ...

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“…The group's process is dynamic and iterative, striving to create an evidence base where there is none. The approach has been research based since the beginning, with members actively applying for and acquiring research funds to develop and test various initiatives [22,23]. Working closely with partners in civil society organizations and the public sector, we are engaging decision makers throughout the research process, so that lessons learned can be formalized within the existing healthcare channels in the long-term.…”

Section: The Way Forwardmentioning

confidence: 99%

Developing Clinical Cancer Genetics Services in Resource-Limited Countries: The Case of Retinoblastoma in Kenya

He¹,

Njambi

Nyamori

et al. 2014

Public Health Genomics

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Background/Aims: Clinical cancer genetics is an integral part of cancer control and management, yet its development as an essential medical service has been hindered in many low-and-middle-income countries. We report our experiences in developing a clinical cancer genetics service for retinoblastoma in Kenya. Methods: A genetics task force was created from within the membership of the existing Kenyan National Retinoblastoma Strategy group. The task force engaged in multiple in-person and telephone discussions, delineating experiences, opinions and suggestions for an evidence-based, culturally sensitive retinoblastoma genetics service. Discussions were recorded and thematically categorized to develop a strategy for the design and implementation of a national retinoblastoma clinical genetics service. Results: Discussion among the retinoblastoma genetics task force supported the development of a comprehensive genetics service that rests on 3 pillars: (1) patient and family counseling, (2) community involvement, and (3) medical education. Conclusions: A coordinated national retinoblastoma genetics task force led to the creation of a unique and relevant approach to delivering comprehensive and accurate genetic care to Kenyan retinoblastoma patients. The task force aims to stimulate innovative approaches in cancer genetics research, education and knowledge translation, taking advantage of unique opportunities offered in the African context.

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Challenging the global retinoblastoma survival disparity through a collaborative research effort

Cited by 54 publications

References 21 publications

A 20-Year Retrospective Review of Retinoblastoma at Two Tertiary Academic Hospitals in Johannesburg, South Africa

A 20-Year Retrospective Review of Retinoblastoma at Two Tertiary Academic Hospitals in Johannesburg, South Africa

A Histopathologic Analysis of Eyes Primarily Enucleated for Advanced Intraocular Retinoblastoma From a Developing Country

Developing Clinical Cancer Genetics Services in Resource-Limited Countries: The Case of Retinoblastoma in Kenya

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