Increased incidence and disparity of diagnosis of retinoblastoma patients in Guatemala

Dean, Michael; Bendfeldt, Giovana; Li, Hong; Girón, Verónica; Garrido, Clàudia; Valverde, Patricia; Barnoya, Margarita; Castellanos, Mauricio; Jiménez-Morales, Silvia; Luna‐Fineman, Sandra

doi:10.1016/j.canlet.2014.04.023

Cited by 26 publications

(20 citation statements)

References 17 publications

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“…It would be important to further investigate this variation in a separate paper to establish global consistency. Another recent paper published from the Asia‐Pacific region uses the same estimates as described in this paper and were found to correlate well with the data from the National Registry of Retinoblastoma in Japan …”

Section: Discussionsupporting

confidence: 68%

“…Another recent paper published from the Asia-Pacific region uses the same estimates as described in this paper and were found to correlate well with the data from the National Registry of Retinoblastoma in Japan. 12,28,29 Using the estimates for Nigeria, a total of 431 cases are predicted each year. ( Supplementary Table S2 This tool primarily estimates for annual cases and does not take into full account the accumulation of cases, therefore underestimating the services required.…”

Section: Discussionmentioning

confidence: 99%

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A tool for planning retinoblastoma services in sub-Saharan Africa

Hampejsková

Bascarán

Zondervan

2016

Pediatr Blood Cancer

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Background: Cancer care remains inadequate in low-middle income countries (LMICs). Children with cancer have 80% chance of surviving in high-income countries compared to 20% in LMICs.Retinoblastoma (RB), an aggressive eye cancer of childhood and top childhood cancer in subSaharan Africa (SSA), has a low survival rate, due to a delay in diagnosis and abandonment of treatment. The purpose of this study is to provide a tool for planning human resources required to manage RB in SSA.Procedure: Online tool was developed with 19 modifiable fields and 23 estimates. Routine data were used to populate modifiable fields: population, birthrate, infant mortality rate, and total fertility rate. Values were held constant: frequency, 1:17,000; familial cases, 8%; unilateral RB, 74%; extraocular disease, 70%; and survival postextraocular treatment, 10%. Results:One thousand twenty-three RB incident and familial cases are estimated each year across Ethiopia, Ghana, Malawi, Nigeria, Tanzania, Uganda, Zambia, and Zimbabwe: 75 familial, 700 unilateral, 717 extraocular disease, and 645 palliative. Nigeria represents 431 cases and Zimbabwe 33 cases. Over the eight countries, a total of 41,558 patient visits are estimated each year consisting of unilateral enucleation, follow-up visits, intensive treatment, and familial screening, with a total of 2,802 prosthetic eyes being required each year. Conclusions:In the absence of data, estimates are essential for planning countrywide medical services. More attention is needed around planning for services from the Ministry level including emphasis on building multidisciplinary teams for diseases such as RB, including countrywide database and integrated clinical practice guidelines among all levels of care.

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Section: Discussionsupporting

confidence: 68%

Section: Discussionmentioning

confidence: 99%

A tool for planning retinoblastoma services in sub-Saharan Africa

Hampejsková

Bascarán

Zondervan

2016

Pediatr Blood Cancer

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“…12 Although prevalence is higher in middle- to low-income countries (Figure 2), most retinoblastoma treatment centres are in middle- and high-income countries, creating a gap in healthcare access (Table 2). Consistent with income being a surrogate for non-economic measures of standard of living, retinoblastoma in low-income countries is associated with low patient survival (~30% 13,14 ) compared with high-income countries (>95% 8 ), but comprehensive nation-wide data are lacking.…”

Section: Epidemiologymentioning

confidence: 99%

“…Low (1:18,000 live births) and high estimates (1:16,000 live births) for retinoblastoma were calculated, following the example of Kivela. 5 Since retinoblastoma arises before and near birth, the usual cancer incidence rate calculations (number of cases diagnosed in a given population, sometimes adjusted for the under-5 14 or under-14 11 populations in children’s cancers) do not provide accurate data for retinoblastoma. OECD, Organisation for Economic Co-operation and Development…”

Section: Figurementioning

confidence: 99%

Retinoblastoma

Dimaras

Corson

Cobrinik

et al. 2015

Nat Rev Dis Primers

424

390

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Retinoblastoma is a rare cancer of the infant retina, which forms when both RB1 alleles mutate in a susceptible retinal cell, likely a cone photoreceptor precursor. Loss of the tumour suppressor functions of the retinoblastoma protein, pRB, leads to uncontrolled cell division and recurrent genomic changes during tumour progression. Although pRB is expressed in virtually all tissues, cone precursors have biochemical and molecular features that may sensitize to RB1 loss to enable tumourigenesis. Retinoblastoma is diagnosed in ~8,000 children each year worldwide. Patient survival is >95% in high-income countries, but <30% globally. However, outcomes are improving through increasing awareness for earlier diagnosis, new guidelines and sharing of expertise. Intra-arterial and intravitreal chemotherapy have emerged as promising methods to salvage eyes. Ongoing international collaborations will replace the multiple different classifications of eye involvement with standardized definitions to consistently assess eligibility, efficacy and safety of treatment options. Life-long follow-up is warranted since survivors of heritable retinoblastoma are at risk for developing second cancers. Defining the molecular consequences of RB1 loss in diverse tissues may open new avenues for treatment and prevention of retinoblastoma as well as second cancers in patients with germline RB1 mutations.

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“…ompared with the United States and Western Europe, retinoblastoma manifests more commonly as advanced disease in Latin America and other less developed areas. 1,2 In Latin America, overt extraocular disease at presentation of retinoblastoma is now less common; however, microscopic extraretinal extension occurs frequently, and patients may need adjuvant therapy to decrease the risk of metastatic relapse. 3,4 Experience at a single institution in Buenos Aires, Argentina (Hospital J. P. Garrahan), over 3 prospective protocols suggested that adjuvant treatment of retinoblastoma may not be necessary in patients with choroidal invasion alone or in combination with prelaminar optic nerve invasion.…”

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confidence: 99%

Treatment of Nonmetastatic Unilateral Retinoblastoma in Children

et al. 2018

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IMPORTANCE Multi-institutional collaborative studies that include large patient populations for the management of retinoblastoma with histopathological risk factors could provide important information for patient management. OBJECTIVE To evaluate the implementation of a strategy for the management of nonmetastatic unilateral retinoblastoma in children based on standardized diagnostic and treatment criteria. DESIGN, SETTING, AND PARTICIPANTS This single-arm prospective study applied a strategy based on a single-center experience. The setting was a multicenter study in Latin America (Grupo de America Latina de Oncologia Pediatrica [GALOP]). Participants were children with nonmetastatic unilateral retinoblastoma (staged with the International Retinoblastoma Staging System). The study opened on July 1, 2008, and closed on December 31, 2014. Follow-up was updated until June 30, 2017. INTERVENTIONS Stage 0 patients (without enucleation) were given conservative therapy without a protocol. Stage I patients (with enucleation and no residual tumor) were divided into a high-risk group (retrolaminar invasion and/or scleral invasion) and a low-risk group (all remaining patients). High-risk children received adjuvant chemotherapy with 4 alternating cycles of regimen 1 (cyclophosphamide [65 mg/kg/d] [plus sodium-2-mercaptoethane sulfonate], idarubicin hydrochloride [10 mg/m 2 /d], and vincristine sulfate [0.05 mg/kg/d]) and 4 cycles of regimen 2 (carboplatin [500 mg/m 2 /d, days 1 and 2] and etoposide [100 mg/m 2 /d, days 1-3]). Low-risk children did not receive adjuvant therapy. Children with buphthalmia received neoadjuvant and adjuvant chemotherapy for a total of 8 cycles. MAIN OUTCOMES AND MEASURES Probability of event-free survival (extraocular relapse and death from any cause were considered events). RESULTS Among 187 children registered in the study, 175 were evaluable (92 [52.5%] female; median age, 22 months; age range, 3-100 months). Forty-two were stage 0 children, 84 were stage I low-risk children, and 42 were stage I high-risk children; there were 7 children in the buphthalmia group. With a median follow-up of 46 months, the 3-year probability of event-free survival was 0.97 (95% CI, 0.94-0.99), and the probability of overall survival was 0.98 (95% CI, 0.94-1.00). Stage 0 patients had no events, stage I low-risk patients had 1 event (orbital relapse treated with second-line therapy), stage I high-risk patients had 2 events (1 central nervous system relapse and 1 death from sepsis), and the buphthalmia group had 1 event (orbital relapse, followed by central nervous relapse and death). CONCLUSIONS AND RELEVANCE Adjuvant therapy may be effective for high-risk unilateral retinoblastoma but is toxic, and neoadjuvant chemotherapy for buphthalmus appears feasible.

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Increased incidence and disparity of diagnosis of retinoblastoma patients in Guatemala

Cited by 26 publications

References 17 publications

A tool for planning retinoblastoma services in sub-Saharan Africa

A tool for planning retinoblastoma services in sub-Saharan Africa

Retinoblastoma

Treatment of Nonmetastatic Unilateral Retinoblastoma in Children

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