Retinoblastoma in Central America: Report from the Central American Association of Pediatric Hematology Oncology (AHOPCA)

Luna‐Fineman, Sandra; Barnoya, Margarita; Bonilla, Miguel; Fú, Ligia; Baez, F.; Rodríguez‐Galindo, Carlos

doi:10.1002/pbc.23307

Cited by 47 publications

(41 citation statements)

References 20 publications

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“…Our data showed that the 5-year survival rate was 80.9%, which was comparable to that in other developing countries and regions: 80.9% in Taiwan (9), 83.1% in Iran (10), and 81% in other developing countries (Argentina, Brazil, Turkey, Jordan, and Venezuela) (11). The survival rate reported in our study was significantly higher than that reported for less developed countries, such as in East Africa (30%) (12) and Central America (48%) (13).…”

Section: Discussioncontrasting

confidence: 50%

Clinical characteristics and prognosis of patients with retinoblastoma: 8-year follow-up

Luo¹,

Ye²,

Ding³

et al. 2015

Turk J Med Sci

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IntroductionRetinoblastoma (Rb) is the most common intraocular malignant tumor in children, accounting for approximately 3%-4% of all malignant tumors in children aged 0-14 years (1,2). Currently, in developed countries with advanced tools for medical diagnosis, Rb can be detected early, thus leading to an improved survival rate of more than 95% for this high-mortality disease. However, the survival rate from this disease is still as low as approximately 50% worldwide (3). This may be due to the poor diagnostic and therapeutic capacity, as well as generally poor health conditions, experienced in low-income countries. This leads to late diagnosis of the disease and thus a high incidence of extraocular complications and a high risk of distant metastasis (4). Rb can be cured if the disease is diagnosed early, if disease severity is appropriately assessed, and if proper treatments are applied (5). Therefore, this study aimed to identify clinical characteristics of Rb patients, to find the potential relationship between clinical manifestations and survival rates from Rb, and to provide a clinical basis for prediction of disease outcomes and treatment options. Materials and methods Clinical dataThis retrospective study with complete follow-up data included 314 patients who were diagnosed as having Rb at the Zhongshan Ophthalmic Center at Sun Yat-sen University from January 2003 to February 2011. Clinical data, including laterality of eyes, sex, age at diagnosis, presenting signs, lag time before treatment, survival conditions, and survival time, were recorded. Patients were followed until death or the cutoff date (February 2013), whichever occurred first. The mean follow-up time was 41.4 ± 27.9 months (median, 33 months; range, 1-110 months).Patients' clinical presentations at diagnosis were categorized into 6 groups based on the description given by the patients or their families: 1) leukocoria (white reflection from the pupil); 2) strabismus; 3) inflammation Background/aim: The aim of this study was to investigate the potential correlation between clinical characteristics and prognosis of patients with retinoblastoma (Rb). Materials and methods:This retrospective study included 314 Rb patients. Clinical data including laterality of eyes, sex, age, presenting signs, lag time, and survival were recorded and analyzed.Results: Leukocoria is the most common clinical presentation of Rb. Patients with isolated leukocoria had shorter lag time and exhibited a high survival rate (85%, 5 years). Patients with strabismus and blurred vision, and who were older and had longer lag time, exhibited an excellent survival rate (100% and 92.3%, respectively, 5 years). Patients with exophthalmos had the longest lag time and the lowest survival rate (17.8%, 5 years). The 5-year survival rate of patients with a lag time of ≤6 months was 84.7%, which was significantly higher than that of patients with a lag time of >6 months (64.7%). Conclusion:Leukocoria, strabismus, and blurred vision are mild clinical manifestations of Rb that are associ...

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Section: Discussioncontrasting

confidence: 50%

Clinical characteristics and prognosis of patients with retinoblastoma: 8-year follow-up

Luo¹,

Ye²,

Ding³

et al. 2015

Turk J Med Sci

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“…24 Retinoblastoma-specific twinning programmes include partnerships between St. Jude’s Children’s Research Hospital (USA) and the Middle-East, 25 Central America 26 and Mexico, 22 and between the Institut Curie and a centre in Bamako, Mali. 27 The Central American Association of Paediatric Hematology Oncology (AHOPCA) created a cooperative group and implemented multicentre protocols for retinoblastoma treatment, 26,28,29 a major achievement, not yet paralleled in developed countries. The AHOPCA funding is now 90% local.…”

Section: Epidemiologymentioning

confidence: 99%

Retinoblastoma

Dimaras

Corson

Cobrinik

et al. 2015

Nat Rev Dis Primers

453

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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“…In developing countries, enucleation refusal and default rate ranges from 13.7% in Taiwan [36], 22.0% in Central America [37], 31.0% in Malaysia [38], 33.0% in Honduras [39] to 42.0% in Nigeria [40]. Treatment course was often punctuated by dropout and therapy defaulters.…”

Section: Discussionmentioning

confidence: 99%

Retinoblastoma: A Sixteen-Year Review of the Presentation, Treatment, and Outcome from a Tertiary Care Institute in Northern India

Singh¹,

Katoch²,

Kaur³

et al. 2017

Ocul Oncol Pathol

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Purpose: To study epidemiology, demographic profile, clinical characteristics, and outcome in pediatric patients with retinoblastoma. Methods: This was a retrospective review of retinoblastoma patients of a tertiary institute from January 1st 1998 to December 31st 2014. Results: The study included 467 patients (618 eyes) with a mean age of 34.7 ± 24.6 months (median = 30; 15 days to 144 months). Retinoblastoma was bilateral in 151 (32.3%) and there were 61.7% males. Intraocular disease was seen in 301 patients (451 eyes [72.9%]) and extraocular in 166 patients (167 eyes; 27.0%). Out of the 347 (74.3%) who received treatment, primary treatment was chemoreduction in 228 (65.7%) and enucleation in 117 (33.7%), while 25.6% of patients refused treatment and 151 (43.5%) defaulted therapy. Local recurrence was seen in 20 (4.3%), metastasis in 2 (0.4%), and deaths in 13 (2.8%) (average follow-up 28.5 ± 44.4 months). Histopathological high risk features were significantly less in the eyes that received chemoreduction (5.0%) versus primary enucleation (20.8%) (p < 0.0004), but there was no difference in the rate of metastasis, recurrence, and death between the two. Conclusions: The majority of retinoblastoma patients in our study had advanced disease, and nearly a third had extraocular extension. There were a significant number of therapy refusals and dropouts. Chemoreduction led to a significant decrease in the histopathological risk factors without affecting the outcomes.

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Retinoblastoma in Central America: Report from the Central American Association of Pediatric Hematology Oncology (AHOPCA)

Cited by 47 publications

References 20 publications

Clinical characteristics and prognosis of patients with retinoblastoma: 8-year follow-up

Clinical characteristics and prognosis of patients with retinoblastoma: 8-year follow-up

Retinoblastoma

Retinoblastoma: A Sixteen-Year Review of the Presentation, Treatment, and Outcome from a Tertiary Care Institute in Northern India

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