Predicted Trends in the Incidence of Retinoblastoma in the Asia-Pacific Region

Usmanov, Rustam H.; Kivelä, Tero

doi:10.1097/apo.0000000000000060

Cited by 50 publications

(51 citation statements)

References 28 publications

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“…Retinoblastoma accounts for 2.5-4% of all childhood cancers in most developed countries, and a 2- to 3-fold higher incidence of tumors of the eye (majority of which are retinoblastoma in children <15 years of age) has been reported in India [1,11,12]. India still accounts for nearly one-third of retinoblastoma cases in the Asia-Pacific region [13]. There is paucity of literature on epidemiological and clinical profile studies from India, with such large numbers.…”

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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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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“…One recent paper has predicted the incidence trends of retinoblastoma in the Asia-Pacific region, based on the assumption that the incidence of retinoblastoma among live-born children is uniform in all countries 25. The predicted number may be used as a surrogate to evaluate the completeness of registration for retinoblastoma.…”

Section: Discussionmentioning

confidence: 99%

“…25 The predicted number may be used as a surrogate to evaluate the completeness of registration for retinoblastoma. Interestingly, the predicted number by its formulation is 160, close to the observed number of 154 in our study.…”

Section: Discussionmentioning

confidence: 99%

Incidence and survival of retinoblastoma in Taiwan: a nationwide population-based study 1998–2011

et al. 2015

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ObjectiveTo study the epidemiology of retinoblastoma in Taiwan from 1998 to 2011.DesignThis was a retrospective population-based cohort study using the Taiwan National Health Insurance Research Database.ResultsThe present study included 154 patients (92 males, 62 females) with retinoblastoma and the documented overall retinoblastoma incidence was 1 in 17 373 live births without a notable trend over the study period. The incidence per million live births examined by gender was 65.8 for males and 48.5 for females. The age-specific sex ratio increased from 1.4 at age younger than 1 year to 3.0 above age 4 years. Enucleation was performed in 109 (70.8%) children with retinoblastoma, and it was more prevalent in males than in females (77.2% vs 61.3%, p=0.0335). Multivariate Cox regression analyses with adjustment for diagnostic age, sex, and birth year elucidated that enucleation was a significant factor associated with survival (OR 0.27, 95% CI 0.10 to 0.61).ConclusionsThe incidence of retinoblastoma in Taiwan exhibited no marked trend over time. There were more cases of males than females and the male-to-female rate ratio increased with age. Survival outcome was significantly associated with the intervention of enucleation.

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“…The experience in the Ocular Oncology Service of the Helsinki University Hospital, a national referral center for retinoblastoma in Finland, is similar to these series: of 116 consecutive retinoblastomas diagnosed during the past three decades, 11 (9%; 95% confidence interval [CI], 5–16) were detected during the neonatal period. Given the estimated number of 8100 retinoblastomas diagnosed annually worldwide,[1011] one could expect 570–810 of these tumors to be neonatal.…”

Section: Frequency Of Neonatal Retinoblastomamentioning

confidence: 99%

“…[12] Altogether, 23% of familial retinoblastoma in developing as opposed to 72% in developed countries were detected through screening, suggesting that neonatal diagnosis of retinoblastoma may be three times more frequent in the latter. Given that the majority of retinoblastomas is diagnosed in developing countries,[1011] a more likely estimate may thus approach a third of the figures above or 190–270 neonatal retinoblastomas per year worldwide. These are still large numbers, and neonatal patients with retinoblastoma form a distinct subgroup that has certain typical characteristics in common.…”

Section: Frequency Of Neonatal Retinoblastomamentioning

confidence: 99%

Neonatal retinoblastoma

Kivelä

Hadjistilianou

2017

Asia-Pacific Journal of Oncology Nursing

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From 7% to 10% of all retinoblastomas and from 44% to 71% of familial retinoblastomas in developed countries are diagnosed in the neonatal period, usually through pre- or post-natal screening prompted by a positive family history and sometimes serendipitously during screening for retinopathy of prematurity or other reasons. In developing countries, neonatal diagnosis of retinoblastoma has been less common. Neonatal retinoblastoma generally develops from a germline mutation of RB1, the retinoblastoma gene, even when the family history is negative and is thus usually hereditary. At least one-half of infants with neonatal retinoblastoma have unilateral tumors when the diagnosis is made, typically the International Intraocular Retinoblastoma Classification (Murphree) Group B or higher, but most germline mutation carriers will progress to bilateral involvement, typically Group A in the fellow eye. Neonatal leukokoria usually leads to the diagnosis in children without a family history of retinoblastoma, and a Group C tumor or higher is typical in the more advanced involved eye. Almost all infants with neonatal retinoblastoma have at least one eye with a tumor in proximity to the foveola, but the macula of the fellow eye is frequently spared. Consequently, loss of reading vision from both eyes is exceptional. A primary ectopic intracranial neuroblastic tumor known as trilateral retinoblastoma is no more common after neonatal than other retinoblastoma. For many reasons, neonatal retinoblastoma may be a challenge to eradicate, and the early age at diagnosis and relatively small tumors do not guarantee the preservation of both eyes of every involved child. Oncology nurses can be instrumental in contributing to better outcomes by ensuring that hereditary retinoblastoma survivors receive genetic counseling, by referring families of survivors to early screening programs when they are planning for a baby, and by providing psychological and practical support for parents when neonatal retinoblastoma has been diagnosed.

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Predicted Trends in the Incidence of Retinoblastoma in the Asia-Pacific Region

Abstract: Reported retinoblastomas during the last decade in the Asia-Pacific account for less than half of predicted numbers, which can be used as surrogates for evaluating completeness of registration and for advocacy toward reducing deaths and blindness from retinoblastoma.

Cited by 50 publications

References 28 publications

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

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

Incidence and survival of retinoblastoma in Taiwan: a nationwide population-based study 1998–2011

Neonatal retinoblastoma

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