Clinical presentation and survival of retinoblastoma in Indian children

Chawla, Bhavna; Hasan, Muhamad Fahmi; Azad, Rajvardhan; Seth, Rachna; Upadhyay, Ashish Datt; Pathy, Sushmita; Pandey, RM

doi:10.1136/bjophthalmol-2015-306672

Cited by 124 publications

(148 citation statements)

References 24 publications

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“…The average age at presentation of our patients (∼35 months) is comparable to the range of 29-33 months in previous reports from India [3,12,14]. It is also similar to studies from other medium Human Development Index (HDI) countries like Egypt [15] (36 months) and Mali [16] (50 months).…”

Section: Discussionsupporting

confidence: 74%

“…There is a definite regional bias in studies from our country as males were generally preferred for treatment in earlier studies as well [3]. Being a tertiary care center, we largely get referrals of children from local hospitals, and it is alarming to note that health problems in female children are often ignored and undetected [20,21].…”

Section: Discussionmentioning

confidence: 99%

“…Survival in cases of extraocular retinoblastoma is even poorer due to intricate and ill-defined protocols for treatment [1,2]. Studies from our country reveal a large population of children with advanced stage at presentation with a mortality as high as 24% [3]. …”

Section: Introductionmentioning

confidence: 99%

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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: Discussionsupporting

confidence: 74%

Section: Discussionmentioning

confidence: 99%

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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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“…In our study, median age of presentation was 24 months which is comparable with previous other studies from North India (29 months), Iran (28.5 months), Taiwan (26.3 months) and Malaysia (24.2 months). 15,20,21 A relatively higher median age at diagnosis has been reported from African countries like Ghana (36 months) and Kenya (37.5 months). 14,22 Tumours were relatively diagnosed at an earlier age in Australia and USA.…”

Section: Discussionmentioning

confidence: 99%

“…13,14 In North India, Bhavna et al, reported a survival probability of 83%, 73% and 65% at 1 year, 2 years and 5 years, respeactively. 15 A comprehensive multimodality treatment is pertinent in RB to improve outcomes. The term chemoreduction is used to describe the technique of using chemotherapy to reduce the retinal tumor size for better control.…”

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confidence: 99%

Retinoblastoma and ndash; pattern, presentation and management: a quintessential experience of 5 years

2016

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Global Retinoblastoma Presentation and Analysis by National Income Level

Fabian¹,

Elhassan²,

Abdullahi³

et al. 2020

JAMA Oncol

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234

117

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Global Retinoblastoma Study Group IMPORTANCE Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale.OBJECTIVES To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. DESIGN, SETTING, AND PARTICIPANTSA total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. MAIN OUTCOMES AND MEASURESAge at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. RESULTSThe cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low-and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI,, and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI,). CONCLUSIONS AND RELEVANCEThis study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs.

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Clinical presentation and survival of retinoblastoma in Indian children

Cited by 124 publications

References 24 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

Retinoblastoma and ndash; pattern, presentation and management: a quintessential experience of 5 years

Global Retinoblastoma Presentation and Analysis by National Income Level

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