Clinical Features Predictive of High-Risk Retinoblastoma in 403 Asian Indian Patients

Kaliki, Swathi; Srinivasan, Visweswaran; Gupta, Aanchal; Mishra, Dilip Kumar; Naik, Milind N.

doi:10.1016/j.ophtha.2015.01.018

Cited by 75 publications

(69 citation statements)

References 24 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…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: 88%

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

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionsupporting

confidence: 88%

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

View full text Add to dashboard Cite

show abstract

“…84–86 Emerging evidence indicates that high grade anaplasia (loss of differentiated characteristics) may be a risk factor for metastasis and death. 87 High-risk features by the Murphree IIRC 78 were observed in 15–33% of Group D and 50–61% of Group E eyes, 85,88 , but by the Shields IIRC (which classifies less affected D eyes as E), only 24–39% 89 of Group E eyes showed high risk. Adjuvant systemic chemotherapy after high-risk features are observed in an enucleated eye may reduce risk of metastatic relapse.…”

Section: Diagnosis Screening and Preventionmentioning

confidence: 99%

“…There is a general consensus that eyes with clinical features such as neovascular glaucoma, pthisis bulbi (a shrunken, non-functional eye), and anterior disease are not appropriate for any conservative therapy and require enucleation to know if further therapy is needed for high risk pathlogical features. 79,85,88,89 This may be particularly problematic when IAC is offered for eyes with advanced disease, since only the eye receives the chemotherapy. 79,85,88,89 Clinical variables strongly associated with high risk features included older age, longer lag from diagnosis to enucleation, anterior tumour or blood, scleral invasion, invasion of the post-laminar optic nerve and orbital cellulitis.…”

Section: Managementmentioning

confidence: 99%

“…79,85,88,89 This may be particularly problematic when IAC is offered for eyes with advanced disease, since only the eye receives the chemotherapy. 79,85,88,89 Clinical variables strongly associated with high risk features included older age, longer lag from diagnosis to enucleation, anterior tumour or blood, scleral invasion, invasion of the post-laminar optic nerve and orbital cellulitis. 89,111,112 Attempted salvage by any method of an eye with features indicating high risk for extraocular extension imposes the danger of undiagnosed disease with risk for metastasis.…”

Section: Managementmentioning

confidence: 99%

“…79,85,88,89 Clinical variables strongly associated with high risk features included older age, longer lag from diagnosis to enucleation, anterior tumour or blood, scleral invasion, invasion of the post-laminar optic nerve and orbital cellulitis. 89,111,112 Attempted salvage by any method of an eye with features indicating high risk for extraocular extension imposes the danger of undiagnosed disease with risk for metastasis. By the Murphree IIRC 78 high-risk features were present in 15–33% of Group D and 50–61% of Group E eyes 85,88 , and by the Shields IIRC 79 24–39% 89 of Group E eyes showed high-risk.…”

Section: Managementmentioning

confidence: 99%

See 2 more Smart Citations

Retinoblastoma

Dimaras

Corson

Cobrinik

et al. 2015

Nat Rev Dis Primers

452

390

View full text Add to dashboard Cite

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.

show abstract