Bilateral advanced (group D or E) intraocular retinoblastoma: outcomes in 72 Asian Indian patients

Kaliki, Swathi; Mittal, Priya; Mohan, Sashwanthi; Chattannavar, Goura; Jajapuram, Sai Divya; Mohamed, Ashik; Palkonda, Vijay Anand Reddy

doi:10.1038/s41433-019-0409-z

Cited by 17 publications

(17 citation statements)

References 26 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…[ 24 25 ] In a study by Kaliki et al . [ 26 ] from India, systemic chemotherapy was the most commonly used primary treatment modality in view of advanced disease. In an attempt to salvage the eye and improve results for advanced disease neoadjuvant chemotherapy was offered to 87.5% patients in our analysis but it failed to help in preservation of the eye.…”

Section: Discussionmentioning

confidence: 99%

Epidemiological profile of retinoblastoma in north India: Implications for primary care and family physicians

Gupta

Pandey

Dimri

et al. 2020

J Family Med Prim Care

View full text Add to dashboard Cite

Background: Retinoblastoma is the most common primary intraocular malignancy among children. Despite being curable in early stages, majority of the cases in India present in late stages, when outcomes are very poor. Objectives: The aim of this study was to assess the epidemiological profile, clinical characteristics, and treatment practices among retinoblastoma patients in north India. Materials and Methods: Data on all patients with retinoblastoma, over a 10-year-time period from 2009 to 2018, who were treated in a tertiary care hospital in north India, were assessed. Data were analyzed to describe the demographic characteristics, clinical features in terms of stage at presentation, and management practices in terms of diagnostic investigations and treatment. The statistical significance for difference in percentages was assessed using Fischer's exact test at a 5% significance level. Results: A total of 25 retinoblastoma patients were enlisted, of whom one was excluded as it was adult onset retinoblastoma. The median age at presentation was 3 years, with a male to female ratio of 1:1.4. Bilateral presentation was seen in 16.6% cases. Majority (66.6%) of the patients underwent magnetic resonance imaging of brain and orbit as a part of the diagnostic workup. Intraocular disease was seen in 58.3% patients, whereas 41.6% patients had extraocular disease. Local therapy with vision preservation could be used only in 8.3% patients, whereas 87.5% patients were referred for enucleation. Chemotherapy with combination of vincristine, etoposide, and carboplatin was used extensively both, in neoadjuvant setting (83.3%) and in the adjuvant setting. Conclusion: Despite availability of treatment for eye preservation, its utility is limited due to the advanced stage at presentation. Awareness about the disease and its symptoms for early diagnosis, especially with the Mid-Level Health Provider at Health and Wellness Centers, is likely to improve early reporting and treatment and meeting the Vision 2020 goals.

show abstract

Section: Discussionmentioning

confidence: 99%

Epidemiological profile of retinoblastoma in north India: Implications for primary care and family physicians

Gupta

Pandey

Dimri

et al. 2020

J Family Med Prim Care

View full text Add to dashboard Cite

show abstract

“…The globe survival rate was 47% [ 110 ] for bilateral group D eyes treated with systemic chemotherapy alone and increased to 68% in combination with low-dose intensity-modulated radiation therapy [ 119 ]. Group E eyes are often considered unsalvageable because of the metastatic risk [ 110 ], although multimodality treatment lead to less enucleation [ 9 ].…”

Section: Treatmentsmentioning

confidence: 99%

“…Studies have demonstrated that globes can be saved without an increased risk of metastatic disease or orbital recurrence by systemic chemoreduction and subsequent enucleation for poor responses [ 8 , 145 ]. Globe salvage can be achieved for advanced group D and E eyes with a lower rate for group E eyes, while some studies report vision salvage [ 9 , 134 , 138 , 151 ]. Clinical indications are needed to predict whether these high-risk pathological features present without histopathological examination, so that the globe can be spared or treated promptly.…”

Section: Treatmentsmentioning

confidence: 99%

“…Retinoblastoma is a curable cancer with ocular survival when treated promptly, but it is universally fatal if left untreated. Tumors become advanced if treatment is delayed, hindering vision and globe salvages with a risk of metastasis [ 8 , 9 , 10 ]. Chemotherapy is the standard of care for patients with a high risk of metastasis but has inevitable toxicity [ 10 , 11 , 12 , 13 , 14 , 15 ].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Retinoblastoma: Etiology, Modeling, and Treatment

Kaewkhaw

Rojanaporn

2020

Cancers

View full text Add to dashboard Cite

Retinoblastoma is a retinal cancer that is initiated in response to biallelic loss of RB1 in almost all cases, together with other genetic/epigenetic changes culminating in the development of cancer. RB1 deficiency makes the retinoblastoma cell-of-origin extremely susceptible to cancerous transformation, and the tumor cell-of-origin appears to depend on the developmental stage and species. These are important to establish reliable preclinical models to study the disease and develop therapies. Although retinoblastoma is the most curable pediatric cancer with a high survival rate, advanced tumors limit globe salvage and are often associated with high-risk histopathological features predictive of dissemination. The advent of chemotherapy has improved treatment outcomes, which is effective for globe preservation with new routes of targeted drug delivery. However, molecularly targeted therapeutics with more effectiveness and less toxicity are needed. Here, we review the current knowledge concerning retinoblastoma genesis with particular attention to the genomic and transcriptomic landscapes with correlations to clinicopathological characteristics, as well as the retinoblastoma cell-of-origin and current disease models. We further discuss current treatments, clinicopathological correlations, which assist in guiding treatment and may facilitate globe preservation, and finally we discuss targeted therapeutics for future treatments.

show abstract

“…Тенденция к расширению показаний для комбинированного органосохраняющего лечения поддерживается большинством специалистов [15,30,31]. Показано, что применение локальной химиотерапии при РБ групп C-D не увеличивает количество рецидивов и частоту смертности от метастатической болезни [15,32,33].…”

Section: результатыunclassified

Survival of retinoblastoma patients: a cohort study by two Russian medical centers

Саакян

Иванова

Тадевосян

et al. 2021

Rossijskij oftalʹmologičeskij žurnal

View full text Add to dashboard Cite

Purpose. To evaluate the overall, disease-specific, event-free, relapse-free survival of retinoblastoma (RB) patients and cumulative probability of eye survival in such patients.Material and methods. The study included 223 children with RB (126 male, 97 female). Monolateral disease was diagnosed in 69.1 % of cases (n = 154), bilateral disease in 30.9 % (n = 69). The median follow-up was 40 months (interquartile range from 27 to 53 months). According to ABC classification, group A was diagnosed in 16 of 292 eyes (5.5 %), group B — in 53 (18.2 %), group C — in 41 (14.0 %), group D — 58 (19.9 %), and group E — in 124 (42.5 %). Group E was detected twice as often in patients with unilateral RB as in bilateral RB (56.5 % versus 26.8 %; p < 0.01). Stage T1 according to TNM classification was diagnosed in 69 cases (23.6 %), stage T2-T3 — in 213 cases (72.9 %). The extraocular form (stage T4) was detected in 3.4 % of cases (n = 10).Results. The overall five-year survival of patients treated for RB (n = 223) was 96.4 ± 1.4 %. The 5-year disease- specific survival (n = 222) was 96.8 ± 1.3 % in the general cohort, 96.5 ± 1.5 % in the group of children with monocular RB, 97.3 ± 2.7 % for binocular RB. Five-year event-free survival rate of children who received treatment (n = 223) was 66.7 ± 3.6 %, with monolateral lesion (n = 154) — 74.1 ± 4.2 %, with bilateral lesion — 50.6 ± 6.2 % (n = 69). Five-year relapse-free survival took place in 83.3 ± 3.1 %, with monolateral RB — 87.8 ± 3.3 %, with bilateral RB — 73.3 ± 6.4 %. Primary enucleation was performed in 121 of the 223 children treated for RB and included in the study. The frequency of primary enucleation with monocular RB was more than twice as high (55.2 %; 85 of 154 eyes) as with binocular RB (26.1 %; 36 of 138 eyes; p < 0.01). Organ-preserving treatment was carried out in 138 cases (171 eyes). It was effective in 123 (89.2 %) children (152 eyes; 88.9 %). 5-year ocular survival was 85.7 ± 3.7 %, with monolateral RB — 78.2 ± 6.6 %, with bilateral RB — 92.2 ± 2.8 %. Ocular survival counted separately for groups A, B, C, D, equaled 100 %, 100%, 76.6 ± 6.9 %, and 71.1 ± 11.9 %, respectively. The five-year disease-specific survival rate of children with monocular lesion given eye-preserving treatment was 100%, significantly higher than after primary enucleation — 93.4 ± 2.9 %.Conclusions. Modern combined retinoblastoma treatment is able to save the sick children’s lifes and and even their eyes and visual functions, which improves social adaptation and quality of life. It has been shown that, with a binocular form, it is possible to save more eyes than with a monocular form. Eye-preserving treatment administered to patients with intraocular RB under strictly observed criteria does not heighten the risk of metastasis. Yet timely enucleation in cases of absolute indications remains the method of choice in saving the life of a child.

show abstract

Bilateral advanced (group D or E) intraocular retinoblastoma: outcomes in 72 Asian Indian patients

Cited by 17 publications

References 26 publications

Epidemiological profile of retinoblastoma in north India: Implications for primary care and family physicians

Epidemiological profile of retinoblastoma in north India: Implications for primary care and family physicians

Retinoblastoma: Etiology, Modeling, and Treatment

Survival of retinoblastoma patients: a cohort study by two Russian medical centers

Contact Info

Product

Resources

About