Clinical Presentation and Outcomes of Stage III or Stage IV Retinoblastoma in 80 Asian Indian Patients

Kaliki, Swathi; Patel, Anamika; Iram, Sadiya; Palkonda, Vijay Anand Reddy

doi:10.3928/01913913-20161019-01

Cited by 12 publications

(9 citation statements)

References 29 publications

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“…Munier, et al Progress in Retinal and Eye Research 73 (2019) 100764 up motivated by clinical features at presentation, presence of classic high-risk pathologic findings (Sastre et al, 2009), or possibly by the degree of anaplasia (Mendoza et al, 2015) after enucleation, or in symptomatic patients during/after conservative treatment. In most cases of metastatic disease at diagnosis, massive orbital extension occurs along with distant metastatic dissemination (Kaliki et al, 2017). The detection of minimally disseminated disease in bone marrow and cerebro-spinal fluid at diagnosis and follow-up can be now evaluated by means of quantitative polymerase chain reaction of cone-rod homeobox (CRX) transcription factor messenger RNA (Laurent et al, 2016).…”

Section: Diagnosis Of Metastatic Retinoblastomamentioning

confidence: 99%

“…Although retinoblastoma is a chemosensitive tumor, metastatic disease is not curable with conventional treatment and is therefore considered to have a poor prognosis, especially in cases of CNS involvement (Kaliki et al, 2017;Pant et al, 2017). High dose chemotherapy, followed by autologous stem cell rescue with radiation to the sites of bulky disease, has been the only treatment reported to date to potentially cure these patients (Dunkel et al, 2010c;Palma et al, 2012), allowing remission in close to 70% of children in a series of 15 patients with metastatic retinoblastoma not involving the CNS (Dunkel et al, 2010c), whereas those with CNS involvement have only anecdotally benefitted from it (Dunkel et al, 2010a).…”

Section: Treatment Of Metastatic Retinoblastomamentioning

confidence: 99%

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Conservative management of retinoblastoma: Challenging orthodoxy without compromising the state of metastatic grace. “Alive, with good vision and no comorbidity”

Munier

Beck‐Popovic

Chantada

et al. 2019

Progress in Retinal and Eye Research

160

157

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Retinoblastoma is lethal by metastasis if left untreated, so the primary goal of therapy is to preserve life, with ocular survival, visual preservation and quality of life as secondary aims. Historically, enucleation was the first successful therapeutic approach to decrease mortality, followed over 100 years ago by the first eye salvage attempts with radiotherapy. This led to the empiric delineation of a window for conservative management subject to a "state of metastatic grace" never to be violated. Over the last two decades, conservative management of retinoblastoma witnessed an impressive acceleration of improvements, culminating in two major paradigm shifts in therapeutic strategy. Firstly, the introduction of systemic chemotherapy and focal treatments in the late 1990s enabled radiotherapy to be progressively abandoned. Around 10 years later, the advent of chemotherapy in situ, with the capitalization of new routes of targeted drug delivery, namely intra-arterial, intravitreal and now intracameral injections, allowed significant increase in eye preservation rate, definitive eradication of radiotherapy and reduction of systemic chemotherapy. Here we intend to review the relevant knowledge susceptible to improve the conservative management of retinoblastoma in compliance with the "state of metastatic grace", with particular attention to (i) reviewing how new imaging modalities impact the frontiers of conservative management, (ii) dissecting retinoblastoma genesis, growth patterns, and intraocular routes of tumor propagation, (iii) assessing major therapeutic changes and trends, (iv) proposing a classification of relapsing retinoblastoma, (v) examining treatable/preventable disease-related or treatment-induced complications, and (vi) appraising new therapeutic targets and concepts, as well as liquid biopsy potentiality.

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Section: Diagnosis Of Metastatic Retinoblastomamentioning

confidence: 99%

Section: Treatment Of Metastatic Retinoblastomamentioning

confidence: 99%

Conservative management of retinoblastoma: Challenging orthodoxy without compromising the state of metastatic grace. “Alive, with good vision and no comorbidity”

Munier

Beck‐Popovic

Chantada

et al. 2019

Progress in Retinal and Eye Research

160

157

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“…Shah et al reported 55.5% overall survival rate in children with Stage 3 and 4 disease at presentation [21]. Kaliki et al analyzed 80 patients with stage 3 or stage 4 disease at presentation and found that the survival rate were 71 and 0% in stage 3 and 4, respectively [23].…”

Section: Retinoblastoma In Indiamentioning

confidence: 99%

Retinoblastoma in Asia

Jain

Rojanaporn

Chawla

et al. 2018

Eye

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Asia-Pacific region bears a significant global burden of retinoblastoma (RB), therefore understanding RB in Asia-Pacific region is important. Based on the year 2013 population estimates, 43% (3452 of 8099 children) of the global burden of RB lives in 6 countries of Asia-Pacific region: 1486 children in India, 1103 children in China, 277 children in Indonesia, 260 children in Pakistan, 184 children in Bangladesh, 142 children in Philippines. There exists a wide disparity, technological and socio-economical, within countries in this region resulting in a varied pattern of clinical presentation and survival varies.Challenges in developing nations are not just technological, but also social. Opportunities emerge for research to study and understand the socio-economical aspects of the disease to develop interventions that are relevant culturally and feasible economically. Possible steps include disease education and counselling, universal screening, highly subsidized/free of cost treatment for low socioeconomic strata, raising funds through the government and non-governmental organizations, sensitization and training of man-power in screening, diagnosis and treatment, and developing new specialized centers with tele-ophthalmology services.

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“…Stage 4 as judged from massive orbital tumour or visible nerve resection margin involvement signaling metastasis is incurable in this setting. This has been shown also in India even when advanced imaging and radiotherapy are available and justifies the decision for palliation alone in Stage 4 disease [20]. Second line chemotherapeutic protocols are available if the standard agents fail but as yet are of uncertain efficacy.…”

Section: Discussionmentioning

confidence: 96%

A ten-year study of Retinoblastoma in Uganda: An approach to improving outcome with limited resources

Waddell

Matua²,

Bidwell³

et al. 2021

Cancer Epidemiology

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Background: Survival of children with cancer in resource-limited regions is very poor compared to better-resourced regions. Retinoblastoma (RB) is a childhood cancer that is commonly reported in many regions of Africa. RB may be safely and effectively treated by non-specialists, which could facilitate more widespread availability of treatment in under-resourced areas.Methods: A ten-year consecutive series of children with RB treated at Ruharo Eye Centre between December 2009 and November 2019 was prospectively followed up. Chemoreduction followed by surgery is the standard approach to therapy. Costs of therapy and also of travel and food are borne by the program which is unaffordable to most families and necessitates donors. Survival by stage of RB and number of eyes affected was described using Kaplan-Meier plots. Visual acuity was assessed for all children with bilateral disease and the retention of sight during follow-up assessed.Results: Among 665 children with RB, 18.2 % (121 children) presented with metastatic (Stage 4) RB with only two of these children surviving >24 months. Five-year survival was 60.2 % among all children with RB rising to 93.3 % and 87.2 % for children with unilateral and bilateral Stage 1 disease, respectively. Among 184 children with bilateral disease, 130 (70.7 %) retained some level of sight following primary treatment with 91 of those (49.5 % of all bilateral children) retaining vision up to their death or to the end of follow-up. Conclusion:Many children in Uganda present with advanced RB and curative treatment is not possible in this setting. Children diagnosed and treated early have good prospects of survival. Retention of sight among many bilaterally affected children is achievable, facilitating access to normal education. Therefore, the strategic priorities for improving survival are changing community perceptions so that children with eye problems are brought without delay, and widening access to modern treatment by using genereal health workers with standard drugs, backed by financial, social and peer support.

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Clinical Presentation and Outcomes of Stage III or Stage IV Retinoblastoma in 80 Asian Indian Patients

Abstract: Compliant multimodality treatment is beneficial in patients with IRSS stage III disease. IRSS stage IV retinoblastoma has poor prognosis despite treatment. [J Pediatr Ophthalmol Strabismus. 2017;54(3):177-184.].

Cited by 12 publications

References 29 publications

Conservative management of retinoblastoma: Challenging orthodoxy without compromising the state of metastatic grace. “Alive, with good vision and no comorbidity”

Conservative management of retinoblastoma: Challenging orthodoxy without compromising the state of metastatic grace. “Alive, with good vision and no comorbidity”

Retinoblastoma in Asia

A ten-year study of Retinoblastoma in Uganda: An approach to improving outcome with limited resources

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