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Retinoblastoma

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2. Focal laser treatment in addition to chemotherapy for retinoblastoma. (PubMed)

Focal laser treatment in addition to chemotherapy for retinoblastoma. Retinoblastoma is the most common primary intraocular malignancy of childhood. Systemic chemotherapy is a common treatment for intraocular retinoblastoma, and laser treatment is used as adjuvant therapy during or immediately after chemotherapy courses in selected cases.To compare the effectiveness and safety of adding focal laser therapy to systemically-delivered chemotherapy in treating intraocular retinoblastoma.We searched (...) , that is the proportion of eyes with recurrence of tumours within three years from treatment MAIN RESULTS: No studies met the inclusion criteria for this review.No evidence from randomised controlled trials was found to support or refute laser therapy in addition to systemic chemotherapy for postequatorial retinoblastoma.

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2017 Cochrane

3. Retinoblastoma

Retinoblastoma Retinoblastoma - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Retinoblastoma Last reviewed: February 2019 Last updated: October 2017 Summary Most common intraocular malignancy in children. 90% of all retinoblastoma cases are diagnosed by 3 years of age. Disease can be unilateral or bilateral. Most common presenting sign is leukocoria (white papillary reflex), which can often be seen in photographs (...) be required. Definition The most common malignant intraocular tumour in children. Villegas VM, Hess DJ, Wildner A, et al. Retinoblastoma. Curr Opin Ophthalmol. 2013 Nov;24(6):581-8 http://www.ncbi.nlm.nih.gov/pubmed/24100372?tool=bestpractice.com It can be unilateral or bilateral, familial or spontaneous, and in 30% to 40% of cases is accompanied by a germinal mutation in the RB1 gene. Schefler AC, Abramson DH. Retinoblastoma: what is new in 2007-2008. Curr Opin Ophthalmol. 2008 Nov;19(6):526-34. http

2017 BMJ Best Practice

4. Multimodal Therapy for Stage III Retinoblastoma (International Retinoblastoma Staging System): A Prospective Comparative Study. (PubMed)

Multimodal Therapy for Stage III Retinoblastoma (International Retinoblastoma Staging System): A Prospective Comparative Study. To compare the efficacy of 2 chemotherapeutic drug combinations as part of multimodal therapy for orbital retinoblastoma.Prospective, comparative, study.Patients with stage III retinoblastoma (International Retinoblastoma Staging System).Demographic and clinical features were recorded at presentation. Treatment consisted of a multimodal protocol with neoadjuvant (...) difference in grade 4 neutropenia (P = 0.002).This study compared the outcomes of VEC chemotherapy with a 5-drug combination of etoposide and carboplatin, alternating with cyclophosphamide, idarubicin, and vincristine, for stage III retinoblastoma. The VEC combination was found to be more effective and may be recommended as neoadjuvant and adjuvant chemotherapy.Copyright © 2016 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

2017 Ophthalmology

5. Association of electroretinography with visual outcomes after ophthalmic artery chemosurgery for retinoblastoma in ICRb D and E eyes. (PubMed)

Association of electroretinography with visual outcomes after ophthalmic artery chemosurgery for retinoblastoma in ICRb D and E eyes. Predictions of visual outcomes are useful in clinical and family decisions regarding treatment for retinoblastoma. Very little has been published on the association of post-treatment visual acuity with pre-treatment electroretinography (ERG), which can be performed on infants too young to reliably quantify visual acuity.To report associations of pre-treatment ERG (...) with post-treatment visual acuity in eyes with advanced retinoblastoma treated with ophthalmic artery chemosurgery (OAC).Retrospective case-control study of eyes treated from 2006 through 2017, with mean follow-up of 51 months (range 2.3-150 months).Single large academic center.Group D and E eyes treated with OAC at Memorial Sloan Kettering Cancer Center with recorded visual acuity and ERG (30Hz flicker).Snellen visual acuity (uncorrected) compared to initial 30Hz flicker ERG.This study included 157

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2019 PLoS ONE

6. Systematic review of the current status of programs and general knowledge of diagnosis and management of retinoblastoma.

Systematic review of the current status of programs and general knowledge of diagnosis and management of retinoblastoma. This systematic review aims to report the current knowledge of retinoblastoma (Rb) and its implications in Mexico. We analyzed clinical and demographic data of patients with Rb at select hospitals with Rb programs or that treat and refer patients with Rb, and identified the gaps in practice. We propose solutions to improve diagnosis, provide adequate treatment, and improve (...) . Knowledge of the disease is limited, despite the fact that the Mexican Retinoblastoma Group has elaborated Rb treatment guidelines and is developing a national Rb registry. Especially in the Southern states, prevalence and outcomes are comparable to African and Asian countries, and only few patients are referred to national treatment centers. Only three institutions have comprehensive Rb programs.There is an immediate need in Mexico to expand primary care providers' knowledge of Rb and to expand

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2018 Boletin medico del Hospital Infantil de Mexico

7. Retinoblastoma in Sub-Saharan Africa: Case Studies of the Republic of Côte d’Ivoire and the Democratic Republic of the Congo (PubMed)

Retinoblastoma in Sub-Saharan Africa: Case Studies of the Republic of Côte d’Ivoire and the Democratic Republic of the Congo In most low-income countries, the diagnosis of retinoblastoma is delayed, resulting in a severe prognosis. The objectives of this study were to describe the access to diagnosis and care of children diagnosed with retinoblastoma and the challenges in two sub-Saharan African countries: the Republic of Côte d'Ivoire and the Democratic Republic of the Congo.A descriptive (...) cross-sectional study was conducted. Data were collected from the medical records of patients admitted during the period of January 1, 2013 to December 31, 2014. Data were entered and analyzed using Epi Info7.1 software and SAS 9.3.One hundred sixteen cases of retinoblastoma were collected, including 60 boys and 56 girls. The median diagnosis age was 3 years for both countries. Ninety-eight patients (84%) had unilateral retinoblastoma. Most of the patients presented with advanced disease (76% had

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2018 Journal of global oncology

8. Retinoblastoma Treatment (PDQ®): Health Professional Version

Retinoblastoma Treatment (PDQ®): Health Professional Version Retinoblastoma Treatment (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf Warning: The NCBI web site requires JavaScript to function. Search database Search term Search NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health. PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. PDQ Cancer Information Summaries [Internet]. Bethesda (MD): ; 2002 (...) -. Search term Retinoblastoma Treatment (PDQ®) Health Professional Version PDQ Pediatric Treatment Editorial Board . Published online: August 22, 2018. This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of retinoblastoma. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions

2018 PDQ - NCI's Comprehensive Cancer Database

9. Atypical intravitreal growth of retinoblastoma with a multi-branching configuration (PubMed)

Atypical intravitreal growth of retinoblastoma with a multi-branching configuration To report the clinical and histopathological findings of atypical intravitreal growth of a retinoblastoma with a multi-branching configuration.A 7-month-old boy was referred to our hospital due to leukocoria in the right eye. Ophthalmic examinations identified multi-branching vessels surrounded by diaphanous tissue behind the lens in the right eye. Imaging modalities showed microphthalmos, band-shaped (...) calcification, and cystic lesions in that eye. Because it was difficult to rule out congenital anomalies such as persistent fetal vasculature due to the atypical clinical features of retinoblastoma, we performed a biopsy using a limbal approach. An intraoperative rapid pathological examination led to the definitive diagnosis of retinoblastoma. The right eye was enucleated and postoperative adjuvant chemotherapy was administered. Immunohistochemical staining of the enucleated eyeball showed that the tumoral

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2017 American journal of ophthalmology case reports

10. Ocular side effects following intravitreal injection therapy for retinoblastoma: a systematic review

Ocular side effects following intravitreal injection therapy for retinoblastoma: a systematic review Ocular side effects following intravitreal injection therapy for retinoblastoma: a systematic review Ocular side effects following intravitreal injection therapy for retinoblastoma: a systematic review Smith SJ, Smith BD, Mohney BG CRD summary This review concluded that significant ocular complications were uncommon following intravitreal injection therapy for retinoblastoma (malignant tumour (...) of the retina), and this risk may be reduced further by the use of careful injection technique and standard dosing regimens. These conclusions appear likely to be reliable, and the review recommendations for research are appropriate. Authors' objectives To describe the ocular side effects in patients receiving intravitreal injection therapy for retinoblastoma. Searching PubMed, Scopus, Science Citation Index, and Conference Proceedings Citation Index-Science were searched up to April 2013 with no language

2014 DARE.

11. APC/C and retinoblastoma interaction: cross-talk of retinoblastoma protein with the ubiquitin proteasome pathway (PubMed)

APC/C and retinoblastoma interaction: cross-talk of retinoblastoma protein with the ubiquitin proteasome pathway The ubiquitin (Ub) ligase anaphase promoting complex/cyclosome (APC/C) and the tumour suppressor retinoblastoma protein (pRB) play key roles in cell cycle regulation. APC/C is a critical regulator of mitosis and G1-phase of the cell cycle whereas pRB keeps a check on proliferation by inhibiting transition to the S-phase. APC/C and pRB interact with each other via the co-activator

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2016 Bioscience reports

12. The N Terminus of the Retinoblastoma Protein Inhibits DNA Replication via a Bipartite Mechanism Disrupted in Partially Penetrant Retinoblastomas (PubMed)

The N Terminus of the Retinoblastoma Protein Inhibits DNA Replication via a Bipartite Mechanism Disrupted in Partially Penetrant Retinoblastomas The N-terminal domain of the retinoblastoma (Rb) tumor suppressor protein (RbN) harbors in-frame exon deletions in partially penetrant hereditary retinoblastomas and is known to impair cell growth and tumorigenesis. However, how such RbN deletions contribute to Rb tumor- and growth-suppressive functions is unknown. Here we establish that RbN directly (...) -terminal Rb loss-of-function deletions contribute to the etiology of partially penetrant retinoblastomas. Copyright © 2016, American Society for Microbiology. All Rights Reserved.

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2016 Molecular and cellular biology

13. Chronic medical conditions in adult survivors of retinoblastoma: Results of the Retinoblastoma Survivor Study. (PubMed)

Chronic medical conditions in adult survivors of retinoblastoma: Results of the Retinoblastoma Survivor Study. Limited data are available regarding long-term morbidity in adult survivors of retinoblastoma (Rb).The Retinoblastoma Survivor Study is a retrospective cohort of adult survivors of Rb diagnosed between 1932 and 1994. Participants completed a comprehensive questionnaire adapted from the Childhood Cancer Survivor Study surveys. Chronic conditions were classified using the National Cancer

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2016 Cancer

14. Non-invasive diagnosis of retinoblastoma using cell-free DNA from aqueous humour. (PubMed)

Non-invasive diagnosis of retinoblastoma using cell-free DNA from aqueous humour. Retinoblastoma is the most common eye malignancy in childhood caused by mutations in the RB1 gene. Both alleles of the RB1 gene must be mutated for tumour development. The initial RB1 mutation may be constitutional germline or somatic (originating in one retinal cell only). Distinguishing between these alternative mechanisms is crucial, with wider implications for management of the patient and family members (...) . Bilateral retinoblastoma is nearly always due to a constitutional mutation; however, approximately 15% of unilateral cases also carry a germline mutation, and identifying these cases is important. This can be achieved by identifying both mutation types in tumour tissue and excluding their presence in blood. Modern eye-saving chemotherapy treatment (systemic, intra-arterial and intravitreal) has resulted in fewer enucleations. As a result, tumour tissue required to identify sporadic RB1 mutation(s

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2019 British Journal of Ophthalmology

15. Patterns of Cause-Specific Mortality Among 2053 Survivors of Retinoblastoma, 1914-2016. (PubMed)

Patterns of Cause-Specific Mortality Among 2053 Survivors of Retinoblastoma, 1914-2016. Previous studies of hereditary retinoblastoma survivors have reported elevated mortality, particularly for sarcomas, compared with the general population. However, cause-specific mortality patterns for long-term hereditary and nonhereditary retinoblastoma survivors are poorly understood.Among 2053 retinoblastoma patients diagnosed during 1914-2006 at two major US treatment centers and followed to 2016, we (...) estimated cumulative mortality, standardized mortality ratios (SMRs), and absolute excess risks (AERs) compared with the US general population.Most deaths occurred in 1129 hereditary retinoblastoma patients (n = 518 deaths, cumulative mortality 70 years after retinoblastoma = 75.8%, 95% CI = 69.0% to 82.6%; SMR = 8.5, 95% CI = 7.7 to 9.2). Of these, 267 were due to subsequent cancers (SMR = 27.4, 95% CI = 24.2 to 30.9; AER = 72.3 deaths/10 000 person-years), for which SMRs were highest 15-29 years after

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2019 Journal of the National Cancer Institute

16. Effects of lentivirus-mediated astrocyte elevated gene-1 overexpression on proliferation and apoptosis of human retinoblastoma cells. (PubMed)

Effects of lentivirus-mediated astrocyte elevated gene-1 overexpression on proliferation and apoptosis of human retinoblastoma cells. To investigate the effect of astrocyte elevated gene-1 (AEG-1) overexpression on the biological behaviour of human retinoblastoma (RB) cells and its possible mechanism.Three human RB cell lines (SO-RB50, Y79 and WERI-RB1) were infected with AEG-1-GFP recombinant lentiviral vectors to induce AEG-1 overexpression, while the cells infected with negative lentiviral

2019 Acta ophthalmologica

17. Ocular toxicity of intravitreal melphalan for retinoblastoma in Chinese patients. (PubMed)

Ocular toxicity of intravitreal melphalan for retinoblastoma in Chinese patients. To evaluate the efficacy, complications, and clinical characteristics, including the ocular toxicity, of intravitreal melphalan(IVM) treatment for vitreous seeding in Chinese retinoblastoma patients.This was a retrospective, non-comparative analysis including 30 consecutive eyes of 23 patients with viable persistent or recurrent vitreous seeding following retinoblastoma treatment. All of the eyes received IVM (...) classification or seed regression patterns.Intravitreal melphalan is an effective treatment for refractory vitreous seeding from retinoblastoma, but exhibits both anterior and posterior segment toxicity in Chinese patients.

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2019 BMC Ophthalmology

18. Preservation of retinoblastoma group E eyes with neovascular glaucoma using intravenous chemotherapy: risk factors and outcomes. (PubMed)

Preservation of retinoblastoma group E eyes with neovascular glaucoma using intravenous chemotherapy: risk factors and outcomes. To report the outcomes of retinoblastoma group E eyes with neovascular glaucoma (NVG) treated conservatively with intravenous chemotherapy and investigate factors associated with eye salvage and secondary enucleation.This is a retrospective, comparative, interventional case series. The outcome measures were life salvage, eye salvage and vision salvage.Of the 37 eyes (...) =0.023). Further, the risk factors for secondary enucleation by univariate analysis were duration of symptoms >10 weeks (p=0.003), presenting IOP >26 mm Hg (p=0.045), buphthalmos (p=0.014) and sterile orbital cellulitis (p=0.023) and by multivariate analysis were age at presentation >6 months (p=0.012) and buphthalmos (p=0.017). At a mean follow-up of 20.5 months, none of the patients in either group developed systemic metastasis.For retinoblastoma group E eyes presenting with NVG, the chance of eye

2019 British Journal of Ophthalmology

19. Examinations under anaesthesia as a measure of disease burden in unilateral retinoblastoma: the London experience. (PubMed)

Examinations under anaesthesia as a measure of disease burden in unilateral retinoblastoma: the London experience. Early diagnosis strategies and advances in retinoblastoma (Rb) management have resulted in nearly 100% survival. More attention should, therefore, be given to quality of life considerations. We aimed to quantify the number of examinations under anaesthesia (EUAs) in a cohort of patients with Rb, as a measure of disease burden.A retrospective analysis of patients with unilateral Rb (...) that presented to the London Rb service from 2006 to 2013, were treated and had long-term follow-up. Correlations of clinical variables to number of EUAs were investigated.A total of 107 patients with Rb were included that presented at a mean age of 26.51 ± 22.68 months. The International Intraocular Retinoblastoma Classification (IIRC) was group B in 5 (5%), C in 13 (12%), D in 48 (45%) and E in 41 (38%) of the cases. Primary treatment was intravenous chemotherapy in 36 (34%) and enucleation in 71 (66

2019 British Journal of Ophthalmology

20. Surface-Modified Melphalan Nanoparticles for Intravitreal Chemotherapy of Retinoblastoma. (PubMed)

Surface-Modified Melphalan Nanoparticles for Intravitreal Chemotherapy of Retinoblastoma. The goal of this work was to design and assess the ability of unmodified and surface-modified poly(lactic-co-glycolic acid) (PLGA) nanoparticles (NPs) to enhance cell association, provide efficacy in retinoblastoma cells, and overcome current administration challenges, including hydrolysis and precipitation, of intravitreal administration.A single emulsion method was used to encapsulate Coumarin 6 (...) , to enable NP visualization via fluorescence microscopy. Melphalan NPs were synthesized using an adapted double-emulsion method to reduce melphalan loss during fabrication. Melphalan loading and release were quantified against a free melphalan standard. The cellular association and internalization of unmodified and surface-modified NPs were determined using flow cytometry, and the efficacy of melphalan NPs was quantified in retinoblastoma cells.The highest cell association was observed with TET1 and MPG

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2019 Investigative Ophthalmology & Visual Science

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