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Indian Journal of Otolaryngology and... Oct 2022Orbital Exenteration is a major surgical procedure that consists of the removal of the orbital bone, orbital fat, eyeball, and its contents including extraocular...
Orbital Exenteration is a major surgical procedure that consists of the removal of the orbital bone, orbital fat, eyeball, and its contents including extraocular muscles. It is an extensive and morbid surgical procedure. Our aim is to systematically review the indications, complications and reconstruction methods utilised for orbital exenteration. An objective electronic database search was conducted in PUBMED Central, MeSH, NLM Catalog, Bookshelf, and PUBMED published in 20 years period from 1999 till 2019. A total of 29 articles were shortlisted for the present review. Most of the studies have eyelid and canthus as most common primary site of malignancy leading to orbital exenteration. Basal cell carcinoma and squamous cell carcinoma being most common pathology. Other intraocular pathology was Retinoblastoma and melanoma. There were various reconstruction methods used by different authors and Sino-orbital fistula was most commonly occurring in majority of studies. Inspite of being a morbid surgery, Orbital Exenteration had acceptable survival and good quality of life. The aggressive pathology that requires orbital exenteration worldwide is mostly periorbital skin, sinus, and intraocular malignancies. The morbidity of the procedure is high with many surgical complications. However, in properly selected patients it can give better outcomes and survival.
PubMed: 36452694
DOI: 10.1007/s12070-020-02270-5 -
Frontiers in Oncology 2022Retinoblastoma (RB) is one of the most common childhood cancers caused by RB gene mutations (tumor suppressor gene in various patients). A better understanding of... (Review)
Review
Retinoblastoma (RB) is one of the most common childhood cancers caused by RB gene mutations (tumor suppressor gene in various patients). A better understanding of molecular pathways and the development of new diagnostic approaches may lead to better treatment for RB patients. The number of studies on ceRNA axes is increasing, emphasizing the significance of these axes in RB. Circular RNAs (circRNAs) play a vital role in competing endogenous RNA (ceRNA) regulatory axes by sponging microRNAs and regulating gene expression. Because of the broadness of ceRNA interaction networks, they may assist in investigating treatment targets in RB. This study conducted a systematic scoping review to evaluate verified loops of ceRNA in RB, focusing on the ceRNA axis and its relationship to circRNAs. This scoping review was carried out using a six-step strategy and the Prisma guideline, and it involved systematically searching the publications of seven databases. Out of 363 records, sixteen articles were entirely consistent with the defined inclusion criteria and were summarized in the relevant table. The majority of the studies focused on the circRNAs circ_0000527, circ_0000034, and circTET1, with approximately two-fifths of the studies focusing on a single circRNA. Understanding the many features of this regulatory structure may help elucidate RB's unknown causative factors and provide novel molecular potential therapeutic targets and medical fields.
PubMed: 35865469
DOI: 10.3389/fonc.2022.910470 -
International Journal of Molecular... Apr 2022The contributory roles of vitamin D in ocular and visual health have long been discussed, with numerous studies pointing to the adverse effects of vitamin D deficiency.... (Review)
Review
The contributory roles of vitamin D in ocular and visual health have long been discussed, with numerous studies pointing to the adverse effects of vitamin D deficiency. In this paper, we provide a systematic review of recent findings on the association between vitamin D and different ocular diseases, including myopia, age-related macular degeneration (AMD), glaucoma, diabetic retinopathy (DR), dry eye syndrome (DES), thyroid eye disease (TED), uveitis, retinoblastoma (RB), cataract, and others, from epidemiological, clinical and basic studies, and briefly discuss vitamin D metabolism in the eye. We searched two research databases for articles examining the association between vitamin D deficiency and different ocular diseases. One hundred and sixty-two studies were found. There is evidence on the association between vitamin D and myopia, AMD, DR, and DES. Overall, 17 out of 27 studies reported an association between vitamin D and AMD, while 48 out of 54 studies reported that vitamin D was associated with DR, and 25 out of 27 studies reported an association between vitamin D and DES. However, the available evidence for the association with other ocular diseases, such as glaucoma, TED, and RB, remains limited.
Topics: Diabetic Retinopathy; Eye; Glaucoma; Humans; Macular Degeneration; Myopia; Vitamin D; Vitamin D Deficiency; Vitamins
PubMed: 35457041
DOI: 10.3390/ijms23084226 -
Evidence-based Complementary and... 2022Melphalan-based intra-arterial chemotherapy was considered an innovative treatment for retinoblastoma patients because high rates of globe salvage could be obtained. Now...
Melphalan-based intra-arterial chemotherapy was considered an innovative treatment for retinoblastoma patients because high rates of globe salvage could be obtained. Now it has been widely applied for primary or secondary treatment of retinoblastoma. This meta-analysis summarizes the most up-to-date evidence regarding the safety and effectiveness of melphalan-based intra-arterial chemotherapy in the treatment of retinoblastoma. The authors searched PubMed, EMBASE, and the Web of Science electronic databases for studies investigating the safety and effectiveness of melphalan-based intra-arterial chemotherapy in the treatment of retinoblastoma. Studies reporting outcomes and complications of melphalan-based intra-arterial chemotherapy for the treatment of retinoblastoma patients would be included. A total of 33 observational studies that involved 1900 patients and 2336 eyes were included. The overall globe salvage rate was 79.6% (773/971 eyes, 0.74 [95% CI: 0.66, 0.80]) for patients treated with IAC as primary therapy in 28 studies. The overall globe salvage rate was 66.4% (923/1391 eyes, 0.68 [95% CI: 0.60, 0.76]) for patients treated with IAC as secondary therapy in 25 studies. The most common ocular complications were retinopathy (32%) and palpebral edema (29.7%). The most common systemic complications were nausea/vomiting (20.9%). The overall metastasis rate was 1.1% (21/1793 patients, 0.038 [95% CI: 0.020, 0.038]). Twenty-nine studies that involved 1783 patients reported the mortality and the overall mortality was 1.5% (26/1783 patients, 0.029 [95% CI: 0.020, 0.048]). Our meta-analysis showed that melphalan-based IAC treatment was an option for retinoblastoma patients with acceptable efficacy according to retrospective studies. Further high-quality randomized control trials are necessary to provide more accurate and reliable results.
PubMed: 35198033
DOI: 10.1155/2022/3156503 -
American Journal of Ophthalmology Jul 2022To determine the visual and refractive outcomes and the ocular and systemic complications of cataract surgery in eyes treated for retinoblastoma. (Review)
Review
PURPOSE
To determine the visual and refractive outcomes and the ocular and systemic complications of cataract surgery in eyes treated for retinoblastoma.
DESIGN
Retrospective consecutive case series and systematic review.
METHODS
Children <18 years of age with retinoblastoma who underwent surgery for secondary cataract between 2000 and 2020 were reviewed. Medline (OVID), Embase, Web of Science, and the Cochrane database were searched from inception to August 2020.
RESULTS
A total of 15 eyes of 15 children were included. The mean age at retinoblastoma diagnosis was 12 months (median, 14; interquartile range [IQR], 4-19). Cataract developed at a mean age of 39 months (median, 31; IQR, 20-52), secondary to multiple treatments (n = 7), pars-plana vitrectomy (n = 3), external-beam radiotherapy (n = 2), laser (n = 2), and retinal detachment (n = 1). The mean preoperative quiescent interval was 44 months (median, 28; IQR, 15-64). Primary intraocular lens implantation was performed in 93%, posterior capsulotomy in 40%, and anterior vitrectomy in 33% of participants. Postoperatively, 100% had improved fundus visibility and 73% had improved vision. Complications included visual axis opacification (11 of 15), capsular phimosis (5 of 15), and zonulopathy (3 of 15). No patient developed intraocular recurrence, extraocular extension, or metastasis at a mean of 76 months (median, 78; IQR, 29-128) follow-up. The systematic review identified 852 studies, with 18 meeting inclusion criteria. Across all studies (n = 220 children), intraocular recurrence occurred in 6%, globe salvage in 91%, and extraocular extension and metastasis in <1%.
CONCLUSIONS
Modern retinoblastoma therapies, including intravitreal chemotherapy and vitrectomy, cause secondary cataract. Following cataract surgery, intraocular recurrence risk is low and extraocular spread is rare. Although surgery improves tumor visualization, visual prognosis may be limited by several factors. Challenges include biometry limitations and a high incidence of zonulopathy.
Topics: Capsule Opacification; Cataract; Cataract Extraction; Child; Child, Preschool; Humans; Infant; Lens Implantation, Intraocular; Male; Postoperative Complications; Retinal Neoplasms; Retinoblastoma; Retrospective Studies; Visual Acuity; Vitrectomy
PubMed: 35172168
DOI: 10.1016/j.ajo.2022.02.001 -
JCO Precision Oncology Feb 2022Soft tissue and bone sarcomas are rare malignancies that exhibit significant pathologic and molecular heterogeneity. Deregulation of the... (Review)
Review
PURPOSE
Soft tissue and bone sarcomas are rare malignancies that exhibit significant pathologic and molecular heterogeneity. Deregulation of the CDKN2A-CCND-CDK4/6-retinoblastoma 1 (Rb) pathway is frequently observed in about 25% of unselected sarcomas and is pathognomonic for specific sarcoma subtypes. This genomic specificity has fueled the clinical evaluation of selective CDK4/6 inhibitors in sarcomas. Here, we highlight successes, opportunities, and future challenges for using CDK4/6 inhibitors to treat sarcoma.
MATERIALS AND METHODS
This review summarizes the current evidence for the use of CDK4/6 inhibitors in sarcoma while identifying molecular rationale and predictive biomarkers that provide the foundation for targeting the CDK4/6 pathway in sarcoma. A systematic review was performed of articles indexed in the PubMed database and the National Institutes of Health Clinical Trials Registry (ClinicalTrials.gov). For each sarcoma subtype, we discuss the preclinical rationale, case reports, and available clinical trials data.
RESULTS
Despite promising clinical outcomes in a subset of sarcomas, resistance to CDK4/6 inhibitors results in highly heterogeneous clinical outcomes. Current clinical data support the use of CDK4/6 inhibitors in subsets of sarcoma primarily driven by CDK4/6 deregulation. When dysregulation of the Rb pathway is a secondary driver of sarcoma, combination therapy with CDK4/6 inhibition may be an option. Developing strategies to identify responders and the mechanisms that drive resistance is important to maximize the clinical utility of these drugs in patients with sarcoma. Potential biomarkers that indicate CDK4/6 inhibitor sensitivity in sarcoma include , , , , , and .
CONCLUSION
CDK4/6 inhibitors represent a major breakthrough for targeted cancer treatment. CDK4/6 inhibitor use in sarcoma has led to limited, but significant, early clinical success. Targeted future clinical research will be key to unlocking the potential of CDK4/6 inhibition in sarcoma.
Topics: Clinical Trials as Topic; Cyclin-Dependent Kinase 4; Cyclin-Dependent Kinase 6; Genomics; Humans; Sarcoma; Soft Tissue Neoplasms; United States
PubMed: 35108033
DOI: 10.1200/PO.21.00211 -
The Lancet. Global Health Mar 2022Despite advancements in globe-preserving treatments, improvements in retinoblastoma outcomes are inconsistent across income levels and geographical locations. We aimed... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Despite advancements in globe-preserving treatments, improvements in retinoblastoma outcomes are inconsistent across income levels and geographical locations. We aimed to investigate trends in global retinoblastoma survival and globe preservation during the past 40 years. We also examined associated socioeconomic and health-care factors and global survival disparity.
METHODS
We did a systematic review and meta-analysis by screening articles in any language in nine databases (PubMed, Embase, ScienceDirect, Web of Science, OpenGrey, Global Burden of Disease, Global Health Data Exchange, Global Index Medicus, and International Agency for the Prevention of Blindness) published between Jan 1, 1981, and Oct 8, 2021. We screened for articles that described retinoblastoma overall survival or globe salvage, or both. All reported studies were subsequently stratified into four periods: 1980-89, 1990-99, 2000-09, and 2010-20. Indicators on socioeconomic and health-care factors were extracted from the World Bank and WHO. Ophthalmology-related indicators were further parsed from the International Agency for the Prevention of Blindness. Between-study heterogeneities by income level were assessed by mixed-effect meta-analysis. Associations of retinoblastoma outcome with socioeconomic and health-care factors and factors for survival prediction were investigated by multivariable linear regressions. This study is registered with PROSPERO, number CRD42020221556.
FINDINGS
Our search identified 14 621 articles, of which 314 studies were included for analysis after screening, including 38 130 patients from 80 regions globally presenting during 1980-2020. 255 articles were entered for time-trend meta-analysis, covering 29 106 patients from 73 countries. Both overall survival (from 79% [95% CI 74-84] to 88% [83-93]; p=0·017) and globe salvage rate (from 22% [14-32] to 44% [36-52]; p=0·0003) improved significantly over the four decades. Wide disparities were observed between higher-income and lower-income countries. Overall survival, globe salvage, and globe salvage for advanced intraocular disease correlated positively with income level. Higher overall survival was associated with lower Gini index (p=0·0001) and with populations that had smaller percentages living in rural areas (p=0·0005). Higher globe salvage was associated with better health-care financing and accessibility (p=0·030). Overall survival (p=0·0024) and globe salvage (p=0·022) were both associated positively with education level. Survival gaps were observed in sub-Saharan Africa and southeast and southwest Asia.
INTERPRETATION
Retinoblastoma treatment outcomes have improved globally over the past four decades but large disparities persist between higher-income and lower-income countries, with some areas having major survival gaps. Targeted health-care policy making with increased health-care financing and accessibility are needed in low-income and lower-middle-income countries to improve retinoblastoma outcomes worldwide.
FUNDING
Health and Medical Research Fund (Hong Kong) and Children Cancer's Foundation (Hong Kong).
Topics: Global Health; Health Care Surveys; Humans; Organ Sparing Treatments; Retinoblastoma; Socioeconomic Factors
PubMed: 35093202
DOI: 10.1016/S2214-109X(21)00555-6 -
Journal of Neurointerventional Surgery Jun 2022
Meta-Analysis
Topics: Antineoplastic Agents; Humans; Infant; Infusions, Intra-Arterial; Melphalan; Retinal Neoplasms; Retinoblastoma
PubMed: 35017205
DOI: 10.1136/neurintsurg-2021-018409 -
Journal of Clinical Laboratory Analysis Oct 2021Retinoblastoma is the most common primary intraocular malignancy in children less than 4 years. Retinoblastoma (RB) contains about 3%-5% of all childhood cancers....
BACKGROUND
Retinoblastoma is the most common primary intraocular malignancy in children less than 4 years. Retinoblastoma (RB) contains about 3%-5% of all childhood cancers. Recent studies demonstrated that interacting between RB tumor suppressor and oncoproteins of DNA tumor viruses such as human papillomavirus (HPV). The objective of the current systematic review study was to present conducted studies in the field of HPV infection and its possible role in retinoblastoma.
METHODS
For this systematic review, all relevant original research studies were assessed by searching in electronic databases include PubMed, Embase, Scopus, Google Scholar, and Web of Science by using relevant keywords. The study was designed based on the PRISMA criteria. All publications with English literature and original researches are considered for screening.
RESULTS
Conducted search results lead to 4070 studies. The title and abstract screening lead to 11 studies. Data extraction was performed on 8 included studies. The prevalence of the HPV was ranged from 0 to 69%, and HPV genotype 16 and 18 were the most detected types. The most used method for the detection of the viruses was PCR, and the most assessed sample was formalin-fixed, paraffin-embedded tissue blocks.
CONCLUSION
The association between HPV and retinoblastoma is still inconsistent. The prevalence of the HPV in RB was ranged from 0 to 69%, which indicates a wide range and highlights the importance of further investigation for more accurate statistical of HPV prevalence in RB. Thus, further worldwide studies of larger sample sizes of cohorts should be investigated to clarify this uncertainty.
Topics: Human papillomavirus 16; Human papillomavirus 18; Humans; Papillomavirus Infections; Prevalence; Retinal Neoplasms; Retinoblastoma
PubMed: 34462972
DOI: 10.1002/jcla.23981 -
Ocular Oncology and Pathology Mar 2021The published data on ocular survival following intravenous chemotherapy of retinoblastoma (RB) seems to be skewed by evolving practice patterns induced by use of... (Review)
Review
PURPOSE
The published data on ocular survival following intravenous chemotherapy of retinoblastoma (RB) seems to be skewed by evolving practice patterns induced by use of intravitreal chemotherapy (iVitc). We aimed to explore potential role of iVitc for vitreous seeding for patients treated with intravenous chemotherapy (IVC).
METHODS
A literature search was performed to identify cases of RB treated with primary IVC prior to advent of iVitc by various search engines (PubMed, Medline, and Google) from 1992 to 2018. Studies were excluded if number of cases were less than 40 or lacked data related to type of recurrence and its treatment. Rates and patterns of recurrence and its management were categorized.
RESULTS
Out of 15 studies identified, only 10 studies (797 eyes) met the inclusion criteria. The mean age at presentation was 15.3 months (range 0-192.8 months). Unilateral cases represented 25% of the cohort. The ocular survival rate with primary IVC was 63% (500/797 eyes). Of the 297 eyes (37%) that failed IVC therapy, additional 99 eyes could be salvaged with EBRT (599/797 eyes, 75%). Remaining 198 eyes were enucleated (198/797 eyes 25%). K-M survival analysis could not be done due lack of sufficient data. Recurrences that occurred (mean 12.2 months) after completion of primary IVC included relapse of retinal tumor (143 eyes [48%]), vitreous seeding (73 eyes [25%]), subretinal seeding (49 eyes [16%]), or any combination (103 eyes [35%]). Out of 73 eyes with vitreous seeding, additional 66 eyes (90%) would have been salvaged with iVitc, potentially improving ocular survival rates to 71% (500 + 66/797).
CONCLUSIONS
Evolving practice patterns of RB treatment have unfavorably skewed published ocular survival rates following IVC. With incorporation of iVitc, the ocular survival rates with IVC can be potentially improved to be non-inferior to those achieved with intra-arterial chemotherapy.
PubMed: 33981697
DOI: 10.1159/000510506