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Child's Nervous System : ChNS :... Jul 2024Pediatric optic pathway/hypothalamic gliomas (OPHG) pose challenges in treatment due to their location and proximity to vital structures. Surgical resection plays a key... (Meta-Analysis)
Meta-Analysis Review
Pediatric optic pathway/hypothalamic gliomas (OPHG) pose challenges in treatment due to their location and proximity to vital structures. Surgical resection plays a key role in the management of OPHG especially when the tumor exhibits mass effect and causes symptoms. However, data regarding outcomes and complications of surgical resection for OPHG remains heterogenous. The authors performed a systematic review on pediatric OPHG in four databases: PubMed, EMBASE, Cochrane Library, and Google Scholar. We included studies that reported on the visual outcomes and complications of OPHG resection. A meta-analysis was performed and reported per the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. A total of 26 retrospective studies were included. Seven hundred ninety-seven pediatric patients with OPHG undergoing surgical resection were examined. A diagnosis of NF1 was confirmed in 9.7%. Gross total resection was achieved in 36.7%. Intraorbital optic pathway gliomas showed a significantly higher gross total resection rate compared to those located in the chiasmatic/hypothalamic region (75.8% vs. 9.6%). Postoperatively, visual acuity improved in 24.6%, remained unchanged in 68.2%, and worsened in 18.2%. Complications included hydrocephalus (35.4%), anterior pituitary dysfunction (19.6%), and transient diabetes insipidus (29%). Tumor progression post-resection occurred in 12.8%, through a mean follow-up of 53.5 months. Surgical resection remains an essential strategy for treating symptomatic and large pediatric OPHG and can result in favorable vision outcomes in most patients. Careful patient selection is critical. Patients should be monitored for hydrocephalus development postoperatively and followed up to assess for tumor progression and adjuvant treatment necessity.
Topics: Humans; Child; Postoperative Complications; Hypothalamic Neoplasms; Glioma; Optic Nerve Glioma; Neurosurgical Procedures; Treatment Outcome; Child, Preschool
PubMed: 38649470
DOI: 10.1007/s00381-024-06407-7 -
Cancers Sep 2022Background: OPG accounts for 3−5% of childhood central nervous system (CNS) tumors and about 2% of pediatric glial lesions. Methods: Article selection was performed by... (Review)
Review
Background: OPG accounts for 3−5% of childhood central nervous system (CNS) tumors and about 2% of pediatric glial lesions. Methods: Article selection was performed by searching PubMed, Web of Science, and Cochrane databases. Results: The pooled mortality rate was 0.12 (95%CI 0.09−0.14). Due to the unrepresentative data, improved and not changed outcomes were classified as favorable outcomes and worsened as unfavorable. Meta-analyses were performed to determine the rate of clinical and radiological favorable outcomes. In terms of visual assessment, the pooled rate of a favorable outcome in chemotherapy, radiotherapy, and surgery was 0.74, 0.81, and 0.65, respectively, and the overall pooled rate of the favorable outcome was 0.75 (95%CI 0.70−0.80). In terms of radiological assessment, the rate of a favorable outcome following chemotherapy, radiotherapy, and surgery was 0.71, 0.74, and 0.67, respectively, and the overall pooled rate of the favorable outcome is 0.71 (95%CI 0.65−0.77). The subgroup analysis revealed no significant difference in the rate of clinical and radiological favorable outcomes between the different treatment modalities (p > 0.05). Conclusion: Our analyses showed that each therapeutic modality represents viable treatment options to achieve remission for these patients.
PubMed: 36230704
DOI: 10.3390/cancers14194781 -
Cureus Aug 2022As oncology practice is rapidly shifting away from toxic chemotherapy, gene therapy provides a highly specific therapeutic approach for brain tumors. In this systematic... (Review)
Review
As oncology practice is rapidly shifting away from toxic chemotherapy, gene therapy provides a highly specific therapeutic approach for brain tumors. In this systematic review, we investigate gene therapy's status in pediatric brain tumors and future recommendations. The search was conducted systematically using PubMed, Cochrane, Google Scholar, and ClinicalTrials.gov databases. The field search used in the process was selected based on the keywords and Medical Subject Headings (MeSH), depending on the database used. We included cases of neurofibromatosis type 1 (NF1) brain tumors in all age groups with the additional inclusion of English language, free full text, articles published within the last 20 years, randomized controlled trials (RCTs), observational studies, systematic reviews, and meta-analyses. We excluded case reports, case studies, and editorials. The search identified a total of 1,213 articles from the databases. We included 19 studies with 16 narrative reviews, one systematic review, and two randomized clinical trials with 43 patients. After reviewing all data in the articles, we found that gene therapy can improve standard treatment efficacy when used as adjuvant therapy. It can be used to overcome barriers such as chemotherapy resistance by downregulating resistance genes. It is associated with mild toxicity when compared with other available treatment options, but given the overall poor prognosis in pediatric brain tumors, further studies are warranted.
PubMed: 36120213
DOI: 10.7759/cureus.27963 -
Frontiers in Surgery 2022Hemorrhage into optic pathway-hypothalamic glioma (OPHG) is rare. Variable clinical presentations and outcomes are associated with such pathology. We aim to present two... (Review)
Review
BACKGROUND
Hemorrhage into optic pathway-hypothalamic glioma (OPHG) is rare. Variable clinical presentations and outcomes are associated with such pathology. We aim to present two infants presented with OPHG and a systematic review of the literature.
METHODS
We describe two cases of infants presenting with sudden decreased vision, poor feeding, and irritability due to OPHG. Both patients underwent urgent craniotomy and subtotal resection followed by chemotherapy. We systematically reviewed the literature using PubMed, Google Scholar, and Embase. In addition, we included all English published reports for all ages discussing the optic pathway (optic nerve and optic chiasm) or hypothalamic glioma associated with hemorrhage from the year of the first reported case (1970) to January 2022.
RESULTS
Of 17,949, 44 articles met the inclusion criteria of this review. A total of 56 cases were described with a mean of 21.35 years (0.5-70), with the male gender 52% and the female gender 45%. The hemorrhage location was sellar/suprasellar in 43% cases. Histopathology of included cases was pilocytic astrocytoma in 41%, followed by pilomyxoid astrocytoma in 16% cases. The outcome was unfavorable; 37.5% cases showed improvement, whereas 18% cases resulted in death.
CONCLUSION
Apoplexy of the OPHG can be fatal and associated with poor outcomes. A systematic review of the literature has shown that younger age, pilocytic or pilomexyoid astrocytoma histopathology, and chiasmal/hypothalamic locations are associated with a higher risk of intertumoral hemorrhage and poor prognosis. Further genetic studies for OPHG may provide information for high-risk patients.
PubMed: 35733436
DOI: 10.3389/fsurg.2022.891556 -
Pediatric Neurosurgery 2022Novel targeted and tailored therapies can substantially improve the prognosis for optic pathway glioma (OPG), especially when implemented in a timely manner. However,...
INTRODUCTION
Novel targeted and tailored therapies can substantially improve the prognosis for optic pathway glioma (OPG), especially when implemented in a timely manner. However, their tremendous potential remains underestimated. Therefore, in this study, we provide an updated overview of the clinical trials, current trends, and future perspectives for OPG's novel therapeutic strategies.
METHODS
We completed an extensive literature review using the PubMed, MEDLINE, and ClinicalTrials.gov databases. We analyzed and reported the data following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.
RESULTS
Thioguanine, procarbazine, lomustine, and vincristine/vinblastine, as well as cisplatin-etoposide, provided excellent results in advanced-phase trials. Selumetinib and trametinib, two oral MEK inhibitors, have been approved for recurrent or refractory OPGs in association with the angiogenetic inhibitor bevacizumab. Among the mTOR inhibitors, everolimus and sirolimus showed the best results. Stereotactic radiosurgery and proton beam radiation therapy have advantages over conventional radiotherapy regimens. Timely treatment is imperative for acute visual symptoms with evidence of tumor progression. This latest evidence can help define a novel "T-Dimension" for pediatric OPG therapies.
CONCLUSION
The novel "T-Dimension" for pediatric OPGs is based on recent evidence-based treatments, including combination chemotherapy regimens, molecular targeted therapies, stereotactic radiosurgery, and proton beam radiation therapy. Additional clinical trials are essential for validating each of these new therapies.
Topics: Child; Combined Modality Therapy; Glioma; Humans; Lomustine; Radiosurgery; Vincristine
PubMed: 35588700
DOI: 10.1159/000524873 -
World Neurosurgery Aug 2022Optic pathway gliomas (OPGs) typically occur in the first decade of life and 40%-50% are not associated with neurofibromatosis 1 (NF1) (sporadic). Management strategies... (Review)
Review
OBJECTIVE
Optic pathway gliomas (OPGs) typically occur in the first decade of life and 40%-50% are not associated with neurofibromatosis 1 (NF1) (sporadic). Management strategies are often patient specific because of the variable and unpredictable course. No study has summarized the effect of treatment strategies on visual outcomes in the subset of pediatric patients with sporadic OPG.
METHODS
We conducted a systematic review to determine the nature of visual outcomes in pediatric patients with sporadic, non-NF1-associated OPG using the PubMed, Embase, Scopus, Cochrane, and CINAHL Plus databases. Visual outcomes were categorized as improved, unchanged, or deteriorated.
RESULTS
Of 1316 results, 31 articles were included. Treatment indications are unknown with full clinical detail. A total of 45.2% (14/31) reported deteriorated outcomes after treatment, 35.5% (11/31) no change, and 19.4% (6/31) improvement. Of radiotherapy studies, 50.0% (4/8) found no change, 37.5% (3/8) deterioration, and 12.5% (1/8) improvement. Of chemotherapy studies, 35.7% (5/14) each showed improvement and deterioration, whereas 28.6% (4/14) showed no change. Of surgical studies, 62.5% (5/8) indicated deterioration, and 37/5% (3/8) indicated no change. The singular study examining observation reported deterioration in visual outcomes. Factors associated with poor visual outcomes included signs and symptoms of visual decline at presentation, involvement of the intraorbital optic nerve, and intracranial hypertension requiring surgery. Causality cannot be determined from systematic review.
CONCLUSIONS
Most studies showed that vision in pediatric patients with sporadic OPG is stable to poor after observation, chemotherapy, radiotherapy, or surgery. Chemotherapy may be associated with most favorable visual outcomes.
Topics: Child; Follow-Up Studies; Humans; Neurofibromatosis 1; Optic Nerve Glioma; Optic Nerve Neoplasms; Retrospective Studies
PubMed: 35430402
DOI: 10.1016/j.wneu.2022.04.033 -
PloS One 2021To systematically review the evidence on the diagnostic accuracy and prognostic value of retinal optical coherence tomography (OCT) to detect visual acuity (VA) or... (Meta-Analysis)
Meta-Analysis
PURPOSE
To systematically review the evidence on the diagnostic accuracy and prognostic value of retinal optical coherence tomography (OCT) to detect visual acuity (VA) or visual field (VF) loss in children with a brain tumour.
METHODS
PubMed, Embase and Cochrane Library databases were searched from inception to February 2021. We included studies evaluating retinal OCT and standard visual function parameters (VA and or VF) in children with a brain tumour. Two authors independently extracted data from each included study. They also assessed the methodological quality of the studies using the QUADAS-2 or QUIPS tool. The diagnostic accuracy of OCT was evaluated with receiver operating characteristic analysis, sensitivity, specificity, positive predictive value and negative predictive value. The prognostic value of OCT was evaluated with predictive measures (odds ratio).
RESULTS
We included five diagnostic studies, with a total of 186 patients, all diagnosed with optic pathway glioma. No prognostic studies were eligible for inclusion. Included studies evaluated either retinal nerve fiber layer (RNFL) thickness or ganglion cell layer-inner plexiform layer (GCL-IPL) thickness. There was considerable heterogeneity between OCT devices, OCT protocols, visual function parameters and threshold values. Sensitivity and specificity for RNFL thickness measurement ranged from 60.0% to 100.0% and 76.6% to 100%, respectively. For GCL-IPL thickness measurement, area under the curve ranged from 0.91 to 0.98 for different diameters.
CONCLUSION
The literature regarding the diagnostic accuracy and prognostic value of OCT parameters in children with a brain tumour is scarce. Due to heterogeneity and a considerable risk of bias of included studies, we cannot draw solid conclusions regarding the accuracy of retinal OCT. Future research should investigate the potential of OCT as diagnostic and prognostic tool for the evaluation of the visual function and detection of visual impairment in children with any type of brain tumour.
Topics: Brain Neoplasms; Child; Humans; Optic Nerve Glioma; Prognosis; Tomography, Optical Coherence; Visual Acuity; Visual Field Tests
PubMed: 34941930
DOI: 10.1371/journal.pone.0261631 -
PloS One 2021Pediatric optic pathway glioma (OPG) can seriously decrease visual function in the case of progression. Systemic anticancer therapy (SAT) is considered the treatment of...
Pediatric optic pathway glioma (OPG) can seriously decrease visual function in the case of progression. Systemic anticancer therapy (SAT) is considered the treatment of first choice for unresectable OPG. New SAT modalities for the treatment of progressive OPG have been introduced in the last decade, including VEGF and MAPK pathway inhibition. This systematic review evaluated the effect of SAT on change in visual acuity and visual field in OPG. A systematic review was performed on SAT for OPG (January 1990 to August 2020). MEDLINE and EMBASE (Ovid) were searched for studies reporting on change in visual acuity and visual field after treatment with SAT for OPG. Overall, 11 series, including 358 patients, fulfilled the eligibility criteria. After follow-up of median 3.7 years (range: cessation of SAT- 8.2 years), improvement in binocular VA was found in 0-45% of studies, stability in 18-77% and a decrease in 0-82%. Two studies reported on change in visual field (improvement in 19% and 71% of patients), although either the change was not defined or the testing strategy was lacking. Considerable heterogeneity was present among the included studies, such as variety in the combinations of SAT administered, status of neurofibromatosis type 1, definition regarding change in visual acuity, 1- or 2-eye analysis, diversity in anatomic location, and extent of follow-up, all of which made meta-analysis inappropriate. This systematic review suggests that the impact of SAT in OPG on visual function is still unclear. The wide ranges reported on the efficacy of SAT and the observed heterogeneity highlight the need for prospective studies with uniform definitions of outcome parameters.
Topics: Adolescent; Child; Child, Preschool; Humans; Optic Nerve Glioma; Vision, Ocular
PubMed: 34673789
DOI: 10.1371/journal.pone.0258548 -
Journal of Pediatric Hematology/oncology May 2021Children with neurofibromatosis type I (NF1) have a higher predisposition for low-grade astrocytomas of the optic pathway, commonly referred to as optic pathway gliomas... (Meta-Analysis)
Meta-Analysis
Children with neurofibromatosis type I (NF1) have a higher predisposition for low-grade astrocytomas of the optic pathway, commonly referred to as optic pathway gliomas (OPGs). OPGs can result in visual deterioration. Treatment outcomes in OPG-NF1 management are often reported around tumor stabilization. We sought to compare vision outcomes associated with different OPG treatment strategies to inform about this important functional metric. A meta-analysis exploring the different modalities to treat children with OPG-NF1 was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines using multiple databases. Of the 113 articles identified in the search, 23 full text articles, representing 564 patients, were included for review. These articles included retrospective, prospective, and randomized controlled studies on observation (n=9), chemotherapy (n=19), radiation therapy (n=6), and surgery (n=7). Of the patients undergoing observation, 87% (60/69) demonstrated stable acuity. In the chemotherapy studies, 27.3% (72/264) demonstrated improved acuity/visual field and/or visual-evoked potential amplitudes, 39.4% (104/264) stable acuity, and 33.3% (88/264) deterioration. Both the radiation and surgical treatments reported worsening acuity at 90.9% (10/11) and 73.3% (11/15), respectively. Causal associations are not known. Indications for and timing of treatment choice warrant larger scale study to provide further understanding.
Topics: Child; Disease Management; Humans; Neurofibromatosis 1; Optic Nerve Glioma; Treatment Outcome; Vision, Ocular; Visual Acuity
PubMed: 33480655
DOI: 10.1097/MPH.0000000000002060 -
JPMA. the Journal of the Pakistan... Dec 2020To review evidence-based data on spontaneous retrogression of low-grade gliomas with respect to interval till regression, type of glioma and patient outcome. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To review evidence-based data on spontaneous retrogression of low-grade gliomas with respect to interval till regression, type of glioma and patient outcome.
METHODS
The systematic review comprised medical literature in English language published from January 1997 to January 2017 on Scopus, PubMed and Google Scholar databases to establish consensus about the possible mechanism of spontaneous regression, the role of therapeutic intervention and failure of management strategies in low-grade gliomas. Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were followed during the review.
RESULTS
Of the 176 articles identified, 73(41.5%) were shortlisted for detailed assessment. Of them, 10(13.7%) were included; 5(50%) case reports and 5(50%) case series. There were 23 cases of spontaneous regression; 15(65.2%) males and 8(34.7%) females. The interval of regression varied from 3 months to 15.5 years, and the most commonly presenting low-grade glioma type was optic pathway glioma 11(47.4%).
CONCLUSIONS
The phenomenon of regression was most evident in optic pathway glioma. Literature suggested that low-grade gliomas should undergo serial imaging before implying any therapeutic intervention. However, the evidencebased proof, large-scale experimental studies and ethical considerations are still required to standardise this strategy.
Topics: Astrocytoma; Brain Neoplasms; Consensus; Female; Glioma; Humans; Language; Male
PubMed: 33475560
DOI: 10.47391/JPMA.581