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Frontiers in Oncology 2023The uncommon -altered primary central nervous system (CNS) tumors were recently added to the World Health Organization 2021 classification under the name Astroblastoma,...
The uncommon -altered primary central nervous system (CNS) tumors were recently added to the World Health Organization 2021 classification under the name Astroblastoma, -altered. Another term used to describe them, "High-grade neuroepithelial tumor with alteration" (HGNET-MN1), makes reference to their distinct epigenetic profile but is currently not a recommended name. Thought to occur most commonly in children and predominantly in females, -altered CNS tumors are associated with typical but not pathognomonic histological patterns and are characterized by a distinct DNA methylation profile and recurrent fusions implicating the (meningioma 1) gene. Diagnosis based on histological features alone is challenging: most cases with morphological features of astroblastoma (but not all) show these molecular features, whereas not all tumors with fusions show astroblastoma morphology. There is large variability in reported outcomes and detailed clinical and therapeutic information is frequently missing. Some patients experience multiple recurrences despite multimodality treatment, whereas others experience no recurrence after surgical resection alone, suggesting large clinical and biological heterogeneity despite unifying epigenetic features and recurrent fusions. In this report, we present the demographics, tumor characteristics, treatment, and outcome (including patient-reported outcomes) of three adults with -altered primary CNS tumors diagnosed genome-wide DNA methylation and RNA sequencing. All three patients were females and two of them were diagnosed as young adults. By reporting our neuropathological and clinical findings and comparing them with previously published cases we provide insight into the clinical heterogeneity of this tumor. Additionally, we propose a model for prospective, comprehensive, and systematic collection of clinical data in addition to neuropathological data, including standardized patient-reported outcomes.
PubMed: 36741001
DOI: 10.3389/fonc.2023.1099618 -
Neuro-oncology Jun 2023Quantitative imaging analysis through radiomics is a powerful technology to non-invasively assess molecular correlates and guide clinical decision-making. There has been...
BACKGROUND
Quantitative imaging analysis through radiomics is a powerful technology to non-invasively assess molecular correlates and guide clinical decision-making. There has been growing interest in image-based phenotyping for meningiomas given the complexities in management.
METHODS
We systematically reviewed meningioma radiomics analyses published in PubMed, Embase, and Web of Science until December 20, 2021. We compiled performance data and assessed publication quality using the radiomics quality score (RQS).
RESULTS
A total of 170 publications were grouped into 5 categories of radiomics applications to meningiomas: Tumor detection and segmentation (21%), classification across neurologic diseases (54%), grading (14%), feature correlation (3%), and prognostication (8%). A majority focused on technical model development (73%) versus clinical applications (27%), with increasing adoption of deep learning. Studies utilized either private institutional (50%) or public (49%) datasets, with only 68% using a validation dataset. For detection and segmentation, radiomic models had a mean accuracy of 93.1 ± 8.1% and a dice coefficient of 88.8 ± 7.9%. Meningioma classification had a mean accuracy of 95.2 ± 4.0%. Tumor grading had a mean area-under-the-curve (AUC) of 0.85 ± 0.08. Correlation with meningioma biological features had a mean AUC of 0.89 ± 0.07. Prognostication of the clinical course had a mean AUC of 0.83 ± 0.08. While clinical studies had a higher mean RQS compared to technical studies, quality was low overall with a mean RQS of 6.7 ± 5.9 (possible range -8 to 36).
CONCLUSIONS
There has been global growth in meningioma radiomics, driven by data accessibility and novel computational methodology. Translatability toward complex tasks such as prognostication requires studies that improve quality, develop comprehensive patient datasets, and engage in prospective trials.
Topics: Humans; Meningioma; Prospective Studies; Neoplasm Grading; Meningeal Neoplasms
PubMed: 36723606
DOI: 10.1093/neuonc/noad028 -
Cancers Jan 2023The safety and efficacy of various pharmacotherapeutic regimens on refractory meningiomas have been the focus of investigations. We present a comprehensive review of the... (Review)
Review
The safety and efficacy of various pharmacotherapeutic regimens on refractory meningiomas have been the focus of investigations. We present a comprehensive review of the previous efforts and the current state of ongoing clinical trials. A PRISMA-compliant review of the MEDLINE and ClinicalTrial.gov databases of the National Library of Medicine were performed. The primary outcomes of interest for included articles were radiographic response, overall survival, progression-free survival, six-month progression-free survival, and adverse events. Overall, 34 completed trials and 27 ongoing clinical trials were eligible. Six-month progression-free survival was reported in 6-100% of patients in the completed studies. Hematological disorders were the most common adverse events. Of the ongoing clinical trials identified, nine studies are phase I clinical trials, eleven are phase II trials, two are phase I and II trials, one is phase II and III, and two trials do not have a designated phase. Currently, there is no effective chemotherapy for refractory or recurrent meningiomas. Several promising targeted agents have been developed and are currently being investigated in the hope of identifying novel therapeutic strategies for the treatment of this pathology.
PubMed: 36672431
DOI: 10.3390/cancers15020483 -
European Journal of Medical Research Jan 2023Primary central nervous system (CNS) tumors are a heterogeneous group of neoplasms, including benign and malignant tumors. Since there are many heterogeneities in the... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Primary central nervous system (CNS) tumors are a heterogeneous group of neoplasms, including benign and malignant tumors. Since there are many heterogeneities in the prevalence reported in previous studies on this type of tumor, this study was performed to determine the overall prevalence of different primary CNS tumors.
METHOD
The study was conducted as a systematic review and meta-analysis by searching international databases, including PubMed, Scopus, Science Direct, Web of science, and the Google Scholar search engine until August 2020. After transferring the studies to information management software (EndNote) and eliminating duplicate studies, the remaining studies were reviewed based on inclusion and exclusion criteria according to three stages of primary and secondary evaluation and qualitative evaluation. Comprehensive Meta-Analysis software, Begg, Mazumdar, and I tests were used for data analysis, publication bias analysis, and heterogeneity analysis, respectively.
RESULTS
After performing the systematic review steps, 80 studies were included for final analysis. Based on 8 studies, the prevalence of brain tumors was 70.9%. Also, studies on 7 other studies showed that the prevalence of spinal tumors was 12.2%. A review of 14 studies showed that the prevalence of neuroepithelial tumors was 34.7%. The analysis of 27 studies reported a prevalence of glioma tumors of 42.8%. Analyses performed on other studies showed that the prevalence of pituitary adenomas was 12.2%, embryonal tumors 3.1%, ependymal tumors 3.2%, meningiomas 24.1%, glial tumors 0.8%, astrocytic 20.3%, oligodendroglial 3.9%, glioblastoma 17.7%, schwannoma 6.7%, medulloblastoma 7.7% and Polycystic astrocytomas 3.8%.
CONCLUSION
As a result, it can be stated that brain tumors are the most common type of primary CNS tumors. It was also observed that tumors involving neuroepithelial cells are more common in patients than other types of tumors.
Topics: Humans; Prevalence; Central Nervous System Neoplasms; Brain Neoplasms; Glioblastoma
PubMed: 36670466
DOI: 10.1186/s40001-023-01011-y -
Transgender Health Nov 2022Gender-affirming hormone therapy is critical to the management of transgender persons. Cyproterone acetate (CPA) is a synthetic, progesterone-like compound commonly used... (Review)
Review
BACKGROUND
Gender-affirming hormone therapy is critical to the management of transgender persons. Cyproterone acetate (CPA) is a synthetic, progesterone-like compound commonly used in high doses as gender-affirming progestogen therapy in transgender women. An association between high-dose CPA and the development and growth of intracranial meningioma, including case reports in transgender women, has been described. This systematic review summarizes these cases at the patient level and discusses their management.
METHODS
This systematic review was registered with PROSPERO (CRD42020191965). A detailed search of the PubMed, EMBASE, and Web of Science electronic bibliographic databases was performed (inception-December 20, 2020). Two review authors independently completed screening, data extraction, and risk of bias assessment in duplicate.
RESULTS
Nine records were included describing (=12) individual case reports and (=35) intracranial meningiomas. The median age at presentation was 48 years (interquartile range [IQR], 43-55 years), most frequent daily CPA doses were 50 mg/day (=5) and 100 mg/day (=5), and the median duration of CPA use was 9.5 years (IQR, 6.5-17.5 years). Multiple meningiomas were common (=7). For most cases (=10), surgical resection was the initial preferred management strategy, but two were successfully managed by CPA cessation.
CONCLUSIONS
Transgender women receiving high doses of CPA may be at increased risk of intracranial meningioma development and/or growth, although this remains a rare disease. For presumed CPA-associated meningioma, drug cessation appears to be an appropriate management strategy when surgery is not imminently required to manage raised intracranial pressure or prevent neurological deterioration. Given the importance of gender-affirming hormone therapy to transgender persons, a suitable alternative hormone regimen should be offered, although the use of CPA in both high doses and for prolonged periods of time is now in decline.
PubMed: 36644118
DOI: 10.1089/trgh.2021.0025 -
Brain & Spine 2022•OGM surgery is much more complex than a simple debate of "from above or from below" (transcranial vs endoscopic).•Lateral Sub-frontal and Superior Interhemispheric... (Review)
Review
Olfactory Groove Meningiomas: Comprehensive assessment between the different microsurgical transcranial approaches and the Endoscopic Endonasal Approaches, systematic review and metanalysis on behalf of the EANS skull base section.
•OGM surgery is much more complex than a simple debate of "from above or from below" (transcranial vs endoscopic).•Lateral Sub-frontal and Superior Interhemispheric seem the most effective, superior and versatile approaches for OGM.•Minimally Invasive Transcranial approaches showed no inferiority in OGM sized <4 cm.•Endoscopic Endonasal Approaches showed inferior results in surgical and in functional outcomes for OGM.
PubMed: 36605386
DOI: 10.1016/j.bas.2022.101661 -
Cancers Dec 2022Spinal meningiomas are the most common primary intradural spinal tumors. Although they are a separate entity, a large portion of the knowledge on spinal meningiomas is... (Review)
Review
BACKGROUND
Spinal meningiomas are the most common primary intradural spinal tumors. Although they are a separate entity, a large portion of the knowledge on spinal meningiomas is based on findings in intracranial meningiomas. Therefore, a comprehensive review of all the literature on spinal meningiomas was performed.
METHODS
Electronic databases were searched for all studies on spinal meningiomas dating from 2000 and onward. Findings of matching studies were pooled to strengthen the current body of evidence.
RESULTS
A total of 104 studies were included. The majority of patients were female (72.83%), elderly (peak decade: seventh), and had a world health organization (WHO) grade 1 tumor (95.7%). Interestingly, the minority of pediatric patients had a male overrepresentation (62.0% vs. 27.17%) and higher-grade tumors (33.3% vs. 4.3%). Sensory and motor dysfunction and pain were the most common presenting symptoms. Despite a handful of studies reporting promising findings associated with the use of non-surgical treatment options, the literature still suffers from contradictory results and limitations of study designs.
CONCLUSIONS
Elderly females with WHO grade 1 tumors constituted the stereotypical type of patient. Compared to surgical alternatives, the evidence for the use of non-surgical treatments is still relatively weak.
PubMed: 36551736
DOI: 10.3390/cancers14246251 -
Cancers Dec 2022Background: Most of the knowledge on spinal meningiomas is extrapolated from their intracranial counterparts, even though they are considered separate entities. This... (Review)
Review
Background: Most of the knowledge on spinal meningiomas is extrapolated from their intracranial counterparts, even though they are considered separate entities. This review aimed to systematically summarize studies covering different aspects of spinal meningiomas and their management. Methods: Databases were searched for all studies concerning spinal meningiomas dating from 2000 and onwards. When possible, a meta-analysis was performed. Results: Neurological outcomes of surgery were consistently favorable across studies, with a complication rate of 7.9% and 78.9% of the patients demonstrating good postoperative neurological function (McCormick score 1−2). The most relevant predictors of unfavorable outcomes were poor preoperative status, longer time from diagnosis to surgery, and surgery of recurrent tumors. The recurrence rate after surgery was estimated at 6%. Meta-analysis and/or survival analysis revealed that higher WHO grade (p < 0.001), higher Simpson grade (p < 0.001), ventral tumor location (p = 0.02), and male sex (p = 0.014) were all associated with higher odds of recurrence. However, the meta-analysis did not show any difference between Simpson grade 1 and grade 2 with respect to the odds of recurrence (p = 0.94). Surgery provided immediate and durable health-related quality-of-life improvement, as well as a high frequency of return to work. Conclusion: Spinal meningioma surgery is a relatively safe procedure with a low risk of tumor recurrence and high likelihood of favorable postoperative outcomes.
PubMed: 36551706
DOI: 10.3390/cancers14246221 -
Journal of Neuro-oncology Dec 2022Spheno-orbital meningiomas are rare tumors, accounting for up to 9% of all intracranial meningiomas. Patients commonly present with proptosis, and visual deficits. These... (Review)
Review
Endoscopic endonasal approach for resection of a recurrent spheno-orbital meningioma resulting in complete resolution of visual symptoms: A case report and review of literature.
PURPOSE
Spheno-orbital meningiomas are rare tumors, accounting for up to 9% of all intracranial meningiomas. Patients commonly present with proptosis, and visual deficits. These slow growing tumors are hard to resect due to extension into several anatomical compartments, resulting in recurrence rates as high as 35-50%. Although open surgical approaches have been historically used for resection, a handful of endoscopic approaches have been reported in recent years. We aimed to review the literature and describe a case of spheno-orbital meningioma with severe vision loss which was resected with an endoscopic endonasal approach achieving complete resolution of visual symptoms.
METHODS
A systematic review of literature was conducted in accordance with the PRISMA guidelines. PubMed, Cochrane, and Web of Science databases were queried for spheno-orbital meningiomas resected via an endoscopic endonasal approach. Furthermore, the presentation, surgical management, and post-operative outcomes of a 53-year-old female with a recurrent spheno-orbital meningioma are described.
RESULTS
The search yielded 26 articles, of which 8 were included, yielding 19 cases. Average age at presentation was 60.5 years (range: 44-82), and 68.4% of patients were female. More than half of the cases achieved subtotal resection. Common complications associated with endoscopic endonasal surgery included CN V2 or CN V2/V3 hypoesthesia. Following surgical intervention, visual acuity and visual field remained stable or improved in the majority of the patients.
CONCLUSION
Endoscopic approaches are slowly gaining momentum for treatment of spheno-orbital meningiomas. Further studies on the clinical benefits of this approach on patient outcomes and post-operative complications is warranted.
Topics: Humans; Female; Adult; Middle Aged; Aged; Aged, 80 and over; Male; Meningioma; Sphenoid Bone; Orbital Neoplasms; Neurosurgical Procedures; Treatment Outcome; Neoplasm Recurrence, Local; Meningeal Neoplasms; Retrospective Studies
PubMed: 36445608
DOI: 10.1007/s11060-022-04141-1 -
Cancers Nov 2022Infratemporal fossa (ITF) tumors represent various pathologies and are seldom described in the literature, reflecting their rarity. Here we review the literature on... (Review)
Review
BACKGROUND
Infratemporal fossa (ITF) tumors represent various pathologies and are seldom described in the literature, reflecting their rarity. Here we review the literature on tumors invading ITF and describe patient characteristics, treatment strategies, and clinical outcomes.
METHODS
Relevant articles were retrieved from PubMed, Scopus, and Cochrane. A systematic review and meta-analysis were conducted on the clinical presentation, treatment protocols, and clinical outcomes.
RESULT
A total of 27 articles containing 106 patients with ITF tumors (median tumor size: 24.3 cm [interquartile range, 15.2-42 cm]) were included (median age: 46 years [interquartile range, 32-55 years]; 59.4% were males]). Of the confirmed tumor pathology data, schwannomas ( = 24; 26.1%) and meningiomas ( = 13; 14.1%) were the most common tumors. Facial hypoesthesia ( = 22; 18.5%), auricular/preauricular pain ( = 20; 16.8%), and headaches ( = 11; 9.2%) were the most common presenting symptoms. Of patients who had surgical resection ( = 97; 95.1%), 70 (73.7%) had transcranial surgery (TCS) and 25 (26.3%) had endoscopic endonasal surgery (EES). Among available details on the extent of resection ( = 84), gross-total resection (GTR) was achieved in 62 (73.8%), and 5 (6.0%) had biopsy only. Thirty-five (33.0%) patients had postoperative complications. Among cases with available data on reconstruction techniques ( = 8), four (50%) had adipofascial antero-lateral thigh flap, three (37.5%) had latissimus dorsi free flap, and one (12.5%) had antero-lateral thigh flap. Fourteen (13.2%) patients had adjuvant chemotherapy, and sixteen (15.1%) had adjuvant radiotherapy. During a median follow-up time of 28 months (IQR, 12.25-45.75 months), 15 (14.2%) patients had recurrences, and 18 (17.0%) patients died. The median overall survival (OS) time was 36 months (95% confidence interval: 29-41 months), and the 5-year progression-free survival (PFS) rate was 61%.
CONCLUSION
Various tumor types with different biological characteristics invade the ITF. The present study describes patient demographics, clinical presentation, management, and outcomes. Depending on the tumor type and patient condition, patient-tailored management is recommended to optimize treatment outcomes.
PubMed: 36358837
DOI: 10.3390/cancers14215420