A multi-institutional retrospective pooled outcome analysis of molecularly annotated pediatric supratentorial ZFTA-fused ependymoma

Background: High-grade glioma (HGG) is associated with a dismal prognosis despite significant advances in adjuvant therapies, including chemotherapy, immunotherapy, and radiotherapy. Extent of resection continues to be the most important independent prognosticator of survival. This underlines the significance of increasing gross total resection (GTR) rates by using adjunctive intraoperative modalities to maximize resection with minimal neurological morbidity. 5-aminolevulinic acid (5-ALA) is the only US Food and Drug Administration–approved intraoperative optical agent used for fluorescence-guided surgical resection of gliomas. Despite several studies on the impact of intra-operative 5-ALA use on the extent of HGG resection, a clear picture of how such usage affects patient survival is still unavailable.Methods: A systematic review was conducted of all relevant studies assessing the GTR rate and survival outcomes [overall survival (OS) and progression-free survival (PFS)] in HGG. A meta-analysis of eligible studies was performed to assess the influence of 5-ALA-guided resection on improving GTR, OS, and PFS. GTR was defined as >95% resection.Results: Of 23 eligible studies, 19 reporting GTR rates were included in the meta-analysis. The pooled cohort had 998 patients with HGG, including 796 with newly diagnosed cases. The pooled GTR rate among patients with 5-ALA–guided resection was 76.8% (95% confidence interval, 69.1–82.9%). A comparative subgroup analysis of 5-ALA–guided vs. conventional surgery (controlling for within-study covariates) showed a 26% higher GTR rate in the 5-ALA subgroup (odds ratio, 3.8; P < 0.001). There were 11 studies eligible for survival outcome analysis, 4 of which reported PFS. The pooled mean difference in OS and PFS was 3 and 1 months, respectively, favoring 5-ALA vs. control (P < 0.001).Conclusions: This meta-analysis shows a significant increase in GTR rate with 5-ALA–guided surgical resection, with a higher weighted GTR rate (~76%) than the pivotal phase III study (~65%). Pooled analysis showed a small yet significant increase in survival measures associated with the use of 5-ALA. Despite the statistically significant results, the low level of evidence and heterogeneity across these studies make it difficult to conclusively report an independent association between 5-ALA use and survival outcomes in HGG. Additional randomized control studies are required to delineate the role of 5-ALA in survival outcomes in HGG.

IntroductionThis study aims to evaluate the differences between cystic vestibular schwannomas (CVS) and solid vestibular schwannomas (SVS) with respect to imaging characteristics, surgical duration, gross total resection (GTR) rates, and postoperative neurological functional outcomes. The goal is to inform individualized surgical planning and perioperative management.MethodsA retrospective analysis was conducted on 273 patients who underwent surgery for vestibular schwannomas. Patient data were divided into CVS and SVS groups. Comparisons were made regarding tumor volume, surgical duration, GTR rates, and postoperative preservation of facial nerve function and hearing. Multivariate analysis was used to identify independent predictors for postoperative functional outcomes.ResultsThe findings revealed that CVS cases had larger tumor volumes and longer surgical durations (96.6 minutes vs. 87.5 minutes) compared to SVS. Additionally, the rates of postoperative facial nerve function preservation and hearing preservation were lower in the CVS group, while no significant difference was found in GTR rates between the two groups. Multivariate analysis identified tumor volume and patient age as independent predictors of postoperative functional outcomes, whereas cystic changes had a limited impact on prognosis.DiscussionThese results underscore the importance of thorough preoperative assessment of tumor volume and cystic characteristics in vestibular schwannomas. Evaluating these factors can optimize individualized surgical strategies and perioperative management, with the aim of improving postoperative functional outcomes for patients.

Pituitary adenomas (PAs) represent a prevalent category of intracranial tumors, frequently resulting in endocrine dysfunction and neurological impairments. Transsphenoidal surgery (TSS) serves as the primary treatment modality, with the endoscopic transsphenoidal approach (ETSA) and microscopic transsphenoidal approach (MTSA) representing the two principal techniques. This systematic review and meta-analysis aimed to assess and compare the outcomes of ETSA and MTSA in treating PAs. A literature search was performed across PubMed, Scopus, Web of Science, and Cochrane Library. A total of 31 studies comprising 38,301 patients were included. The primary outcomes assessed were gross total resection (GTR) and cerebrospinal fluid (CSF) leak rates. Secondary outcomes encompass endocrine and surgical complications and mortality rates. Statistical analyses utilized R software, employing random-effects models. The analysis indicated insignificant differences in GTR rates between ETSA and MTSA (RR: 1.05, 95% CI [0.97, 1.15]) or in CSF leak rates (RR: 1.03, 95% CI [0.82, 1.31]). Meta-regression analysis revealed that nonfunctional tumors correlated with increased GTR rates, regardless of the surgical technique employed. No notable differences were detected in endocrine or surgical complications between the two methods. Notably, meta-regression analysis indicated that nonfunctional tumors and higher Knosp grade tumors are more likely to be completely resected. Moreover, larger preoperative tumor volume was significantly correlated with an increased risk of postoperative hypopituitarism. Nevertheless, a narrative review of total mortality demonstrated a higher rate in the MTSA group compared to the ETSA group (198 vs. 149 deaths), even with a larger patient cohort in the ETSA group. No publication bias was observed for GTR or CSF leak outcomes. ETSA and MTSA exhibit similar efficacy and safety profiles in treating PAs, with no significant differences in resection rates or complications. The findings highlight the necessity of personalized surgical planning, considering tumor characteristics and institutional expertise. Future research should prioritize long-term outcomes, patient-reported metrics, and the incorporation of emerging technologies to enhance surgical strategies for PAs.

ObjectiveThe aim of this study was to evaluate the clinical outcome of sphenoid wing meningioma with periorbital invasion (PI) after operation. MethodsSixty one patients with sphenoid wing meningioma were enrolled in this study. Their clinical conditions were monitored after the operation and followed up more than 5 years at the outpatient clinic of a single institution. Clinical and radiologic information of the patients were all recorded including the following parameters : presence of PI, presence of peri-tumor structure invasion, pathologic grade, extents of resection, presence of hyperostosis, exophthalmos index (EI), and surgical complications. We compared the above clinical parameters of the patients with sphenoid wing meningioma in the presence or absence of PI (non-PI), then linked the analyzed data with the clinical outcome of the patients. ResultsOf 61 cases, there were 14 PI and 47 non-PI patients. PI group showed a significantly higher score of EI (1.37±0.24 vs. 1.00±0.01, p<0.001), more frequent presence of hyperostosis (85.7% vs. 14.3%, p<0.001), and lower rate of gross total resection (GTR) (35.7% vs. 68.1%, p=0.032). The lower score of pre-operative EI, the absence of both PI and hyperostosis, smaller tumor size, and the performance of GTR were associated with lower recurrence rates in the univariate analysis. However, in the multivariate analysis, the performance of GTR was the only significant factor to determine the recurrence rate (p=0.043). The incidences of surgical complications were not statistically different between the subtotal resection (STR) and GTR groups, but it was strongly associated tumor size (p=0.017). ConclusionThe GTR group showed lower recurrence rate than the STR group without differences in the surgical complications. Therefore, the GTR is strongly recommended to treat sphenoid wing meningioma with PI for the better clinical outcome.

Introduction Endoscopic endonasal approach (EEA) and keyhole transcranial approaches (TCAs) are being increasingly used in anterior skull base meningioma (ASBM) surgery. Objective We compare tumor resection rates and complication profiles of EEA and supraorbital keyhole approach (SOKHA) with conventional TCAs. Methods Fifty-four patients with ASBM (olfactory groove meningioma [OGM], n = 19 and planum sphenoidale/tuberculum sellae meningioma [PSM/TSM], n = 35) operated at a single center over 7 years were retrospectively analyzed. Results The overall rate of gross total resection (GTR) was higher in OGM (15/19, 78.9%) than PSM-TSM group (23/35, 65.7%, p = 0.37). GTR rate with OGM was 90% and 75% with TCA and EEA. Death ( n = 1) following medical complication (TCA) and cerebrospinal fluid leak requiring re-exploration ( n = 2, one each in TCA and EEA) accounted for the major complications in OGM. For the PSM/TSM group, the GTR rates were 73.3% ( n = 11/15), 53.8% ( n = 7/13), and 71.4% ( n = 5/7) with TCA, EEA, and SOKHA, respectively. Seven patients (20%) of PSM-TSM developed major postoperative complications including four deaths (one each in TCA and SOKHA, and two in EEA groups) and three visual deteriorations. Direct and indirect vascular complications were common in lesser invasive approaches to PSM-TSM especially if the tumor has encased intracranial arteries. Conclusion No single approach is applicable to all ASBMs. TCA is still the best approach to obtain GTR but has tissue trauma-related problems. SOKHA may be a good alternative to TCA in selected PSM-TSMs, while EEA may be an alternate option in some OGMs. A meticulous patient selection is needed to derive reported results of EEA for PSM-TSM.

Despite the increasing utility of the endoscopic endonasal approach (EEA) for management of anterior skull base (ASB) pathologies, the optimal treatment strategy for olfactory groove meningiomas (OGM) remains unclear. This project sought to systematically compare outcomes of EEA management with conventional transcranial approach (TCA) for the treatment of OGMs. A systematic review was performed to identify studies that compared outcomes following EEA and TCA for OGMs. Data extracted from each study included gross total resection (GTR), incidence of cerebrospinal fluid (CSF) leaks, and post-operative complications including anosmia. The results of the search yielded 5 studies which met the criteria for inclusion and analysis. All studies compared TCA (n = 922) with EEA (n = 141) outcomes for OGMs. Overall, the rate of gross total resection (GTR) was lower among the endoscopic group (70.9%) relative to the transcranial group (91.5%). The rate of post-operative CSF leak was 6.3% vs. 25.5% for the transcranial and endoscopic groups, respectively. Post-operative anosmia was higher for patients undergoing EEA (95.9%) compared with patients in the transcranial group (37.4%). In this analysis, EEA was associated with a lower rate of GTR and higher incidences of CSF leaks and post-operative anosmia. However, with increasing surgeon familiarity of the endoscopic anatomy and technique for managing ASB pathologies, a nuanced approach may be used to minimize patient morbidity and widen the spectrum of skull base surgery.

Adult Supratentorial Ependymoma: The Mayo Clinic Experience

BackgroundPreoperative embolization has been used for intracranial meningiomas for nearly 40 years with varying preferences for embolic materials and limited comparative data on their efficacy.MethodsConsecutively treated patients from 2013 until...

Intraoperative magnetic resonance imaging in epilepsy surgery: A systematic review and meta-analysis

Objective To investigate the clinical effectiveness of magnetic resonance spectroscopy (MRS) combined with sodium fluorescein(FL) in the treatment of high grade gliomas(HGG). Methods From August 2013 to 2015 November,the clinical data of 72 supratentorial HGG(WHO grade Ⅲ-Ⅳ) patients who had received surgical treatment in our hospital were retrospectively studied,among whom 43 cases received MRS combined with intra-perative FL navigation(observation group),and 29 cases only received conventional surgery(control group). Post-operative radiotherapy and chemotherapy were applied for more than 3 months. Routine enhanced MRI were performed 24-48 hours after the operation to investigate the extent of tumor resection. Six months after the operation,the quality of life of patients was evaluated by using the Karnofsky score,and 1-year postoperative survival rate and progression-free survival(PFS) were observed. Results Postoperative MRI showed that the rate of gross total resection(GTR) in observation group was significantly higher than that in control group(72.09%vs.51.72%;χ2=23.88,P=0.001),and the GTR rate of WHO grade Ⅳ tumors was significantly higher than that of WHO grade Ⅲ tumors in observation group(92.86% vs.62.07%;χ2=6.06,P=0.042). The postoperative Karnofsky score in the observation group was significantly higher than that in control group(μ=2.34,P=0.021). The mean time of follow-up was(16.4±2.4) months(8-21 months) and there was no statistical significant difference between observation group and control group in 1-year survival rate(74.07% vs.77.50%;χ2=4.90,P=0.165) and PFS [(13.2±1.2) months vs.(12.7±2.0) months;χ2=7.26,P=0.067]. In observation group,the PFS of WHO grade Ⅳ patients was significantly higher than that in control group [(14.2±0.3) months vs.(10.0±1.1) months;χ2=11.03,P=0.031]. There was also no statistical significant difference between WHO grade Ⅳ tumors in two groups in terms of 1-year survival rate(71.43% vs.72.54%;χ2=5.33,P=0.089),and there was no statistical significant difference between WHO grade Ⅲ tumors in two groups in 1-year survival rate(75.86% vs. 72.22%;χ2=3.78,P=0.250) and in PFS [(13.7±1.4) months vs.(12.4±0.8) months;χ2=4.85,P=0.083]. Conclusions MRS combined with intraoperative FL navigation technology can improve the resection rate and improve survival quality of patients,and there is no evidence that MRS combined with intraoperative FL navigation prolong the overall survival of patients with high-grade gliomas. Different outcome may be found with longer follow-up and increased simple size.

Single Center Neurosurgical Outcomes and Trends in Endoscopic Endonasal Resection of 297 Sellar/Suprasellar Tumors Stratified by Duration of Neurosurgical Career

Introduction Intracranial tumors associated with PATZ1 gene rearrangements are extremely rare and poorly studied pathologies. These tumors are not currently included in the 2021 WHO Classification of CNS tumors. Objective To analyze and describe the clinical, morphological, and molecular characteristics of patients with PATZ1-rearranged intracranial tumors. Materials and Methods This study included eight pediatric patients with PATZ1-rearranged intracranial tumors. The initial morphological diagnoses included pleomorphic xanthoastrocytoma (4), ependymoma (2), papillary glioneuronal tumor (1), and high-grade glioma (1). Molecular genetic analyses were performed in all cases. Results The male-to-female ratio was 1:1 (4:4). The median age at the time of diagnosis was 8.1 years (range, 1–12.9). The initial symptoms included intracranial hypertension (4), seizures (3), and ataxia (1). The main radiological features were cystic-solid tumor structure (8) which located periventricular (7) and in the cerebellum (1). Gross total resection (GTR) was performed in four patients, while subtotal resection was performed in the other four cases. DNA methylation analysis was conducted for four patients, all of whom were classified as NET-PATZ1. RNA sequencing was performed for all eight patients, revealing a chimeric transcript of MN1::PATZ1 in five cases and EWSR1::PATZ1 in three cases. As first-line treatment, six patients received radiotherapy and one patient underwent chemotherapy. Among the patients who received GTR, two have been stable for 37 and 109 months, although four patients experienced disease progression, with two of them having multiple recurrences. At the time of follow-up, 7/8 patients are alive. The median follow-up is 3.6 years (1.6–9). Conclusion Intracranial tumors with PATZ1-rearrangements exhibit morphological and clinical variability, complicating their diagnosis and necessitating molecular diagnostic studies. In some cases, with GTR, observation may be sufficient.

Background/Objectives: Tuberculum sellae meningiomas (TSMs) constitute 5-10% of intracranial meningiomas, often causing visual impairment. Traditional microsurgical transcranial approaches (MTAs) have been effective, but the emergence of innovative surgical trajectories, such as endoscopic endonasal approaches (EEAs), has sparked debate. While EEAs offer advantages like reduced brain retraction, they are linked to higher cerebrospinal fluid leak (CSF leak) risk. This meta-analysis aims to comprehensively compare the efficacy and safety of EEAs and MTAs for the resection of TSMs, offering insights into their respective outcomes and complications. Methods: A comprehensive literature review of the databases PubMed, Ovid MEDLINE, and Ovid EMBASE was conducted for articles published on TSMs treated with either EEA or MTA until 2024. The systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. Meta-analysis was performed to estimate pooled event rates and assess heterogeneity. Fixed- and random-effects were used to assess 95% confidential intervals (CIs) of presenting symptoms, outcomes, and complications. Results: A total of 291 papers were initially identified, of which 18 studies spanning from 2000 to 2024 met the inclusion criteria. The exclusion of 180 articles was due to reasons such as irrelevance, non-reporting of selected results, systematic literature review or meta-analysis, and a lack of details on method/results. The 18 studies comprised a total sample of 1093 patients: 444 patients who underwent EEAs and 649 patients who underwent MTAs for TSMs. Gross total resection (GTR) rates ranged from 80.9% for EEAs to 79.8% for MTAs. The rate of visual improvement was 86.6% in the EEA group and 65.4% in the MTA group. The recurrence rate in the EEA group was 6.9%, while it was 5.1% in MTA group. The postoperative complications analyzed were CSF leak, infections, dysosmia, intracranial hemorrhage (ICH), and endocrine disorders. The rate of CSF leak was 9.8% in the EEA group and 2.1% in MTA group. The rate of infections in the EEA group was 5.7%, while it was 3.7% in the MTA group. The rate of dysosmia ranged from 10.3% for MTAs to 12.9% for EEAs. The rate of ICH in the EEA group was 0.9%, while that in the MTA group was 3.8%. The rate of endocrine disorders in the EEA group was 10.8%, while that in the MTA group was 10.2%. No significant difference was detected in the rate of GTR between the EEA and MTA groups (OR 1.15, 95% CI 0.7-0.95; p = 0.53), while a significant benefit in visual outcomes was shown in EEAs (OR 3.54, 95% CI 2.2-5.72; p < 0.01). There was no significant variation in the recurrence rate between EEA and MTA groups (OR 0.92, 95% CI 0.19-4.46; p = 0.89). While a considerably increased chance of CSF leak from EEAs was shown (OR 4.47, 95% CI 2.52-7.92; p < 0.01), no significant difference between EEA and MTA groups was detected in the rate of infections (OR 1.92, 95% CI 0.73-5.06; p = 0.15), the rate of dysosmia (OR 1.25, 95% CI 0.31-4.99; p = 0.71), the rate of ICH (OR 0.61, 95% CI 0.20-1.87; p = 0.33), and the rate of endocrine disorders (OR 1.16, 95% CI 0.69-1.95; p = 0.53). Conclusions: This meta-analysis suggests that both EEAs and MTAs are viable options for TSM resection, with distinct advantages and drawbacks. The EEAs demonstrate superior visual outcomes in selected cases while GTR and recurrence rates support the overall effectiveness of MTAs and EEAs. Endoscopic endonasal approaches had a higher chance of CSF leaks, but there are no appreciable variations in other complications. These results provide additional insights regarding patient outcomes in the intricate clinical setting of TSMs.

The aim of this study was to quantitatively assess the role of intraoperative high-field 3-T MRI (3T-iMRI) in improving the gross-total resection (GTR) rate and the extent of resection (EOR) in endoscopic transsphenoidal surgery (TSS) for pituitary adenomas. Radiological and clinical data from a prospective database were retrospectively analyzed. Volumetric measurements of adenoma volumes pre-, intraoperatively, and 3 months postoperatively were performed in a consecutive series of patients who had undergone endoscopic TSS. The quantitative contribution of 3T-iMRI was measured as a percentage of the additional rate of GTR and of the EOR achieved after 3T-iMRI. The cohort consisted of 50 patients (51 operations) harboring 33 nonfunctioning and 18 functioning pituitary adenomas. Mean adenoma diameter and volume were 21.1 mm (range 5-47 mm) and 5.23 cm(3) (range 0.09-22.14 cm(3)), respectively. According to Knosp's classification, 10 cases were Grade 0; 8, Grade 1; 17, Grade 2; 12, Grade 3; and 4, Grade 4. Gross-total resection was the surgical goal (targeted [t]GTR) in 34 of 51 operations and was initially achieved in 16 (47%) of 34 at 3T-iMRI and in 30 (88%) of 34 cases after further resection. In this subgroup, the EOR increased from 91% at 3T-iMRI to 99% at the 3-month MRI (p < 0.05). In the 17 cases in which subtotal resection (STR) had been planned (tSTR), the EOR increased from 79% to 86% (p < 0.05) and GTR could be achieved in 1 case. Intrasellar remnants were present in 20 of 51 procedures at 3T-iMRI and in only 5 (10%) of 51 procedures after further resection (median volume 0.15 cm(3)). Overall, the use of 3T-iMRI led to further resection in 27 (53%) of 51 procedures and permitted GTR in 15 (56%) of these 27 procedures; thus, the GTR rate in the entire cohort increased from 31% (16 of 51) to 61% (31 of 51) and the EOR increased from 87% to 95% (p < 0.05). The use of high-definition 3T-iMRI allowed precise visualization and quantification of adenoma remnant volume. It helped to increase GTR and EOR rates in both tGTR and tSTR patient groups. Moreover, it helped to achieve low rates of intrasellar remnants. These data support the use of 3T-iMRI to achieve maximal, safe adenoma resection.

Comparison of fluorescein sodium, 5-ALA, and intraoperative MRI for resection of high-grade gliomas: A systematic review and network meta-analysis