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The efficacy of radiosurgery for neurofibromatosis type 2 (NF2)-associated vestibular schwannoma (VS) remains debatable. We retrospectively analyzed radiosurgical outcomes for NF2-associated VS compared to sporadic VS using our database of 422 consecutive VS patients. Twenty-five patients with 30 NF2-associated VSs with a mean follow-up of 121 months were identified. NF2-associated VSs exhibited excellent tumor control (10-year cumulative rate, 92% vs. 92% in sporadic VSs; p = 0.945) and worse overall survival (73% vs. 97%; p = 0.005), mainly due to tumor progression other than the treated VSs. The presence of NF2 was not associated with failed tumor control via multivariate Cox proportional hazard analyses. No difference in radiation-induced adverse events (RAEs) was confirmed between cohorts, and prescription dose (hazard ratio 8.30, 95% confidence interval 3.19–21.62, p < 0.001) was confirmed as a risk for cranial nerve injuries via multivariate analysis. Further analysis after propensity score matching using age, volume, and sex as covariates showed that NF2-associated VSs exhibited excellent local control (100% vs. 93%; p = 0.240) and worse overall survival (67% vs. 100%; p = 0.002) with no significant difference in RAEs. Excellent long-term tumor control and minimal invasiveness may make radiosurgery a favorable therapeutic option for NF2 patients with small to medium VS, preferably with non-functional hearing or deafness in combination with postoperative tumor growth or progressive non-operated tumors, or with functional hearing by patients' wish. [ABSTRACT FROM AUTHOR]

To determine whether accurate inferior petrosal sinus sampling (IPSS) tumor lateralization is associated with improved clinical outcomes following the surgical treatment of Cushing disease. The presented study was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Data regarding patient demographics, IPSS tumor lateralization, and postoperative endocrinologic outcomes were abstracted and pooled with random effects meta-analysis models. Additional meta-regression models were used to examine the association between the accuracy of IPSS tumor lateralization and postoperative outcomes (recurrence/persistence or remission/cure). Statistical analyses were performed using the Comprehensive Meta-Analysis software (significance of P < 0.05). Seventeen eligible articles were identified, yielding data on 461 patients. Within average follow-up duration (∼59 months), the rate of correct IPSS tumor lateralization was 69% [95% confidence interval: 61%, 76%], and the rate of postoperative remission/cure was 78% [67%, 86%]. Preoperative IPSS tumor lateralization was concordant with magnetic resonance imaging lateralization for 53% of patients [40%, 66%]. There was no significant association between the rate of correct IPSS tumor lateralization and postoperative remission/cure among study-level data (P = 0.735). Additionally, there was no association among subgroup analyses for studies using stimulatory agents during IPSS (corticotropin-releasing hormone or desmopressin, P = 0.635), nor among subgroup analyses for adult (P = 0.363) and pediatric (P = 0.931) patients. Limited data suggest that the rate of correct IPSS tumor lateralization may not be positively associated with postoperative remission or cure in patients with Cushing disease. These findings bring into question the utility of IPSS tumor lateralization in the context of preoperative planning and surgical approach rather than confirming a pituitary source. [ABSTRACT FROM AUTHOR]

Background: We previously reported that early alendronate administration accelerated bone formation and improved the quality of repaired cartilage in the donor site in rabbits. To investigate whether alendronate administration has effects in humans similar to those observed in rabbits.Methods: The study cohort included 35 patients over the age of 12-years old who underwent mosaicplasty without osteoporotic therapy from March 2011 to October 2012. The donor sites were medial or lateral in the patellofemoral joint. Placebo (P) or Bonalon containing 35 mg of alendronate (A) was administered orally every week for 8 weeks. The cohort comprised 15 male and 20 female, including 14 right and 21 left knees. The mean age at the time of surgery was 57.1 years. Bone formation was examined using computer tomography and lateral knee radiography, and cartilage formation was examined using magnetic resonance imaging (MRI), second-look assessment, and intraoperative acoustic evaluation. The clinical outcomes were assessed using the Japanese Orthopaedic Association knee score and visual analog scale (VAS). Bone and cartilage formation in the donor site and clinical outcomes were assessed at 3, 6, and 12 months after mosaicplasty.Results: The ratio of TRAP-5b in group A was significantly smaller than that in group P at 2 and 8 weeks after mosaicplasty. The extent of bone formation in the donor sites in group A was significantly greater than that in group P at 3 and 6 months after mosaicplasty. Cartilage formation did not differ significantly between the two groups as determined by MRI, macroscopic assessment, and intraoperative acoustic evaluation. Clinical outcomes did not differ significantly between the two groups, and no negative clinical outcomes were observed.Conclusion: Early alendronate administration accelerated bone formation but not cartilage formation in the mosaicplasty donor site in humans. [ABSTRACT FROM AUTHOR]

Intracranial artery stenosis (ICAS) is a significant contributor to ischemic stroke, with the RNF213 p.Arg4810Lys variant identified as a related genetic factor. We explored the clinical outcomes of the RNF213 genotype in patients with asymptomatic ICAS. Between November 2011 and March 2019, 139 patients with asymptomatic ICAS were enrolled in this study. Genotyping for RNF213 p.Arg4810Lys was performed using Sanger sequencing. A comprehensive analysis was conducted to compare the RNF213 genotype with background characteristics and clinical outcomes such as ipsilateral ischemic cerebrovascular events and stenosis progression. RNF213 p.Arg4810Lys was found in 25% of cases, revealing distinct clinical features between carriers and non-carriers. The incidence of ipsilateral ischemic cerebrovascular events was 4.3% (6/139 cases), and stenosis progression was observed in 13% (18/139 cases) during a mean follow-up period of 58 months. Stenosis progression rates were notably higher in the RNF213 variant group (25.7%; 9/35 cases) than in the RNF213 wild-type group (8.7%; 9/104 cases). Cumulative stenosis progression rate was significantly higher in the RNF213 variant group than in the RNF213 wild-type group (log-rank test, P = 0.0004). Multivariate Cox regression analysis indicated a significant association between the RNF213 p.Arg4810Lys variant and an increased risk of stenosis progression (P = 0.03, odds ratio 3.2; 95% confidence interval, 1.1-9.0). The RNF213 p.Arg4810Lys variant exhibits clinical disparities in asymptomatic ICAS and is notably linked to a heightened risk of stenosis progression. These results suggest a distinct difference in the vascular stenosis mechanism associated with this variant, warranting further investigation into its clinical implications and potential mechanistic insights., Competing Interests: Declarations Ethics Approval All participants provided written informed consent, with documents approved by the Institutional Review Board of each participating hospital or institution. The Ethical Committee of the University of Tokyo approved this study (approval number, G10026; approval date, September 12, 2011). The research conducted in our manuscript adheres to ethical guidelines outlined in the Declaration of Helsinki. Conflict of Interest The authors declare no competing interests., (© 2024. The Author(s).)

Background: Medial soft tissue release is occasionally performed to achieve mediolateral ligament balance in total knee arthroplasty (TKA), whose sequential effect on mediolateral and anteroposterior stability remains unclear. This study aimed to quantitatively evaluate the difference in mediolateral and anteroposterior stability according to a sequential medial soft tissue release in TKA., Methods: Cruciate-retaining TKA was performed in six cadaveric knees. Medial and lateral joint gaps, varus-valgus angle, and tibial anterior and posterior translations relative to the femur with pulling and pushing forces, respectively, were measured. All measurements were performed at full extension and 45° and 90° flexion after release of the deep medial collateral ligament (MCL) (stage 1), the posteromedial capsule (stage 2), and the superficial MCL (stage 3). Mediolateral and anteroposterior stability were compared between stages, and correlations between mediolateral and anteroposterior stability were analyzed., Results: Medial joint gap significantly increased from stages 1 to 3 by 3.2 mm, 6.8 mm, and 7.2 mm at extension, 45° flexion, and 90° flexion, respectively, and from stages 2 to 3 by 3.5 mm at extension. Varus-valgus angle was varus at stage 2, which turned to valgus at stage 3 (-2.7° to 0.8°, -2.2° to 4.3°, and -5.5° to 2.5° at extension, 45° flexion, and 90° flexion, respectively). Anterior translation at 90° flexion significantly increased from stages 1 and 2 to stage 3 by 11.5 mm and 8.2 mm, respectively, which was significantly correlated with medial gap (r = 0.681) and varus-valgus angle (r = 0.495)., Conclusions: Medial soft tissue release also increased tibial anterior translation as well as medial joint gap, and medial joint gap and tibial anterior translation were significantly correlated. Surgeons should be careful not to create too large medial joint gap and tibial anterior translation in flexion by excessive medial release up to the superficial MCL for achieving an equal mediolateral joint gap in extension., (© 2024. The Author(s).)

Background and Objectives: Patients with brain arteriovenous malformations (BAVMs) sometimes experience migraine-like chronic headaches. Reportedly, this symptom is common in occipital lobe BAVMs; however, the exact prevalence has not been clarified, and the details of visual auras have been scarcely reported. Furthermore, no comprehensive studies exist on the improvement of migraine associated with visual auras in occipital lobe BAVMs after stereotactic radiosurgery (SRS). Therefore, this study investigated the prevalence of headache with visual aura in patients with occipital lobe BAVMs and evaluated the impact of SRS on preexisting symptoms., Methods: As a single-center retrospective cohort study, a total of 104 patients with occipital lobe BAVMs treated with SRS from 1990 to 2024 were analyzed. Pre-SRS and post-SRS symptoms of headache and visual aura were documented. The outcomes measured were headache severity, frequency, aura characteristics, and BAVM obliteration rates., Results: Of the 104 patients, 41 (39%) reported chronic headache pre-SRS, and 37 (36%) experienced migraine with visual aura. Compared with pre-SRS, 90% (28/31) of the patients experienced a >50% reduction in headache severity and frequency at a median follow-up of 11 months, and 89% (25/28) experienced a >50% reduction in aura frequency. Complete headache remission was achieved in 35% (13/31) of patients. Complete obliteration of BAVMs was confirmed in 75% (76/102) of the patients at a median of 33 months. A smaller nidus volume was associated with both complete headache remission and BAVM obliteration, whereas age and sex were not significant predictors of headache outcome., Conclusion: Patients with occipital lobe BAVMs frequently experience migraine-like headaches with visual aura, which may be more prevalent than in the general population. SRS for occipital lobe BAVMs could not only achieve favorable BAVM obliteration with minimal adverse effects but also significantly improve migraine-like symptoms, including visual aura, particularly in patients with smaller nidus volumes., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)

Purpose: We aimed to predict the neurological prognosis of cardiac arrest (CA) patients using quantitative imaging biomarkers extracted from brain computed tomography images., Methods: We retrospectively enrolled 86 CA patients (good prognosis, 32; poor prognosis, 54) who were treated at three hospitals between 2017 and 2019. We then extracted 1131 quantitative imaging biomarkers from whole-brain and local volumes of interest in the computed tomography images of the patients. The data were split into training and test sets containing 60 and 26 samples, respectively, and the training set was used to select representative quantitative imaging biomarkers for classification. In univariate analysis, the classification was evaluated using the p-value of the Brunner-Munzel test and area under the receiver operating characteristic curve (AUC) for the test set. In multivariate analysis, machine learning models reflecting nonlinear and complex relations were trained, and they were evaluated using the AUC on the test set., Results: The best performance provided p = 0.009 (<0.01) and an AUC of 0.775 (95% confidence interval, 0.590-0.960) for the univariate analysis and an AUCof0.813 (95% confidence interval, 0.640-0.985) for the multivariate analysis. Overall, the gray level with the maximum gradient in the histogram of the three-dimensionally low-pass-filtered image was an important feature for prediction across the analyses., Conclusions: Quantitative imaging biomarkers can be used in neurological prognosis prediction for CA patients. Relevant biomarkers may contribute to protocolized computed tomography image acquisition to ensure proper decision support in acute care., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Associazione Italiana di Fisica Medica e Sanitaria. Published by Elsevier Ltd. All rights reserved.)

Objective: This study compared the effectiveness of the endoscopic endonasal approach (EEA) with the conventional transcranial approach (TCA) for treating tuberculum sellae meningiomas (TSMs), aiming to identify the superior surgical method and the risk factors affecting outcomes., Methods: Patients treated for TSM from 1998 to 2023 at our institution were retrospectively analyzed, evaluating patient characteristics, tumor features, outcomes, and complications. A novel grading system for preoperative evaluation of TSMs was proposed., Results: Among 49 patients, 26 underwent EEA and 23 underwent TCA. The maximum diameters were comparable between the groups (mean 22 mm vs. 23 mm). Gross total resection rates were 62% for EEA and 70% for TCA, showing no significant difference. However, postsurgical visual improvement was significantly higher in the EEA group compared with the TCA group (77% vs. 44%; P = 0.020), with fewer complications in the EEA group (8% vs. 35%; P = 0.032)., Conclusions: EEA is a safe and effective treatment approach for small to medium TSMs, with outcomes comparable to TCA in terms of resection but superior in visual improvement and fewer complications. Selection of surgical approach should consider patient and tumor characteristics as well as surgeon experience., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Vagal paragangliomas (VPs) are rare tumors in the upper cervical region. Although surgical resection is the standard treatment for these tumors, it carries significant risks due to the tumor's high vascularity and proximity to vital structures. Stereotactic radiosurgery (SRS) for skull base paraganglioma could be a minimally invasive alternative., Case Description: We report the case of a 47-year-old man with a large, asymptomatic VP who was successfully treated with SRS with Gamma Knife Icon, which was performed in the parapharyngeal space (volume: 25.7 mL) using a marginal dose of 14 Gy to the 45% isodose line. This case illustrates the successful treatment of a lesion near the conventional limits (lower limit of C2 vertebral body) using noninvasive mask fixation. Excellent tumor control without neurological deficits was achieved for 25 months after SRS. The tumor volume decreased by 70% (final volume: 7.6 mL)., Conclusion: This study demonstrates the utility of Gamma Knife Icon, which facilitates optimal SRS for upper cervical lesions, including VPs., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Surgical Neurology International.)

Background: Recent anatomical studies have reported the feasibility of the endoscopic endonasal approach to the middle fossa. However, its clinical applicability has been discussed in only a few cases. This article describes the case of a middle fossa epidural abscess successfully drained through a fully endoscopic endonasal corridor and discusses the key technical points., Observations: The authors describe an 8-year-old boy who presented with worsening headache, fatigue, emesis, and fever and was diagnosed with a left middle fossa epidural abscess associated with sphenoid sinusitis. Following endoscopic sinus surgery performed by a rhinologist, the middle fossa was accessed by removing the maxillary strut through the transmaxillary transpterygoid corridor. Complete drainage of the abscess was confirmed on postoperative magnetic resonance imaging. The patient tolerated the surgery without neurological deficit and demonstrated prompt symptom improvement. He was discharged home after completing a 6-week course of antibiotic therapy and remained free from recurrence at 1 year following surgery., Lessons: The endoscopic endonasal approach may be applicable to a middle fossa epidural abscess, resulting in prompt clinical improvement. The maxillary strut is a key structure for entering the middle fossa. https://thejns.org/doi/10.3171/CASE24288.

Background and Purpose: The impact of cobalt-60 dose rate (Co-60 DR) on outcomes of stereotactic radiosurgery (SRS) for brain arteriovenous malformations (AVMs) remains fully elucidated. This study explored the association between Co-60 DR and SRS outcomes in AVM treatment., Materials and Methods: 772 AVM patients from 1990 to 2020 were included. High DR was defined as ≥ 2.4 Gy/min. AVM patients were categorized into 3 cohorts based on the nidus volume: small (<5 mL), medium (≥5 mL and < 10 mL), and large (≥10 mL). The primary endpoint was AVM obliteration; secondary endpoints included post-SRS hemorrhage., Results: Cumulative obliteration rates of the large AVM were significantly increased in the high DR group than those in the low DR group (84 % vs. 45 % at 5 years, log-rank test; p = 0.011). Multivariable analysis showed that the obliteration rate was significantly elevated for the high DR group in the large AVM cohort with an adjusted hazard ratio (HR) of 1.78 (95 % confidence interval [CI]: 1.00-3.17, p = 0.049). Post-SRS hemorrhage rates of the entire cohort were significantly decreased in the high DR group than in the low DR group (2.5 % vs. 5.3 % at 5 years, log-rank test; p = 0.035). Multivariable analysis revealed post-SRS hemorrhage was reduced in the high DR group with an adjusted HR of 0.47 (95 % CI: 0.24-0.92, p = 0.026)., Conclusion: High DR may lead to increased efficiency for large AVMs and decreased post-SRS hemorrhage in all AVM cases. Sustaining a higher Co-60 DR could potentially yield favorable outcomes for SRS for AVMs., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Clinical implications of RNF213 genetic variants, other than p.Arg4810Lys, in moyamoya disease (MMD), remain unclear. This study aimed to investigate the association of RNF213 variants with clinical phenotypes in MMD. This retrospective cohort study collected data regarding the clinical characteristics of 139 patients with MMD and evaluated the angioarchitectures of 253 hemispheres using digital subtraction angiography at diagnosis. All RNF213 exons were sequenced, and the associations of clinical characteristics and angiographical findings with p.Arg4810Lys, p.Ala4399Thr, and other rare variants (RVs) were examined. Among 139 patients, 100 (71.9%) had p.Arg4810Lys heterozygote (GA) and 39 (28.1%) had the wild type (GG). Fourteen RVs were identified and detetcted in 15/139 (10.8%) patients, and p.Ala4399Thr was detected in 17/139 (12.2%) patients. Hemispheres with GG and p.Ala4399Thr presented with significantly less ischemic events and more hemorrhagic events at diagnosis (p = 0.001 and p = 0.028, respectively). In asymptomatic hemispheres, those with GG were more susceptible to de novo hemorrhage than those with GA (adjusted hazard ratio [aHR] 5.36) with an increased risk when accompanied by p.Ala4399Thr or RVs (aHR 15.22 and 16.60, respectively). Within the choroidal anastomosis-positive hemispheres, GG exhibited a higher incidence of de novo hemorrhage than GA (p = 0.004). The GG of p. Arg4810Lys was a risk factor for de novo hemorrhage in asymptomatic MMD hemispheres. This risk increased with certain other variants and is observed in choroidal anastomosis-positive hemispheres. A comprehensive evaluation of RNF213 variants and angioarchitectures is essential for predicting the phenotype of asymptomatic hemispheres in MMD., (© 2023. The Author(s).)

Background and Objectives: To survey the applied definitions of 'cystic' among pituitary adenomas and evaluate whether postoperative outcomes differ relative to non-cystic counterparts., Methods: A literature search and meta-analysis was performed using PRISMA guidelines. Studies were eligible if novel data were reported regarding the applied definition of 'cystic' and postoperative outcomes among cases of surgically treated pituitary adenomas. Data were pooled with random effects meta-analysis models into cohorts based on the applied definition of 'cystic'. Categorical meta-regressions were used to investigate differences between cohorts. Among studies comparing cystic and non-cystic pituitary adenomas, meta-analysis models were applied to determine the Odds Ratio [95% Confidence Interval]. Statistical analyses were performed using Comprehensive Meta-Analysis (CMA, 4.0), with a priori significance defined as P < 0.05., Results: Ten studies were eligible yielding 283 patients with cystic pituitary adenomas. The definitions of 'cystic' mainly varied between the visual appearance of cystic components on preoperative magnetic resonance imaging and a volumetric definition requiring 50% or greater of tumor volume exhibiting cystic components. Tumor diameter was seldom reported with an associated standard deviation/error, limiting meta-analyses. When the data were pooled in accordance with the definition applied, there were no significant differences in the rates of gross total resection (P = 0.830), endocrinologic remission (P = 0.563), and tumor recurrence (P = 0.320). Meta-analyses on studies comparing cystic versus non-cystic pituitary adenomas indicated no significant difference in the rates of gross total resection (P = 0.729), endocrinologic remission (P = 0.857), and tumor recurrence (P = 0.465)., Conclusion: Despite some individual studies describing a significant influence of pituitary adenoma texture on postoperative outcomes, meta-analyses revealed no such differences between cystic and non-cystic pituitary adenomas. This discrepancy may be explained in part by the inconsistent definition of 'cystic' and between-group differences in tumor size. A notion of a field-standard definition of 'cystic' among pituitary adenomas should be established to facilitate inter-study comparisons., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background and Objectives: Access to the amygdala and hippocampus (A/H) is complex. To address the limitations and invasiveness of traditional approaches, including the Transsylvian, Subtemporal, and Supracerebellar infratentorial approaches, we developed the suprapetrous infratemporal (SPIT) approach. This study describes the nuances of this approach in both cadaveric studies and clinical cases., Methods: Three unilateral exposures were performed using microscopic and endoscopic methodologies in the SPIT approach. After cadaveric investigation, this approach was successfully implemented in representative clinical cases., Results: The SPIT approach enabled direct access to the inferior A/H, circumventing the requirement for temporal lobe retraction and detachment of the temporal lobe from the dura through a subtemporal route by drilling the upper part of the mastoid, consequently mitigating tension on the vein of Labbé. This enabled a bottom-up view because one would gain with a zygomatic osteotomy and forward projection like a mini-posterior petrosal view by using a transmastoid view, without cutting down the zygomatic arch and opening the dura subtemporally, limiting patient pain and preventing case comorbidity. The SPIT approach was performed in 2 cases of mesial temporal cavernoma presenting with seizures. The lesion was visualized intraoperatively and was successfully removed in these cases. The postoperative course was excellent with no complications, and gross total resection was radiographically confirmed with Engel Class 1a seizure freedom., Conclusion: The SPIT approach is a complementary approach for inferior A/H disease, combining the combined middle fossa approach modified for intradural pathology. Limited drilling of the upper aspect of the mastoid with a medial dural opening at the level of the arcuate eminence provides a direct trajectory with minimal brain retraction. Additional research encompassing a larger patient cohort and extended follow-up periods is required to substantiate the advantages of SPIT in the management of inferior A/H lesions., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)

Objective: To assess the impact of tumor extension into the occipital condyle (OC) in lower clival chordoma management and the need for occipito-cervical fusion (OCF)., Methods: A retrospective analysis was conducted on 35 patients with lower clival chordoma. The preoperative area of the intact OCs, Hounsfield units, and the integrity of the apical ligament and the tectorial membrane were assessed using preoperative imaging., Results: Seven (20%) patients were in the OCF group. The OCF group exhibited a higher prevalence of preoperative pain in the neck or head (P = 0.006), ligament absence (P = 0.022), and increased propensity for postoperative wound issues (P = 0.022) than the non-OCF group. The OCF group had less intact OCs (P < 0.001) and higher spinal instability neoplastic score (P = 0.002) than the non-OCF group. All patients with intact OCs < 60% underwent OCF, and those with OCs ≥ 70% were treated without OCF. Those with OCs between 60% and 69% underwent OCF if the ligaments were eroded, and did not undergo OCF if the ligaments were intact. Treatment strategies varied, with endoscopic endonasal approach alone being common. Radiation therapy was administered to 89% of patients. All 3 patients treated with OCF after tumor resection had wound issues; none treated with OCF before resection had wound issues. None developed atlanto-occipital instability. Survival rates did not significantly differ between groups., Conclusions: In the absence of mobility-related neck pain, patients with lower clival chordoma and intact OC ≥ 60%, intact apical ligament, and intact tectorial membrane, may not require OCF., (Copyright © 2024 Elsevier Inc. All rights reserved.)

To investigate the geometric accuracy of the radiation focal point (RFP) and cone-beam computed tomography (CBCT) over long-term periods for the ICON Leksell Gamma Knife radiosurgery system. This phantom study utilized the ICON quality assurance tool plus, and the phantom was manually set on the patient position system before the implementation of treatment for patients. The deviation of the RFP position from the unit center point (UCP) and the positions of the four ball bearings (BBs) in the CBCT from the reference position were automatically analyzed. During 544 days, a total of 269 analyses were performed on different days. The mean ± standard deviation (SD) of the deviation between measured RFP and UCP was 0.01 ± 0.03, 0.01 ± 0.03, and -0.01 ± 0.01 mm in the X, Y, and Z directions, respectively. The deviations with offset values after the cobalt-60 source replacement (0.00 ± 0.03, -0.01 ± 0.01, and -0.01 ± 0.01 mm in the X, Y, and Z directions, respectively) were significantly (p = 0.001) smaller than those before the replacement (0.02 ± 0.03, 0.02 ± 0.01, and -0.02 ± 0.01 mm in the X, Y, and Z directions, respectively). The overall mean ± SD of four BBs was -0.03 ± 0.03, -0.01 ± 0.05, and 0.01 ± 0.03 mm in the X, Y, and Z directions, respectively. Geometric positional accuracy was ensured to be within 0.1 mm on most days over a long-term period of more than 500 days., (© 2024. The Author(s).)

Objective: This study aimed to provide data on extended outcomes in primary clival chordomas, focusing on progression-free survival (PFS) and overall survival (OS)., Methods: A retrospective single-center analysis was conducted on patients with clival chordoma treated between 1987 and 2022 using surgery, stereotactic radiosurgery, or proton radiation therapy (PRT)., Results: The study included 100 patients (median age 44 years, 51% male). Surgery was performed using the endoscopic endonasal approach in 71 patients (71%). Gross-total resection (GTR) or near-total resection (NTR) was attained in 39 patients (39%). Postoperatively, new cranial nerve deficits occurred in 7%, CSF leak in 4%, and meningitis in none of the patients. Radiation therapy was performed in 79 patients (79%), with PRT in 50 patients (50%) as the primary treatment. During the median follow-up period of 73 (interquartile range [IQR] 38-132) months, 41 recurrences (41%) and 31 deaths (31%) were confirmed. Patients with GTR/NTR had a median PFS of 41 (IQR 24-70) months. Patients with subtotal resection or biopsy had a median PFS of 38 (IQR 16-97) months. The median PFS of patients who received radiation therapy was 43 (IQR 26-86) months, while that of patients who did not receive radiation therapy was 18 (IQR 5-62) months. The Kaplan-Meier method showed that patients with GTR/NTR (p = 0.007) and those who received radiation therapy (p < 0.001) had longer PFS than their counterparts. The PFS rates following primary treatment at 5, 10, 15, and 20 years were 51%, 25%, 17%, and 7%, respectively. The OS rates at the same intervals were 84%, 60%, 42%, and 34%, respectively. Multivariate Cox regression analysis showed that age < 44 years (p = 0.02), greater extent of resection (EOR; p = 0.03), and radiation therapy (p < 0.001) were associated with lower recurrence rates. Another multivariate analysis showed that age < 44 years (p = 0.01), greater EOR (p = 0.04), and freedom from recurrence (p = 0.02) were associated with lower mortality rates. Regarding pathology data, brachyury was positive in 98%, pan-cytokeratin in 93%, epithelial membrane antigen in 85%, and S100 in 74%. No immunohistochemical markers were associated with recurrence., Conclusions: In this study, younger age, maximal safe resection, and radiation therapy were important factors for longer PFS in patients with primary clival chordomas. Preventing recurrences played a crucial role in achieving longer OS.

Background: Acinic cell carcinomas (AcCCs), rare malignancies of the salivary glands, often recur and metastasize, particularly in the skull base. Conventional radical resection can be invasive for skull base AcCCs adjacent to cranial nerves and major vasculature, and the effectiveness of stereotactic radiosurgery (SRS) as an alternative is not well established., Observations: This case report details the application of SRS for recurrent skull base AcCCs. A 71-year-old male with a history of resection for a right mandibular AcCC 23 years earlier experienced tumor recurrence involving the right cavernous sinus and nasal cavity. He underwent endoscopic transnasal surgery followed by SRS targeting different tumor locations-the cavernous sinus to the pterygopalatine fossa, maxillary sinus, and clivus-each with a prescribed dose of 20 Gy to the 40% to 50% isodose line. After the first skull base metastasis, additional sessions of localized SRS after endoscopic surgery led to a 12-year survival without sequela., Lessons: This is a report indicating that SRS for skull base AcCCs can achieve favorable local control, functional preservation, and long-term survival. SRS may be suitable for skull base AcCC given the lesion's tendency toward multiple local recurrences. Further investigation is needed to validate the treatment's efficacy.

Purpose: This study investigated whether Ki-67 labeling index (LI) correlated with clinical outcomes after SRS for atypical meningiomas., Methods: This retrospective study examined 39 patients with atypical meningiomas who underwent SRS over a 10-year study period. Ki-67 LI was categorized into 3 groups: low (< 5%), intermediate (5%-10%), and high (> 10%). Local tumor control rates (LCRs), progression-free rates (PFRs), disease-specific survival (DSS) rates, and adverse radiation-induced events (AREs) were evaluated., Results: The median follow-up periods were 26 months. SRS was performed at a median prescription dose of 18 Gy for tumors with a median Ki-67 LI of 9.6%. The 3-year LCRs were 100%, 74%, and 25% in the low, intermediate, and high LI groups, respectively (p = 0.011). The 3-year PFRs were 100%, 40%, and 0% in the low, intermediate, and high LI groups (p = 0.003). The 5-year DSS rates were 100%, 89%, and 50% in the low, intermediate, and high LI groups (p = 0.019). Multivariable Cox proportional hazard analysis showed a significant correlation of high LI with lower LCR (hazard ratio [HR], 3.92; 95% confidence interval [CI] 1.18-13.04, p = 0.026), lower PFR (HR 3.80; 95% CI 1.46-9.88, p = 0.006), and shorter DSS (HR 6.55; 95% CI 1.19-35.95, p = 0.031) compared with intermediate LI. The ARE rates were minimal (8%) in the entire group., Conclusion: Patients with high Ki-67 LI showed significantly more tumor progression and tumor-related death. Ki-67 LI might offer valuable predictive insights for the post-SRS management of atypical meningiomas., (© 2024. The Author(s).)

Purpose: Neurofibromatosis type 2 (NF2) is intractable because of multiple tumors involving the nervous system and is clinically diverse and genotype-dependent. Stereotactic radiosurgery (SRS) for NF2-associated schwannomas remains controversial. We aimed to investigate the association between radiosurgical outcomes and mutation types in NF2-associated schwannomas., Methods: This single-institute retrospective study included consecutive NF2 patients with intracranial schwannomas treated with SRS. The patients' types of germline mutations ("Truncating," "Large deletion," "Splice site," "Missense," and "Mosaic") and Halliday's genetic severity scores were examined, and the associations with progression-free rate (PFR) and overall survival (OS) were analyzed., Results: The study enrolled 14 patients with NF2 with 22 associated intracranial schwannomas (median follow-up, 102 months).　The PFRs in the entire cohort were 95% at 5 years and 90% at 10-20 years. The PFRs tended to be worse in patients with truncating mutation exons 2-13 than in those with other mutation types (91% at 5 years and 82% at 10-20 years vs. 100% at 10-20 years, P = 0.140). The OSs were 89% for patients aged 40 years and 74% for those aged 60 years in the entire cohort and significantly lower in genetic severity group 3 than in the other groups (100% vs. 50% for those aged 35 years; P = 0.016)., Conclusion: SRS achieved excellent PFR for NF2-associated intracranial schwannomas in the mild (group 2A) and moderate (group 2B) groups. SRS necessitates careful consideration for the severe group (group 3), especially in cases with NF2 truncating mutation exons 2-13., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Purpose: This study aimed to analyze the treatment outcomes of single-fraction stereotactic radiosurgery (SRS) for adenoid cystic carcinoma patients., Methods: Retrospective analysis was conducted for 55 patients with 66 lesions. SRS intentions were categorized as definitive, adjuvant, salvage, and palliative. Tumor control was defined as local (within 50% isodose line), marginal (outside 50% isodose line), and distant (metastasis outside head/neck)., Results: The median age was 60 years (range 21-85), with 53% males. Tumor origin was head/neck for 88% and trachea/lung for 12%. 61% were recurrent lesions. Median interval from diagnosis to SRS was 14 months. Preceding surgery was performed in 30%. SRS was administered as definitive (30 lesions), adjuvant (13), salvage (19), and palliative (4). SRS was used as a boost to external beam radiation therapy (EBRT) in 39%. Concurrent chemotherapy was administered in 26%. 5-, 10-, and 15-year local control rates were 60%, 33%, and 27%, respectively; local/marginal control rates were 29%, 13%, and 10%. For recurrent lesions treated with SRS without EBRT, 5-year local control rate was 14%, and local/marginal control rate was 5%. For recurrent lesions treated with SRS and EBRT, 5-year local control rate was 100%, and local/marginal control rate was 40%. The rate of distant failure after SRS was 40%. Older age and distant metastasis before SRS were negative factors for overall survival., Conclusion: SRS provided a high rate of local tumor control, but marginal failure was frequent. Integrating SRS with added EBRT exhibits potential for enhancing local and local/marginal tumor control, particularly in recurrent cases., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: PIT1 is a pituitary transcription factor that is associated with either growth hormone (GH), prolactin (PRL), or thyroid-stimulating hormone (TSH) production. However, PIT1-positive pituitary neuroendocrine tumors (PitNETs) are occasionally immunonegative for GH, PRL, and TSH. This paper describes the clinical presentation of PIT1 positive however immunonegative PitNETs., Methods: We conducted a retrospective analysis, identifying 228 PIT1-positive PitNET patients between 2017 and 2022. Out of these, ten (4%) tested negative for GH, PRL, and TSH. Functioning PitNETs were defined as those causing hormonal excess symptoms or hormonal overproduction., Results: As for 10 patients immunonegative for all three hormones however PIT1-positive, the mean ( ± standard deviation) age was 46 ± 13 years with 70% women. Six patients exhibited signs of excess GH or PRL, and three had visual problems. Additionally, one patient had secondary hypothyroidism and adrenal insufficiency resulting from the mass effect. All tumors were macroadenoma, with a median volume of 2.1 cm 3 (range, 0.8-17.5 cm 3 ). Gross total resection was attained in six patients by trans-sphenoidal surgery. Postoperatively, eight patients experienced clinical improvement: three in vision, two in amenorrhea, two in headache, and one in acromegaly symptoms. Biochemical improvement was observed in six patients, with all experiencing remission in hormonal excess and one showing improvement in secondary hypothyroidism. Stereotactic radiosurgery was performed in three patients., Conclusions: Patients with functioning PitNETs may exhibit PIT1 staining without GH, PRL, or TSH staining. Hormonally active tumors exist in this patient population; therefore, close endocrine follow-up is necessary despite the lack of staining for GH, PRL, and TSH., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Purpose: Because patients with untreated brain arteriovenous malformations (BAVMs) are at variable risks of cerebral hemorrhage and associated mortality and morbidity, it is essential to identify patient populations who benefit most from prophylactic interventions. This study aimed to examine age-dependent differences in the therapeutic effect of stereotactic radiosurgery (SRS) on BAVMs., Methods and Materials: This retrospective observational study enrolled patients with BAVMs who underwent SRS at our institution between 1990 and 2017. The primary outcome was post-SRS hemorrhage, and the secondary outcomes included nidus obliteration, post-SRS early signal changes, and mortality. To investigate age-related differences in outcomes after SRS, we performed age-stratified analyses using the Kaplan-Meier analysis and weighted logistic regression with the inverse probability of censoring weighting (IPCW). To address significant differences in patient baseline characteristics, we also performed inverse probability of treatment weighting (IPTW) adjusted for possible confounders to investigate age-related differences in outcomes after SRS., Results: A total of 735 patients with 738 BAVMs were stratified by age. Age-stratified analysis using a weighted logistic regression model with IPCW showed a direct correlation between patient age and post-SRS hemorrhage (odds ratio [OR], 95% confidence interval [CI], and P value: 2.20, 1.34-3.63, and .002 at 18 months; 1.86, 1.17-2.93, and .008 at 36 months; and 1.61, 1.05-2.48, and .030 at 54 months, respectively). The age-stratified analysis also showed an inverse relationship between age and obliteration over the first 42 months after SRS (OR, 95% CI, and P value: 0.05, 0.02-0.12, and <.001 at 6 months; 0.55, 0.44-0.70, and <.001 at 24 months; and 0.76, 0.63-0.91, and .002 at 42 months, respectively). These results were also confirmed with the IPTW analyses., Conclusions: Our analysis demonstrated that patients' age at SRS is significantly associated with hemorrhage and the nidus obliteration rate after treatment. In particular, younger patients are more likely to exhibit reduced cerebral hemorrhages and achieve earlier nidus obliteration compared with older patients., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: This study aims to examine the clinical autologous osteochondral grafts (AOG) outcomes for knee osteochondral diseases at operative ages >60 years, and to determine whether patients are able to sit straight in Japanese style after AOG., Methods: All patients who underwent AOG for knee osteochondral diseases between November 2001 and April 2018 were retrospectively identified. The inclusion criteria were AOG only without osteotomy, operative ages between 60 and 79 years, >2 years of follow-up, and involved femorotibial angle between 169° and 179° (normal alignment). Patients who underwent osteotomy to improve knee alignment and patients with inflammatory diseases such as rheumatoid arthritis were excluded. The patients' knee symptoms and their clinical outcome were evaluated according to the criteria of the knee scoring system of the Japanese Orthopedic Association (JOA), International Knee Documentation Committee (IKDC) subjective score, and the ability of straight sitting in Japanese style., Results: This study enrolled 57 cases and 60 knee joints during the study period. The follow-up ratio was 85.1%. Moreover, 14 men and 43 women and 29 right and 31 left knee joints were included in this study. The mean operative age and mean follow-up period were 67.8 years (range 60-76 years) and 81.1 months (range 24-167 months), respectively. In addition, the study involved 30 cases and 32 knee joints (60s group), and 27 cases and 28 knee joints (70s group). Moreover, 34 cases and 36 knee joints had osteonecrosis (ON group), and 23 cases and 24 knee joints had cartilage injury (CI group). The IKDC subjective and JOA scores in both the 60s and 70s groups showed significant differences: 2 years after AOG ＞at the follow-up period, ＞at the preoperative period. The scores in both the CI and ON groups showed similar significant differences. Furthermore, 8.3% and 53.5% of the patients could sit straight in Japanese style at the preoperative period and 2 years after AOG, respectively., Conclusion: Even if the patient's operative age was >60 years, the AOG only for their knee osteochondral diseases had good clinical outcomes, including the ability to sit straight in Japanese style., Level of Evidence: IV, Therapeutic case series Key words: autologous osteochondral grafts, aged patients, clinical outcome, knee joint, straight sitting in Japanese style., (© 2023 The Authors.)

Objective: Non-vascularized multilayer fascial closure technique (NMFCT) can be used instead of nasoseptal flap reconstruction for dural repair in endoscopic transnasal surgery (ETS); however, due to the lack of blood supply, its long-term durability and possible limitations need to be clarified., Methods: This was a retrospective study on patients who underwent ETS with intraoperative cerebrospinal fluid (CSF) leakage. We assessed the postoperative and delayed CSF leakage rates and the associated risk factors., Results: Among 200 ETSs with intraoperative CSF leakage, 148 (74.0%) ETSs were performed for skull base pathologies other than pituitary neuroendocrine tumor. The mean follow-up period was 34.4 months. Esposito grade 3 leakage was confirmed in 148 (74.0%) cases. NMFCT was used either with (67 [33.5%]) or without (133 [66.5%]) lumbar drainage. There were 10 cases (5.0%) of postoperative CSF leakage that necessitated reoperation. In 4 other cases (2.0%), CSF leakage was suspected but lumbar drainage alone successfully restored the condition. Multivariate logistic regression analyses revealed that posterior skull base location (P < 0.01, odds ratio 11.5, 95% CI 1.99-2.17 × 10 2 ) and craniopharyngioma pathology (P = 0.03, odds ratio 9.4, 95% CI 1.25-1.92 × 10 2 ) were significantly associated with postoperative CSF leakage. No delayed leakage occurred during the observation period except for 2 patients who underwent multiple radiotherapies., Conclusions: NMFCT is a reasonable alternative with long-term durability, though vascularized flap may be a better choice for cases in which vascularity of the surrounding tissues is significantly impaired due to interventions including multiple radiotherapies., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: White epidermoid cysts (WECs) are a rare type of epidermoid cyst with atypical radiological features. The epidemiological aspects and mechanisms of their onset remain unknown. Herein, the authors report a unique case of WEC transformation from a typical epidermoid cyst after stereotactic radiosurgery (SRS), confirmed by radiological and pathological findings., Observations: The case involved a 78-year-old man with a history of 2 surgeries for a left cerebellopontine angle typical epidermoid cyst 23 years earlier and SRS using the CyberKnife for recurrent trigeminal neuralgia (TN) 14 years earlier. The tumor with high intensity on T1-weighted imaging, low intensity on T2-weighted imaging, without restriction on diffusion-weighted imaging had gradually enlarged after SRS. Therefore, a salvage surgery was performed via a left suboccipital craniotomy, and the intraoperative findings showed a cyst with a brown, viscous liquid component, consistent with those of WECs. Histopathologically, keratin calcification and hemorrhage were identified, leading to a diagnosis of WEC. The postoperative course was uneventful, and the TN resolved. No tumor recurrence was recorded at 2 years postoperatively., Lessons: To the best of the authors' knowledge, this is the first world case of WEC transformation from a typical epidermoid cyst after SRS, confirmed by radiological and pathological findings. Radiation effects could have been involved in this transformation.

The genetic background of intracranial artery stenosis (ICAS), a major cause of ischemic stroke, remains elusive. We performed the world's first genome-wide association study (GWAS) of ICAS using DNA samples from Japanese subjects, to identify the genetic factors associated with ICAS and their correlation with clinical features. We also conducted a phenome-wide association study (PheWAS) of the top variant identified via GWAS to determine its association with systemic disease. The GWAS involved 408 patients with ICAS and 349 healthy controls and utilized an Asian Screening Array of venous blood samples. The PheWAS was performed using genotypic and phenotypic data of the Biobank Japan Project, which contained information on 46 diseases and 60 quantitative trait data from > 150,000 Japanese individuals. The GWAS revealed that the East Asian-specific functional variant of RNF213, rs112735431 (c.14429G > A, p.Arg4810Lys), was associated with ICAS (odds ratio, 12.3; 95% CI 5.5 to 27.5; P = 7.8 × 10 -10 ). Stratified analysis within ICAS cases demonstrated that clinical features of those with and without the risk allele were different. PheWAS indicated that high blood pressure and angina were significantly associated with RNF213 rs112735431. The first GWAS of ICAS, which stratifies subpopulations within the ICAS cases with distinct clinical features, revealed that RNF213 rs112735431 was the most significant variant associated with ICAS. Thus, RNF213 rs112735431 shows potential as an important clinical biomarker that characterizes pleiotropic risk in various vascular diseases, such as blood pressure and angina, thereby facilitating personalized medicine for systemic vascular diseases in East Asian populations., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Lacrimal gland pleomorphic adenomas (LGPAs) are common, benign, and intraorbital tumours that cause exophthalmos, ptosis, and visual disturbances. The curative treatment for LGPAs is gross total resection, and radiotherapy is considered adjunctive for recurrence or an alternative for inoperable LGPAs. Stereotactic radiosurgery (SRS) can be used for precise delivery of high radiation doses to the tumour, crucial in the treatment of intra-and extracranial neoplasms. Here, we present a 95-year-old woman who had a rapidly growing, recurrent LGPA and was successfully treated with SRS. The tumour was controlled without any adverse events over 21 months following SRS. SRS is a potential alternative treatment for recurrent LGPA., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)