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Objective: Despite pituitary neuroendocrine tumor (PitNET) being extra‐axial tumors without direct damage to brain tissue, patients with PitNET exhibit neuropsychological impairments. However, it remains unclear whether there are neuropsychological differences between PitNET and intra‐axial tumors that directly destroy the brain parenchyma. This prospective study aims to clarify this distinction to inform decision‐making for intracranial tumors of diverse origins. Methods: A total of 146 patients with PitNET, 74 patients with glioma representing intra‐axial tumors, and 52 age‐, sex‐, and education‐matched healthy controls were recruited. All patients received standard treatment and postoperative rehabilitation. Clinical data were meticulously collected, and neuropsychological tests were administered to all participants both before and 3 months after surgery. Results: Both PitNET and glioma patients experience the dual burden of cognitive and affective deficits. However, the feature of these deficits differs substantially. In PitNET patients, the deficits are relatively mild and focal, whereas in glioma patients, they are severe and extensive. Specifically, PitNET patients exhibit deficits in memory, anxiety, and negative affect. In contrast, glioma patients display deficits in executive function, attention, anxiety, positive/negative affect, and empathy. Notably, except for persistent memory deficits, the majority of neuropsychological scores declines in PitNET patients are restorable and can reach improvement within a short period after standard surgical therapy and perioperative management. Conversely, glioma patients not only fail to show improvements but also demonstrate worsening in terms of general cognition and memory postoperatively. Interpretation: As an extra‐axial tumor, PitNET may exhibit distinctive cognitive and affective functioning compared to intra‐axial tumors, highlighting the need for specific treatment approaches for PitNET patients. [ABSTRACT FROM AUTHOR]

Objective: To investigate the visual outcomes and optimal timing for repeat surgery in cases of postoperative hematoma following transsphenoidal surgery for pituitary neuroendocrine tumors (PitNETs). Methods: A retrospective study was conducted on 28 patients who developed evident postoperative hematoma out of a total of 9,010 patients. The hematomas were classified into three types based on their CT appearance. Type 1a - mild high density with no tension, Type 1b - thin-layer high density; Type 2a - solid high density with large empty cavities, Type 2b - solid high density with small empty cavities; Type 3 –solid high density with no cavity showing high tension. Patient data were collected for analysis. Results: The study cohort comprised 10 female and 18 male patients, with a mean age of 51.5±11.9 years. Most patients presented with large adenomas (median diameter 36mm). Postoperative visual sight improved in 12 patients, remained stable in 11 patients, and worsened in 5 patients. Notably, no patients experienced worsened visual sight beyond twenty-four hours after the operation. Among the five patients with visual deterioration, four had CT type 3 hematoma (4/6, 66.7%), and one had CT type 2b hematoma (1/9, 11.1%). Patients in the type 3 CT group were significantly more prone to experience visual deterioration compared to those in the type 2 group (odds ratio [OR] 2.154 [95% CI 1.858-611.014], P=.027). Four patients underwent repeat surgery after visual deterioration, resulting in visual improvement following a prolonged recovery period. Postoperative hematoma had limited impact on pituitary dysfunction and hyponatremia. Conclusion: Our study reveals a significant association between postoperative hematoma CT types and visual deterioration. For patients with stable visual sight and type 1 or 2a hematoma, conservative strategies may be considered. Conversely, type 2b and 3 patients are at higher risk of visual deterioration, especially within the first 24 hours after the operation. Consequently, early reoperation before vision worsens may be a prudent approach to reduce risks and improve visual outcomes, particularly in type 3 patients. [ABSTRACT FROM AUTHOR]

BACKGROUND: NF2-schwannomatosis (NF2) is an autosomal dominant disorder prone to hearing loss. Auditory brainstem implants (ABIs) offer a promising solution for hearing rehabilitation in NF2. OBJECTIVE: To synthesize existing literature on ABI implantation in NF2, focusing on audiological outcomes and ABI-related complications. METHODS: The systematic review followed PRISMA guidelines and was registered in the PROSPERO database (CRD42022362155). Relevant studies were identified by searching PubMed, EMBASE, CENTRAL, CMB, and CNKI from inception to August 2023. Data on environmental sound discrimination, open-set discrimination, closed-set discrimination, and ABI-related complications were extracted and subjected to meta-analysis. Publication bias was evaluated using funnel plots and Egger's test. RESULTS: Thirty-three studies were included. The pooled estimate was 58% (95% CI 49–66%) for environmental sound discrimination and 55% (95% CI 40–69%) for closed-set discrimination. Regarding open-set discrimination, the pooled estimates were 30% (95% CI 19–42%) for sound only, 46% (95% CI 37–54%) for lip-reading only, and 63% (95% CI 55–70%) for sound plus lip-reading. The pooled occurrence of ABI-related complications was 33% (95% CI 15–52%). CONCLUSION: This meta-analysis underscores the effectiveness and safety of ABIs in NF2, providing valuable insights for evidence-based decision-making and hearing rehabilitation strategies. [ABSTRACT FROM AUTHOR]

Abstract Background Methods Results Conclusions McCune-Albright syndrome (MAS) is a rare genetic, non-inheritable disease and is characterized by fibrous dysplasia, hyperendocrinism, and café-au-lait macules. Pituitary adenomas could be concurrent with this syndrome but clinicopathological features and the surgical management of such disorders is unclear.We retrospectively reviewed ten MAS-associated pituitary adenoma patients with follow-up in Beijing Tiantan Hospital and analyzed their clinicohistological data, surgical strategies, neuro-imaging, genetic mutations, and prognosis. Moreover, a critical review of the English language literature was also conducted.All of the ten MAS-associated adenoma patients underwent surgeries to remove the tumor (nine transsphenoidal approaches and one transcranial approach). None of these patients had a decompression of the optic canal. Notably, the growth hormone (GH), prolactin (PRL), and IGF-1 level had a significant reduction after the resection of the tumor while vision improvement was observed in most patients (6/7) with visual deficits. No tumor recurrence was observed during the follow-up from 16 to 150 months. The pathological examination showed a moderate Ki-67 LI (mean 1.19%, range from 0.1% to 3.3%) and the positive staining of Gsα and PKA C-beta. GNAS gene mutation (R201C) was detected in one patient.Hormone excess (including GH and PRL) could be significantly reduced and the visual deficits are greatly improved after the surgery without the decompression of the optic canal. In addition, MAS-associated pituitary adenomas have a moderate expression of Ki-67 and positive expression of Gsα and PKA C-beta, indicating a mildly proliferative nature of these tumors and the possible linking between MAS and adenomas. [ABSTRACT FROM AUTHOR]

Excessive artificial electromagnetic radiation poses significant health risks and disrupts military equipment. To address this, high-performance microwave absorption materials are urgently required. While ferromagnetic materials have excellent absorption but suffer from high density, limited bandwidth, and perishable properties. Carbon materials, especially metal-organic frameworks (MOFs), offer promise due to low density, chemical stability, and high conductivity. In this study, we synthesized a novel multicore-shell Fe 3 O 4 @Fe@C composite via solvothermal and in-situ pyrolysis methods. This composite integrated Zn-MOF-derived porous carbon with core-shell Fe 3 O 4 @Fe@C, enabling multiple electromagnetic wave routes, strong interfacial polarization, and magnetic-dielectric synergy. It exhibited exceptional electromagnetic wave absorption, with a −59.1 dB reflection loss at 13.36 GHz and a 5.36 GHz effective absorption bandwidth. Our innovative MOF-core-shell approach holds great promise for highly efficient microwave absorbers to mitigate artificial electromagnetic radiation's adverse effects on health and military equipment. [Display omitted] • A MOF-derived multicore-shell cucumber-like Fe 3 O 4 @Fe@C composite was synthesized. • Fe 3 O 4 @Fe@C composite showed ultrastrong electromagnetic wave absorption performance. • Porous carbon network provided huge specific surface area, multiple reflection sites. • Fe 3 O 4 /Fe and Fe/C heterogeneous interface enhanced the interfacial polarization loss. [ABSTRACT FROM AUTHOR]

Objective: To investigate the visual outcomes and optimal timing for repeat surgery in cases of postoperative hematoma following transsphenoidal surgery for pituitary neuroendocrine tumors (PitNETs). Methods: A retrospective study was conducted on 28 patients who developed evident postoperative hematoma out of a total of 9,010 patients. The hematomas were classified into three types based on their CT appearance. Type 1a - mild high density with no tension, Type 1b - thin-layer high density; Type 2a - solid high density with large empty cavities, Type 2b - solid high density with small empty cavities; Type 3 –solid high density with no cavity showing high tension. Patient data were collected for analysis. Results: The study cohort comprised 10 female and 18 male patients, with a mean age of 51.5±11.9 years. Most patients presented with large adenomas (median diameter 36mm). Postoperative visual sight improved in 12 patients, remained stable in 11 patients, and worsened in 5 patients. Notably, no patients experienced worsened visual sight beyond twenty-four hours after the operation. Among the five patients with visual deterioration, four had CT type 3 hematoma (4/6, 66.7%), and one had CT type 2b hematoma (1/9, 11.1%). Patients in the type 3 CT group were significantly more prone to experience visual deterioration compared to those in the type 2 group (odds ratio [OR] 2.154 [95% CI 1.858-611.014], P=.027). Four patients underwent repeat surgery after visual deterioration, resulting in visual improvement following a prolonged recovery period. Postoperative hematoma had limited impact on pituitary dysfunction and hyponatremia. Conclusion: Our study reveals a significant association between postoperative hematoma CT types and visual deterioration. For patients with stable visual sight and type 1 or 2a hematoma, conservative strategies may be considered. Conversely, type 2b and 3 patients are at higher risk of visual deterioration, especially within the first 24 hours after the operation. Consequently, early reoperation before vision worsens may be a prudent approach to reduce risks and improve visual outcomes, particularly in type 3 patients. [ABSTRACT FROM AUTHOR]

Background: Advances in microscopic and endoscopic surgical techniques have outpaced traditional classification and transcranial surgical strategies, especially with reference to the treatment of trigeminal schwannomas (TSs). A modified TS classification is proposed and appropriate surgical strategies are discussed. Methods: The cases of 93 patients who underwent surgical treatment in Beijing Tiantan Hospital in the previous 6 years were analyzed retrospectively, and a literature review was conducted. Results: Classification is based on surgical direction. Tumors were classified as follows: type A, backward orientation, located in the orbit or orbit and middle cranial fossa (8 cases, 8.6%); type B, upward orientation, located in the pterygopalatine fossa, infratemporal fossa or pterygopalatine fossa, infratemporal fossa, and middle cranial fossa (23 cases, 24.7%); type C, forward and backward orientations, located in the middle cranial fossa, posterior cranial fossa or both (58 cases, 62.4%); and type D, located in multiple regions (4 cases, 4.3%). 91.40% of patients underwent gross total resection (GTR) with 29 cases receiving endoscopic resection of whom 93.10% (27/29) experienced GTR. Conclusion: The 93 cases were satisfactorily divided into four types, according to tumor location and surgical orientation, enabling safe and effective removal by appropriate surgery. [ABSTRACT FROM AUTHOR]

Background: Endoscopic transsphenoidal surgery is the primary method used to treat pituitary adenomas (PAs) at present; however, this technique is associated with certain risks, including cerebrospinal fluid leakage (CFL) and residual tumors (RTs). In this study, we aimed to identify specific risk factors for intraoperative CFL (ioCFL) and postoperative RT in patients with pituitary adenoma and construct a corresponding nomogram for risk assessment. Methods: We collected a range of information from 782 patients who underwent endoscopic transsphenoidal PA resection in the Department of Neurosurgery at Beijing Tiantan Hospital between 2019 and 2021. Patients were then randomly assigned to training and validation groups (in a 8:2 ratio) with R software. Univariate and multivariable logistic regression models were then used to screen variables related to ioCFL and RT. These variables were then used to construct a predictive nomogram. Finally, the accuracy of the nomogram was validated by receiver operating characteristic curve (ROC) analysis, calibration plots, and decision curve analysis (DCA). Results: Univariate and multivariable logistic regression models identified four risk factors for ioCFL (Hardy grade, tumor size, position, and consistency) and five risk factors for RT (operation time, tumor size, consistency, Knosp grade, and primary/recurrence type). The area under the ROC curve (AUC) for the ioCFL risk model was 0.666 and 0.697 for the training and validation groups, respectively. For RT, the AUCs for the two groups were 0.788 and 0.754, respectively. The calibration plots for the ioCFL and RT models showed high calibration quality and DCA analysis yielded excellent efficiency with regards to clinical decision making. Conclusion: Tumor size, growth characteristics, and invasion location were identified as the main factors affecting intraoperative CFL and RT. With our novel nomogram, surgeons can identify high-risk patients according to preoperative and intraoperative tumor performance and reduce the probability of complications. [ABSTRACT FROM AUTHOR]

Adult posterior fossa ependymomas (PF-EPN) with preoperative cerebrospinal metastases are extremely rare. Only 3 cases have been reported in previous literature. A case of a 32-year-old male patient complained of headaches for three months. Pure tone audiometry showed a slight decrease in bilateral hearing. Auditory evoked potential indicated that the hearing on the left was slightly weaker than that on the right. Magnetic resonance imaging (MRI) revealed a primary tumor arising within the fourth ventricle and metastasizing to bilateral cerebellopontine angle (CPA), the third ventricle, the left lateral ventricle, T1, L1-2 and L5. A gross total resection (GTR) was performed on the lesion located in the left CPA. The histological examination showed a papillary ependymoma (WHO grade II). Immunohistochemical staining for H3K27me3 showed that nuclear positivity in more than 80% of cells. No NF2 mutation was observed. No progression was found during a 24-month follow-up. Our data indicate that preoperative multiple metastases in adult PF-EPN are extremely rare. This kind of disease usually has a low WHO grade and a favorable prognosis. GTR should be achieved when feasible and patients need a long-term follow-up with MRI. [ABSTRACT FROM AUTHOR]

In the present study, we evaluate the characteristics of cognitive and affective function in patients with somatotroph adenomas (SAs) that secrete excess growth hormone and the effects of surgical intervention. We conducted a prospective longitudinal study, recruiting 27 patients with SAs, 29 patients with nonfunctional pituitary adenomas (NFPAs) as the lesion control group, and 24 healthy participants as the healthy controls (HCs). These three groups were matched for sex, age, and years of education. We performed multidimensional cognitive function and neuropsychological assessments 1–2 days before endoscopic endonasal transsphenoidal surgery and at 3 months postoperatively. The Mini-mental state examination, Montreal cognitive assessment, Frontal assessment battery, Trail making test, and Digit span test were used to assess multidimensional cognitive function, including general intelligence, frontal lobe function, executive function, and memory. The Hamilton anxiety scale, Beck depression inventory, and Positive and Negative Affect Schedule scale were used for the neuropsychological assessment, including anxiety, depressed mood, and positive and negative emotions. Compared with the HCs, the patients with SAs showed poor performance in the memory (P = 0.009) and anxiety (P = 0.013) assessments. However, no statistically significant difference was observed between patients with SAs and NFPAs for either cognitive function or effective performance. Moreover, patients with SAs did not show significant changes in cognition and affective behavior after surgery. In contrast, patients with NFPAs displayed significant improvements in memory (P = 0.015), executive function (P < 0.001), and anxiety mood (P = 0.001) performance postoperatively. Patients with SAs showed specific cognitive deficits and abnormal moods, which might be attributed to the overproduction of growth hormone. However, surgical intervention had a limited effect on improving the impaired cognitive function and abnormal moods in patients with SAs at short-term follow-up. [ABSTRACT FROM AUTHOR]

Purpose: Assessing the invasiveness of pituitary adenomas (PAs) is critical to making the best surgical and treatment plan. However, it is difficult to determine the invasiveness of pituitary adenomas based on current clinical methods, such as imaging and histological methods. The present article aims to investigate noninvasive methods to discover viable biomarkers for invasive pituitary adenomas and provide a basis for early intervention of pituitary adenomas. Methods: E-cadherin, N-cadherin, Epcam, TGF-β, Smad3, and Smad7 were detected in the tissues and exosomes in 10 cases of invasive PAs and 10 cases of noninvasive PAs by real-time quantitative reverse transcription polymerase chain reaction (qRT-PCR), Western blotting, and immunohistochemical analysis. Results: Compared with that in the noninvasive group, the expression of N-cad in the exosomes of the invasive group was significantly increased, and the expression of E-cad and Epcam was reduced. In the invasive group, the expression levels of TGF-β 1 and Smad3 were reduced. These results were consistent across exosomes and groups. In further cell experiments, the EMT ratio in the SIS3 treatment group, and especially in the TGF-β 1 plus SIS3 treatment group (P < 0.001), was significantly increased, and the EMT ratio was significantly lower when one-half the dose of TGF-β and SIS3. Conclusion: The results indicate that EMT-related biomarkers in serum exosomes can be potentially used for assessing the invasiveness of pituitary adenoma. [ABSTRACT FROM AUTHOR]

Purpose: Spontaneous cerebrospinal fluid (CSF) rhinorrhea is related to intracranial pressure (ICP) and dynamic changes. This study investigated CSF dynamics along the optic nerve (ON) in patients with spontaneous CSF rhinorrhea. Methods: The computed tomographic (CT) cisternographies of 66 patients (132 eyes) with spontaneous CSF rhinorrhea were analysed. The contrast‐loaded CSF (CLCSF) density was measured in Hounsfield units (HU) at three regions of interest (ROIs) along the ON and adjusted by the basal cistern density. The CLCSF density and ON sheath diameter (ONSD) were analysed between both sides in the different ICP groups. Results: When comparing the density of CLCSF along the ON, no significant differences were found between the ipsilateral and contralateral sides of the leakage. The distribution of CLCSF along the ON showed a highly significant density reduction from the canalicular segment to the bulbar segment on both sides. The CLCSF density significantly decreased on the ipsilateral ON in the canalicular segment and tended to decrease on the ipsilateral ON in the bulbar and canal segments compared with that on the contralateral ON in the low‐ICP group. The ONSD tended to decrease on the ipsilateral side of leakage. Conclusions: According to the CLCSF density on CT cisternography, CSF dynamics along the ON may bilaterally decrease from the optic canal to the retrobulbar segment. Cerebrospinal fluid (CSF) dynamics are possibly influenced by differences in ICP, and a lower ICP may cause more obvious differences or impairments in CSF dynamics along the ipsilateral ON. [ABSTRACT FROM AUTHOR]

Cerebrospinal fluid (CSF) leakage is a major complication after extended endonasal transsphenoidal surgery (EETSS), which is commonly used in the treatment of anterior skull base tumors. Dural suturing and graded reconstruction are promising techniques to further decrease the incidence of postoperative CSF (poCSF) leakage. The effect of continuous dural suturing in endoscopic surgery was investigated in this retrospective study. A total of 79 EETSS patients were included; the procedures were performed for subdural tumor removal by a single endoscopic neurosurgical team. Comparisons were applied between patients who did and did not undergo endoscopic dural suturing after tumor removal. Multivariate logistic regression analysis was performed to identify variables that significantly influenced the incidence of poCSF leakage. In all, 79 adult patients developed Esposito's grade 3 intraoperative high-flow CSF leakage. Ten patients (12.7%) experienced poCSF leakage. One of the 36 patients who underwent intraoperative dural suturing developed poCSF leakage, compared with nine of 43 patients who did not undergo dural suturing (p = 0.016). Regression analysis showed that dural suturing could significantly decrease the incidence of poCSF leakage (p = 0.049, OR 0.108, 95% CI 0.013–0.899). Prophylactic lumbar drainage could also help decrease the CSF leakage rate. Dural suturing under endoscopy is a promising and effective method for application in skull base reconstruction after subdural skull base tumor removal. With future progress, lumbar drainage and even nasoseptal flap placement could be replaced in certain groups of patients undergoing EETSS. [ABSTRACT FROM AUTHOR]

Objective: The incidence of and risk factors for intra- and postoperative cerebrospinal fluid (poCSF) leak in patients who underwent endoscopic surgery for pituitary adenoma were investigated in this retrospective study. Methods: A total of 216 consecutive patients operated on by a single neurosurgical team were included. Logistic regression was applied to identify risk factors for intraoperative CSF (ioCSF) and poCSF leaks, and the outcome and management of ioCSF and poCSF leaks were analyzed. Results: Sixty-five patients (30.1%) experienced ioCSF leak, of whom 10 developed poCSF leak. Three of 151 patients developed poCSF leak without obvious ioCSF leak. Multiple regression analysis revealed that tumor with lobular or irregular contour and gonadotrophic-positive staining increased the risk of ioCSF leak; additionally, nonfunctional tumor (p = 0.058) and preoperative gonadotrophic hormone abnormalities (p = 0.08) tended to increase this risk. The presence of ioCSF leak and preoperative visual impairment were predictors for poCSF leak. Tumor with Knosp grades 3–4 and a higher grade of ioCSF leak could increase the risk of poCSF leak. Tailored pedicled vascularized nasoseptal flaps (NSFs) preparation before tumor removal greatly decreased the rate of poCSF leak with ioCSF leak and the overall leak rate. Three prophylactic lumbar drains (LDs) were performed in patients with grade 3 ioCSF leak, none of whom developed poCSF leak. Conclusions: Lobular or irregular tumor contour and gonadotrophic-positive staining were associated with a high risk of ioCSF leak, while ioCSF leak and preoperative visual impairment were associated with poCSF leak. NSF preparation, preventive sellar reconstruction and LD use could likely decrease the rate of poCSF leak. [ABSTRACT FROM AUTHOR]

Medulloblastoma (MB) is the most common malignant pediatric brain tumor arising in the cerebellum or the 4th ventricle. Cerebellopontine angle (CPA) MBs are extremely rare tumors, with few cases previously described. In this study, we sought to describe the clinical characteristics, molecular features and outcomes of CPA MB. We retrospectively reviewed a total of 968 patients who had a histopathological diagnosis of MB at the Beijing Neurosurgical Institute between 2002 and 2016. The demographic characteristics, clinical manifestations and radiological features were retrospectively analyzed. Molecular subgroup was evaluated by the expression profiling array or immunohistochemistry. Overall survival (OS) and progression-free survival (PFS) were calculated using Kaplan-Meier analysis. In this study, 15 patients (12 adults and 3 children) with a mean age at diagnosis of 25.1 years (range 4–45 years) were included. CPA MBs represented 1.5% of the total cases of MB (15/968). Two molecular subgroups were identified in CPA MBs: 5 WNT-MBs (33%) and 10 SHH-MBs (67%). CPA WNT-MBs had the extracerebellar growth with the involvement of brainstem (P = 0.002), whereas CPA SHH-MBs predominantly located within the cerebellar hemispheres (P = 0.004). The 5-year OS and PFS rates for CPA MB were 80.0% ± 10.3% and 66.7% ± 12.2%, respectively. Pediatric patients with CPA MBs had worse outcomes than adult patients (OS: P = 0.019, PFS: P = 0.078). In conclusion, CPA MB is extremely rare and consists of two subgroups. Adult patients with CPA MB had a good prognosis. Maximum safe surgical resection combined with adjuvant radiotherapy and chemotherapy can be an effective treatment strategy for this rare tumor. [ABSTRACT FROM AUTHOR]

Background: 5-Hydroxymethylcytosine (5hmC) is a novel epigenetic mark and may be involved in the mechanisms of tumorigenesis and malignant transformation. However, the role of 5hmC in ependymoma, the third most common brain tumor in children, remains unclear. The aim of this study sought to identify the characterization of 5hmC levels in pediatric posterior fossa ependymoma and to evaluate whether 5hmC levels could be a potential factor to predict clinical outcomes. Results: Our results showed that 5hmC levels were globally decreased in posterior fossa ependymoma compared with normal cerebellum tissues (P < 0.001). Group A posterior fossa ependymomas had higher 5hmC levels than group B tumors (P = 0.007). Moreover, 5hmC levels positively correlated with Ki-67 index in posterior fossa ependymoma (r = 0.428, P = 0.003). Multivariate Cox hazards model revealed that patients with high 5hmC levels (> 0.102%) had worse PFS and OS than patients with lower 5hmC levels (< 0.102%) (PFS: HR = 3.014; 95% CI, 1.040–8.738; P = 0.042; OS: HR = 2.788; 95% CI, 0.974–7.982; P = 0.047). Conclusions: Our findings suggest that loss of 5hmC is an epigenetic hallmark for pediatric posterior fossa ependymoma. 5hmC levels may represent a potential biomarker to predict prognosis in children with posterior fossa ependymoma. [ABSTRACT FROM AUTHOR]

Background Malignant peripheral nerve sheath tumors (MPNSTs) and cellular schwannomas (CSs) of the eighth cranial nerve are exceedingly rare. The purpose of the present study was to evaluate clinical and genetic characterization of these rare tumors. Methods The clinical and radiological features were analyzed retrospectively. The histopathological characteristics were assessed by hematoxylin and eosin staining and immunohistochemistry. Genomic abnormalities were evaluated using array comparative genomic hybridization. Results Of the 1287 surgeries for vestibular schwannomas from 2014 to 2017, 2 were for MPNSTs and 5 were for CSs. The mean age at diagnosis was older for patients with MPNSTs (57.0 ± 4.2 years) than that of patients with CS (35.8 ± 9.4 years; P = 0.03). Two patients with MPNST died of tumor recurrence. None of the patients with CS died. The 2-year overall and progression-free survival of patients with MPNSTs were worse than those for patients with CSs (overall survival, 50.0% ± 35.4% vs. 100%, P = 0.027; progression-free survival, 0% vs. 100%; P = 0.012). The Ki-67 index for the MPNSTs (29.0% ± 3.5%) was greater than that for the CSs (10.3% ± 3.1%; P = 0.001). The common alterations in MPNSTs mainly included gains of chromosomes 7p, 8p, 9q, 12, and 17 and loss of heterozygosity of 1p, 6 and 9p. The common alterations in CSs included gain of 4p16.3, loss of heterozygosity of 2p15-14, and 22q11.1-13.3. Conclusions To the best of our knowledge, the present study is the first high-resolution genomic analysis of MPNSTs and CSs of the eighth cranial nerve and has shown a significant difference that might be more accurate to distinguish between these 2 types of rare tumors. [ABSTRACT FROM AUTHOR]

Purpose: There is no targeted drug therapy for NF2 patients, and surgery or radiosurgery is not always effective. Therefore, the exploration of new therapeutic pathways is urgently needed.Methods: We analyzed the expression of cytokines in the serum of NF2 patients and determined the percentage of HLA-DR−CD33+CD11b+ cells in blood and NF2-associated schwannomas. Furthermore, we analyzed the role of HLA-DR−CD33+CD11b+ cells in inhibiting T-cell proliferation, cytokine production, and transforming growth factor expression.Results: NF2 patients are in an immunosuppressed state with elevated IL-10 and TGF-β expression in plasma and the lymphocytes from NF2 patients secrete less IFN-γ and CD3+ T cells proliferate slower than normal healthy donors. HLA-DR−CD33+CD11b+ cells frequency significantly increased in the PBMCs and infiltrated in the tumor, these cells express higher iNOS, NOX2 and TGF-β, and induce TGF-β secretion to inhibit CD8+ T-cell proliferation, and induce T-cell transformation to a CD4+CD25+Foxp3+ regulatory T cells phenotype. NF2-associated schwannoma cells induced monocytes transformation into an HLA-DR−CD33+CD11b+ phenotype, and surgical removal of the tumor reduced the percentage of these cells.Conclusions: HLA-DR−CD33+CD11b+ cells may represent a population of MDSCs in NF2 patients. Dissecting the mechanisms behind these suppressive mechanisms will be helpful for the design of effective immunotherapeutic protocols and likely provide a new effective treatment for NF2 patients. [ABSTRACT FROM AUTHOR]

Background: Mixed gangliocytoma-pituitary adenoma is an extremely rare tumor occurring in the sellar region, histologically composed of both gangliocytic and pituitary adenomatous architectures. The histogenesis of these tumors remains unknown, and the diagnosis, treatment, and prognosis are yet to be fully understood.Methods: We retrospectively reviewed the clinical, radiological, and histopathological profiles from a series of 20 patients with sellar mixed gangliocytoma-pituitary adenomas. All patients underwent surgical tumorectomy via an endoscopic or microscopic transsphenoidal approach. Perioperative magnetic resonance imaging (MRI) and computed tomography (CT) data were reviewed. Immunohistochemical stains and electron microscopy examination were performed. Follow-up outcomes were presented.Results: This case series consisted of 13 females and seven males (age range, 20-59 years; mean age, 42.3 ± 11.2 years). Preoperative endocrine examination showed elevated growth hormone (GH) in nine patients and hyperprolactinemia in eight patients. The tumors were positive for GH in 15 cases, prolactin in 13 cases, adrenocorticotropic hormone in three cases, and thyroid-stimulating hormone in one case. Gross total resection was achieved in 15 patients, and subtotal resection in five patients. During an average follow-up period of 42.5 ± 29.0 months, no recurrence was noted.Conclusions: The clinical and neuroimaging features of sellar mixed gangliocytoma-pituitary adenomas are non-specific, and invasion into the cavernous sinus is common. Surgical resection via a transsphenoidal approach is the preferred treatment, and the surgical outcomes are favorable. Moreover, our histopathological findings are more likely to support the theory that mixed gangliocytoma-pituitary adenoma originates from the neuronal transdifferentiation of adenomatous cells. [ABSTRACT FROM AUTHOR]

Objectives: Auditory brainstem implants (ABIs) may be the only opportunity for patients with NF2 to regain some sense of hearing sensation. However, only a very small number of individuals achieved open-set speech understanding and high sentence scores. Suboptimal placement of the ABI electrode array over the cochlear nucleus may be one of main factors for poor auditory performance. In the current study, we present a method of awake craniotomy to assist with ABI placement. Methods: Awake surgery and hearing test via the retrosigmoid approach were performed for vestibular schwannoma resections and auditory brainstem implantations in four patients with NF2. Auditory outcomes and complications were assessed postoperatively. Results: Three of 4 patients who underwent awake craniotomy during ABI surgery received reproducible auditory sensations intraoperatively. Satisfactory numbers of effective electrodes, threshold levels and distinct pitches were achieved in the wake-up hearing test. In addition, relatively few electrodes produced non-auditory percepts. There was no serious complication attributable to the ABI or awake craniotomy. Conclusions: It is safe and well tolerated for neurofibromatosis type 2 (NF2) patients using awake craniotomy during auditory brainstem implantation. This method can potentially improve the localization accuracy of the cochlear nucleus during surgery. [ABSTRACT FROM AUTHOR]

Lithium is considered a first-line therapy for the treatment of bipolar disorder and was recently shown to be associated with a reduced overall cancer risk. A growing body of evidence has indicated the potential antitumor benefits of this drug. Lithium likely functions as an antitumor agent. In this study, we found that lithium chloride (LiCl) significantly inhibits the proliferation of both RT4 cells and human NF2-associated primary schwannoma cells by inhibiting the expression of apoptosis-related proteins. LiCl-induced cell death exhibits ultrastructural features of necrosis and is reversed by the RIPK1-specific inhibitor necrostatin-1 in a dose-dependent manner, indicating that LiCl induces the necroptosis type of cell death. Moreover, LiCl treatment induces ROS generation and activates the AKT/mTOR pathway, which is reversed by necrostatin-1 treatment. Based on our results, LiCl treatment may induce the programmed cell death of schwannoma cells through AKT- and mTOR-mediated necroptosis, potentially representing a new mechanism by which LiCl induces tumor cell death. Moreover, LiCl may prove to be a new drug for treating schwannoma. [ABSTRACT FROM AUTHOR]

Objectives To determine risk factors and management of intraoperative cerebrospinal fluid (CSF) leakage in endoscopic endonasal transsphenoidal pituitary adenoma surgery. Methods We conducted a retrospective review of 492 patients who, between April 2012 and August 2015, underwent endoscopic endonasal transsphenoidal surgeries for resection of pituitary adenoma. A multivariate statistical analysis was performed to investigate the association of some risk factors with intraoperative CSF leakage. Intraoperative CSF leaks were classified as grade 0, no leak observed; grade 1, small leak without obvious diaphragmatic defect; grade 2, moderate leak; or grade 3, large diaphragmatic defect. Repair methods were based on the CSF leak grade. Results Intraoperative CSF leakage occurred in 86 cases (17.5%). On univariate analysis, there were 3 factors associated with an increased intraoperative CSF leak rate: 1) repeat surgery (repeat 30.0% vs. primary 16.4%; P = 0.033), 2) consistency of the adenoma (tenacious, 27.3% vs. soft, 13.5%; P = 0.000), and 3) tumor size (22.0 ± 9.7mm vs. 25.4 ± 11.5 mm; P = 0.007). However, on multivariate analysis, only tumor consistency ( P = 0.001; odds ratio, 2.379) and tumor size ( P = 0.026; odds ratio, 1.032) were independently associated with intraoperative CSF leaks. In the 86 cases with intraoperative CSF leaks, the degree of intraoperative CSF leakage was categorized grade 1 in 30 cases, grade 2 in 25 cases, and grade 3 in 31 cases. Postoperative CSF leak repair failures occurred in 6 cases (1.2%). Conclusions Intraoperative CSF leaks have a propensity to occur in cases with fibrous or large tumors. Once an intraoperative leak is identified, our graded cranial base repair method is safe and reliable. [ABSTRACT FROM AUTHOR]

Background Xanthogranulomas are uncommon granulomatous entities, and xanthogranulomas occurring in the sellar region are extremely rare, with a limited number of cases reported in the literature. Diagnosis, management, and prognosis of sellar xanthogranulomas are poorly understood. Methods We retrospectively reviewed 14 patients with histologically proven sellar xanthogranulomas treated at our institution between January 2006 and July 2016. Clinical manifestations, radiologic features, operative records, and follow-up outcomes were analyzed. Results There were 9 male and 5 female patients with a mean age of 29.2 years. Clinical manifestations included headache ( n = 12; 85.7%), visual deficiencies ( n = 10; 71.4%), and endocrine disturbances ( n = 6; 42.9%). On magnetic resonance imaging, sellar xanthogranulomas revealed isointensity to hyperintensity on T1-weighted imaging and hyperintensity or heterogeneous intensity on T2-weighted imaging with variable enhancement. Gross total resection of the lesion was achieved in 10 cases, and subtotal resection was achieved in 4 cases. Postoperatively, headache was relieved in all cases; transient diabetes insipidus was observed in 2 cases. There were no procedure-related complications. During a mean follow-up period of 52.9 months, visual deficiencies were improved in 7 (70.0%) cases, whereas endocrine disturbances were improved in only 2 (33.3%) cases. No recurrence or regrowth of the residual lesions was observed. Conclusions Sellar xanthogranuloma should be considered in the differential diagnosis of lesions in the sellar region. Some radiologic characteristics, including hyperintensity on T1-weighted imaging, heterogeneous hyperintensity on T2-weighted imaging, variable enhancement, and absence of calcification, can assist in making the preoperative diagnosis. Surgical outcomes are favorable. [ABSTRACT FROM AUTHOR]

Background Vision is important for patients with hearing loss caused by neurofibromatosis type 2 (NF2). Tumors adjacent to the anterior visual pathway can potentially impair the vision. Only a few case reports and small-series studies have been reported. Objective To evaluate the clinical features of tumors adjacent to the anterior visual pathway in a large series of patients with NF2. Methods Seventy-three patients with potentially vision-impairing tumors were carefully screened from among 467 patients with NF2. Results Among the 73 patients, 31 had intraorbital tumors, 21 had suprasellar meningiomas, and 21 had medial sphenoid ridge meningiomas. Of the 31 patients with intraorbital tumors, 17 had optic nerve sheath meningiomas, 9 had intraorbital schwannomas, 3 had spheno-orbital meningiomas, 1 had an anterior cranial fossa-orbital meningioma, and 1 had a cranio-orbital schwannoma. To the date of the last follow-up, 43 patients (58.9%) experienced visual loss. In most cases, hearing loss tended to occur earlier than visual loss. Six patients underwent early operations, and they recovered well without any further vision damage. Six other patients underwent operations after having no functional visual ability in the affected eyes, and their visual ability was not saved. Conclusions Tumors adjacent to the anterior visual pathway, although uncommon in patients with NF2, can cause progressive visual loss. Early surgical intervention seems to be the primary treatment strategy, except for in patients' optic nerve sheath meningiomas. If patients adopt a wait and see policy, regular visual examination seems to be mandatory. [ABSTRACT FROM AUTHOR]

Objective:Granular cell tumors (GCTs) in the central nervous system (CNS) are extremely rare. We report here a series of eight GCTs at various locations in the CNS and provide a full review of the published literature. Methods:Eight patients with pathologically confirmed GCT in the CNS were retrospectively reviewed. The patients were followed up via telephone interview or an outpatient department. Results:Five patients were female, and three were male. Of the eight tumors, three were located at the sellar region, two were located in the spinal canal, one was located at the cerebral hemisphere, one was parasellar-nasal communicating, and one was combined with an enterogenous cyst at the ventral aspect of the medulla oblongata. Six patients were symptomatic, and two patients were found incidentally. Most GCTs tended to be homogeneous and well defined on radiological images. The parasellar-nasal communicating GCT showed destruction of the skull base and an evident cystic component in the sphenoid sinus. The various GCTs had similar histological features, and they tended to be histologically benign. Most tumors did not recur after the operation. After subtotal resection, one residual spinal GCT regrew during the follow-up period. The patient with the parasellar-nasal communicating GCT developed progressive visual loss in the right eye after the operation; and she received adjuvant radiation therapy. Conclusion:GCTs at different locations in the CNS can have significantly different clinical features and should be considered distinct entities. Except for the granular cell astrocytoma (GCA), most GCTs in the CNS tended to be benign. Malignant GCTs are rare and difficult to treat. [ABSTRACT FROM AUTHOR]

Objective: To investigate changes of vision-related resting-state activity in pituitary adenoma (PA) patients with visual damage through comparison to healthy controls (HCs). Methods: 25 PA patients with visual damage and 25 age- and sex-matched corrected-to-normal-vision HCs underwent a complete neuro-ophthalmologic evaluation, including automated perimetry, fundus examinations, and a magnetic resonance imaging (MRI) protocol, including structural and resting-state fMRI (RS-fMRI) sequences. The regional homogeneity (ReHo) of the vision-related cortex and the functional connectivity (FC) of 6 seeds within the visual cortex (the primary visual cortex (V1), the secondary visual cortex (V2), and the middle temporal visual cortex (MT+)) were evaluated. Two-sample t-tests were conducted to identify the differences between the two groups. Results: Compared with the HCs, the PA group exhibited reduced ReHo in the bilateral V1, V2, V3, fusiform, MT+, BA37, thalamus, postcentral gyrus and left precentral gyrus and increased ReHo in the precuneus, prefrontal cortex, posterior cingulate cortex (PCC), anterior cingulate cortex (ACC), insula, supramarginal gyrus (SMG), and putamen. Compared with the HCs, V1, V2, and MT+ in the PAs exhibited decreased FC with the V1, V2, MT+, fusiform, BA37, and increased FC primarily in the bilateral temporal lobe (especially BA20,21,22), prefrontal cortex, PCC, insular, angular gyrus, ACC, pre-SMA, SMG, hippocampal formation, caudate and putamen. It is worth mentioning that compared with HCs, V1 in PAs exhibited decreased or similar FC with the thalamus, whereas V2 and MT+ exhibited increased FCs with the thalamus, especially pulvinar. Conclusions: In our study, we identified significant neural reorganization in the vision-related cortex of PA patients with visual damage compared with HCs. Most subareas within the visual cortex exhibited remarkable neural dysfunction. Some subareas, including the MT+ and V2, exhibited enhanced FC with the thalamic pulvinar, which indicates an important role in the compensatory mechanism following visual impairment. In addition, neural dysfunction within the visual cortex was associated with neural activity alternation in the higher-order cognitive cortex, especially subareas in default mode network (DMN) and salience network (SN). [ABSTRACT FROM AUTHOR]

Background: Clear cell meningioma (CCM) is a rare subtype of meningioma. We present the largest series of 36 CCMs and evaluate several prognostic factors of patient's clinical outcome. Methods: Thirty-six patients with pathologically confirmed CCM among a total of 10,529 meningioma patients were retrospectively reviewed. Results: CCM constituted 0.3 % of the intracranial meningiomas and 1.4 % of the intraspinal meningiomas. The male-to-female ratio (36 vs 64 %) for CCMs was similar to that for total meningiomas (28 vs 72 %) patients (chi-squared test, p = 0.3). The mean age at diagnosis of CCM patients (29.3 ± 18.4 years) was significantly younger than that of total meningiomas (49.8 ± 11.9 years) patients ( t-test, p = 0). During the follow-up, 15 patients (42 %) suffered from tumor recurrence. The recurrence time ranged from 10 months to 12 years, with a median time of 29 months. Kaplan-Meier survival analysis showed that patients after total resection (Simpson grades I and II) had significantly longer progression-free survival (PFS) time than those after subtotal resection (Simpson grades III and IV) (log-rank test, p = 0.006). However, age (≤20 years or >20 years, p = 0.9), gender ( p = 0.3), postoperative radiotherapy ( p = 0.4), progesterone receptor staining (positivity or negativity, p = 0.2), and Ki-67 index (≤5 % or >5 %, p = 0.4) did not have significant effects on patients' PFS time. Conclusions: The proportion of CCM in spinal meningiomas is likely to be much larger than that in intracranial meningiomas. CCMs should be resected totally when possible to decrease the risk of recurrence or prolong patient's PFS time. [ABSTRACT FROM AUTHOR]

The deprivation of sensory input after hearing damage results in functional reorganization of the brain including cross-modal plasticity in the sensory cortex and changes in cognitive processing. However, it remains unclear whether partial deprivation from unilateral auditory loss (UHL) would similarly affect the neural circuitry of cognitive processes in addition to the functional organization of sensory cortex. Here, we used resting-state functional magnetic resonance imaging to investigate intrinsic activity in 34 participants with UHL from acoustic neuroma in comparison with 22 matched normal controls. In sensory regions, we found decreased regional homogeneity (ReHo) in the bilateral calcarine cortices in UHL. However, there was an increase of ReHo in the right anterior insular cortex (rAI), the key node of cognitive control network (CCN) and multimodal sensory integration, as well as in the left parahippocampal cortex (lPHC), a key node in the default mode network (DMN). Moreover, seed-based resting–state functional connectivity analysis showed an enhanced relationship between rAI and several key regions of the DMN. Meanwhile, lPHC showed more negative relationship with components in the CCN and greater positive relationship in the DMN. Such reorganizations of functional connectivity within the DMN and between the DMN and CCN were confirmed by a graph theory analysis. These results suggest that unilateral sensory input damage not only alters the activity of the sensory areas but also reshapes the regional and circuit functional organization of the cognitive control network. [ABSTRACT FROM AUTHOR]

This study aims to explore the probable mechanism of better result of dural reconstruction by CO2 laser soldering and the effect of exogenous basic fibroblast growth factor (bFGF) or transforming growth factor-beta1(TGFβ1) on wound healing. In part I of the study, ten minipigs were randomized into two equal groups, and the dural defects were reconstructed by conventional fibrin glue (FG) bonding (group I a) or by CO2 laser soldering (group Ib). In part II, 36 minipigs were randomized into three equal groups, and the dural defect was reconstructed by CO2 laser soldering; then exogenous bFGF or TGFβ1 was administered in group IIb and group IIc, respectively, while group IIa served as control group. The dural specimens were harvested at 1st week postoperatively in part I; and at 1st, 2nd, 3rd, and 4th week postoperatively in part II, they were examined for healing condition and subjected to hematoxylin-eosin (HE) staining and immunohistochemical (IHC) staining with antibodies against bFGF and TGFβ1. In part I, group Ib showed higher fibroblast cell density than group Ia (P < 0.05). The optical density (OD) for IHC staining with antibodies against bFGF of group Ib was significantly higher than that of group Ia (P < 0.05), and for IHC staining with antibodies against TGFβ1, group Ib showed positive staining while group Ia was negative. In part II, administering exogenous bFGF or TGFβ1 made a left shift of fibroblast cell number-time curve compared with control group. For specimens' IHC staining with antibodies against bFGF, the OD of group IIb was higher than that of group IIa in the corresponding time. For specimens' IHC staining with antibodies against TGFβ1, the OD of groups IIb and IIc was both higher than that of group IIa (P < 0.05 and P < 0.01, respectively). In conclusion, CO2 laser may trigger fibroblast proliferation through stimulating the secretion of bFGF and TGFβ1. Topically administering exogenous bFGF or TGFβ1 could accelerate the healing of the reconstructed dura by enhancing secretion of bFGF and/or TGFβ1 and promoting the process of fibroblast gathering-degrading. [ABSTRACT FROM AUTHOR]

This study aims to explore the probable mechanism of better result of dural reconstruction by CO laser soldering and the effect of exogenous basic fibroblast growth factor (bFGF) or transforming growth factor-beta1(TGFβ) on wound healing. In part I of the study, ten minipigs were randomized into two equal groups, and the dural defects were reconstructed by conventional fibrin glue (FG) bonding (group I ) or by CO laser soldering (group I). In part II, 36 minipigs were randomized into three equal groups, and the dural defect was reconstructed by CO laser soldering; then exogenous bFGF or TGFβ was administered in group II and group II, respectively, while group II served as control group. The dural specimens were harvested at 1st week postoperatively in part I; and at 1st, 2nd, 3rd, and 4th week postoperatively in part II, they were examined for healing condition and subjected to hematoxylin-eosin (HE) staining and immunohistochemical (IHC) staining with antibodies against bFGF and TGFβ. In part I, group I showed higher fibroblast cell density than group I ( P < 0.05). The optical density (OD) for IHC staining with antibodies against bFGF of group I was significantly higher than that of group I ( P < 0.05), and for IHC staining with antibodies against TGFβ, group I showed positive staining while group I was negative. In part II, administering exogenous bFGF or TGFβ made a left shift of fibroblast cell number-time curve compared with control group. For specimens' IHC staining with antibodies against bFGF, the OD of group II was higher than that of group II in the corresponding time. For specimens' IHC staining with antibodies against TGFβ, the OD of groups II and II was both higher than that of group II ( P < 0.05 and P < 0.01, respectively). In conclusion, CO laser may trigger fibroblast proliferation through stimulating the secretion of bFGF and TGFβ. Topically administering exogenous bFGF or TGFβ could accelerate the healing of the reconstructed dura by enhancing secretion of bFGF and/or TGFβ and promoting the process of fibroblast gathering-degrading. [ABSTRACT FROM AUTHOR]

Cognitive impairments and social-function deficits are severe complaints in neurofibromatosis type 1 (NF1) patients. Empathetic pain perception may be disrupted in NF1 patients because of high-level cognitive deficits. This study investigated the empathy profiles of adult patients with NF1, especially concerning whether explicit and implicit empathetic pain perception are abnormal in this population. We examined empathetic pain perception through a paradigm based on perceiving another person's pain; in this task, patients were required to make judgments about the presence of pain or the laterality of the body part, as shown in a picture. Twenty NF1 patients without obvious social or communication difficulties completed the task, and the results were compared with results from the normal controls (NCs). Regarding explicit empathetic pain processing, i.e., judging the presence of "pain" or "no pain", there were no significant differences between patients and controls in accuracy or reaction time. However, in implicit empathetic processing, i.e., judging the laterality of "pain" or "no-pain" pictures, NF1 patients had significantly lower accuracy (p = 0.038) and significantly higher reaction times (p = 0.004) than the NCs. These results were consistent with those of a previous study showing that high-level cognitive deficits were prominent in NF1 patients when performing challenging tasks. The mechanisms and related brain network activity underlying these deficits should receive attention in the future. [ABSTRACT FROM AUTHOR]

Objective Vestibular schwannomas (VSs) can cause serious neurological defects including hearing loss and facial paralysis. The aim of this study is to identify whether Hippo signaling could be a potential targetable pathway for clinical treatment in VSs. Methods Gene expression profiling was performed in 10 sporadic VSs and 4 normal nerves to identify aberrant genes expression of the Hippo pathway. Western blotting and immunohistochemical staining were used to examine the expression of Hippo core components in 20 VS samples. Neurofibromatosis type 2 ( NF2 ) gene sequencing was also performed in all tumors using sanger sequencing. Verteporfin, inhibitor of yes-associated protein (YAP)-TEA domain family member, was used to assess the effect of proliferation inhibition in human primary VS cells and RT4-D6P2T cell line. Results We found 51 differentially expressed genes of the Hippo pathway between VSs and healthy controls. Unsupervised analysis identified the 2 molecular variants that significantly related with distinct NF2 mutation status. The phosphorylation levels of large tumor suppressor 1 and YAP were significantly decreased in NF2 -mutated VSs compared with wild-type VSs and normal nerves. Immunohistochemical staining showed that increased nuclear YAP expression in VSs was positively correlated with high Ki-67 index and low Merlin expression. Verteporfin reduced viability of primary VS cells and RT4-D6P2T cells. Conclusions Our findings implicate that deregulation of the Hippo pathway as a molecular mechanism of pathogenesis in human VSs, and suggest inhibition of this pathway as a potential treatment strategy. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 16-year-old boy with neurofibromatosis 1 (NF1) who developed bilateral cranio-orbital neurofibromas. Topics discussed include the decrease in the child's visual acuity, the frontotemporal craniotomy and orbitozygomatic osteotomy was administered for the resection of the child's right-sided tumor, and the efficacy of pterional approach in resecting the cranio-orbital communicating tumors.