Background The association between pregnancy and the risk of symptomatic hemorrhage (SH) in brainstem cavernous malformations remains uncertain. We aimed to quantify the risk of pregnancy‐associated SH in women with brainstem cavernous malformations. Methods We analyzed women with brainstem cavernous malformations in a prospective registry (2016–2022) experiencing pregnancy after enrollment. Follow‐up was divided into pregnancy‐associated segments (pregnancy plus ≤6 weeks postpartum or postabortion) and nonpregnant segments. Primary analysis used case‐crossover models; propensity score‐matched analyses assessed robustness. Results Among 63 women (median age, 30.0 years), 53 (84.1%) had bled before enrollment, including 45 (71.4%) with SH in the preceding year. Prospectively, they contributed 72 pregnancies and 371.7 patient‐years, observing 34 SHs. The pregnancy annual SH rate was 20.4% versus 7.2% nonpregnant (adjusted incidence rate ratio: 2.56, 95% CI, 1.11–5.93; P=0.028). Trimester‐specific and postpartum annual SH rates were 5.9%, 16.8%, 34.6%, and 38.3%; risk significantly increased only during the third trimester (P=0.004) and postpartum (P=0.007). This pregnancy effect was confined to segments with SH in the preceding year (annual SH rates 46.7% versus 9.4%; P=0.007), which was an independent risk factor. Propensity score‐matched analyses yielded consistent results. Conclusions Pregnancy is associated with an elevated SH risk in women with brainstem cavernous malformations, specifically among those with SH in the preceding year. Because of selection bias, generalizability to asymptomatic or incidental lesions is limited, warranting cautious extrapolation. Registration URL: www.chictr.org.cn; Unique Identifier: ChiCTR‐POC‐17011575.

Low triiodothyronine (T3) syndrome, also known as non-thyroidal illness syndrome (NTIS), was one of the common endocrinopathies in critical illness. The potential impacts of low T3 syndrome on survival, endocrine function, and nutritional status of patients with aggressive mature B-cell non-Hodgkin lymphoma (NHL) needed to be explored. We enrolled 225 patients. T3 levels were captured when starting chemotherapy, finishing chemotherapy, and at the first follow-up visit from 6 months after chemotherapy. Latest ultrasound results were recorded. Kaplan-Meier curves were used to compare overall survival (OS) or progression-free survival (PFS). We performed Cox's proportional hazards regression model to analyze prognostic factors of OS and PFS. Ultrasound abnormality and weight gain were tested with the χ2 test. The percentage of patients with low T3 syndrome decreased from 55.1% (124 out of 225) to 2.0% (4 out of 201), then further dropped down to 0 (0 out of 173). With a median follow-up of 32.9 months, low T3 syndrome was identified as a statistically significant factor affecting both OS (p = .047; hazard ratio [HR] = 8.18, 95% CI: 1.03-64.97) and PFS (p = .049; HR = 4.64, 95% CI: 1.01-21.31) in multivariate analysis. No significant effects of low T3 syndrome on abnormal thyroid ultrasound results and weight gain were found. In conclusion, low T3 syndrome has a high incidence in pediatric patients with aggressive mature B-cell NHL, and low T3 syndrome has a significant impact on long-term survival. It appears transient and could not contribute to impaired thyroid function.

Objectives This study aimed to develop a MRI-based prognostic nomogram including radiomics and clinical information for brainstem cavernous malformation patients (BSCMs). Methods 114 BSCMs were randomly divided into a training cohort and a validation cohort. Clinical and radiomics nomograms were constructed. Radiomics features were selected using three algorithms: univariate analysis, Pearson correlation, and the elastic net algorithm. A Cox regression model was employed to build the radiomics nomogram. The concordance index (C-index), time-independent receiver operating characteristic (ROC) analysis, and decision curve analysis (DCA) were used to evaluate the clinical utility of the nomogram. Results The radiomics signature score was calculated using 11 radiomics features related to hemorrhage-free survival (HFS) from the training cohort. Patients were stratified into high-risk and low-risk groups based on the radiomics signature, with the low-risk group demonstrating significantly better HFS. Additionally, three clinical factors—number of hemorrhages, lesion size, and modified Rankin Scale score—along with the radiomics score were used to develop the radiomics nomogram. Calibration plots indicated good agreement between predicted and actual survival probabilities. The C-index for the training and validation cohorts was 0.784 and 0.787, respectively, in predicting HFS. The area under the curve was 72.51 and 76.41 for 3-year survival, and 67.62 and 72.57 for 5-year survival in the training and validation cohorts, respectively. The DCA curve demonstrated that the radiomics nomogram had superior clinical utility compared to the clinical model. Conclusions The radiomics nomogram showed great potential as a sensitive prognostic tool in predicting hemorrhage-free survival in BSCMs.

Background and ObjectivesFamilial cavernous malformations (FCMs) are vascular lesions that pose a lifelong risk of symptomatic hemorrhage (SH) and seizures, yet their natural history remains unclear. This study aims to determine the cumulative lifetime risk of a first SH and/or seizure and assess whether genetic variations influence these risks.MethodsThis international, multicenter retrospective cohort study included data from 16 tertiary referral centers and 1 patient advocacy group. Eligible patients had confirmed or suspected FCM, available magnetic resonance imaging (MRI) data, documented baseline clinical features, and longitudinal follow-up (FU). Functional outcomes were assessed using the modified Rankin Scale (mRS) at last FU. Direct adjusted survival curves and mixed-effects Cox regression analyses were performed to estimate cumulative lifetime risk. The association between genetic variations and SH/seizure rates was evaluated, and mixed-effects logistic regression assessed the effect of SH/seizures on mRS outcomes.ResultsA total of 1,592 patients with FCM were included, with a mean age of 37.6 years (SD 17.1) and 55.7% female. The median FU was 42 years (IQR: 27-55), totaling 64,146 person-years. Of these, 869 (54.6%) had confirmed FCM, 775 (48.7%) experienced at least 1 hemorrhage, and 447 (28.1%) had at least 1 seizure. Genetic testing was performed in 47.7%, identifying CCM1 (31.0%), CCM2 (4.8%), and CCM3 (1.9%) variations. The lifetime risk of a first SH was similar to 80%, with an event rate that remained constant beyond age 20. The lifetime risk of a first seizure was similar to 45%. Patients with CCM3 variations exhibited a more aggressive hemorrhagic course than those with CCM1 (hazard ratio 1.799, 95% CI 1.008-3.208). SH and seizures were independently associated with worse mRS outcomes at last FU.DiscussionThe event rate of SH and seizures remained stable over time, leading to high cumulative lifetime risks. Patients with CCM3 variations exhibited a more aggressive disease course. Limitations include the non-population-based design, selection bias from tertiary centers, retrospective data collection, and variability in data extraction across centers. However, this study represents the largest international FCM cohort to date, improving the precision of risk estimates and providing valuable insights into disease progression.

High-grade meningioma remains a therapeutic challenge. The first-line guideline drugs for high-grade meningioma are still lacking, highlighting the urgent need to uncover new therapeutic targets. As a cationic amino acid transporter, SLC7A1 was highly expressed in high-grade meningioma and associated with poor prognosis of patients. In this study, transcriptomic analyses at both the single-cell and bulk levels were employed to investigate the molecular function of SLC7A1. The Genomics of Drug Sensitivity in Cancer (GDSC) database was utilized for predicting potential drugs targeting high-SLC7A1 meningiomas. RNA sequencing was conducted to explore the differential activity of cancer hallmark pathways and transcription factors. The effects of SLC7A1 knockdown and drug treatment were validated in vitro and in vivo. Our results revealed that SLC7A1 regulates multiple signaling pathways involved in tumor proliferation, including E2F targets, G2M checkpoint, and MYC targets. Knockdown of SLC7A1 significantly inhibited the proliferation, invasion, and xenograft tumor growth of meningioma cells. Furthermore, SLC7A1-FOXM1/E2F4 regulatory axis may contribute to the malignant progression of meningioma. AZ628, predicted as a small molecule drug targeting high-SLC7A1 meningiomas, exhibited an excellent antitumor effect against meningioma in vitro, in vivo, and in organoid models. Additionally, AZ628 treatment also inhibited the transcriptional activity and protein expression of FOXM1 and E2F4, mirroring the effects of SLC7A1 knockdown in meningioma. In brief, our study demonstrated the tumor-promoting function of SLC7A1 by regulating the transcription factors FOXM1 and E2F4 in meningioma and identified SLC7A1 as a potential therapeutic target. Meanwhile, AZ628 is a promising small molecule drug for high-grade meningioma.

Background This study investigates the predictive value of baseline quantitative susceptibility mapping (QSM) metrics for assessing the risk of future symptomatic haemorrhages in patients with brainstem cavernous malformations (CMs). Methods From July 2020 to September 2023, a prospective multicentre cohort of 155 patients with brainstem CMs was enrolled from 12 institutions. We analysed baseline QSM metrics, including lesional mean, median, IQR and maximum susceptibility. Propensity score matching was adjusted for baseline confounders, and Cox regression models assessed haemorrhage risk. Risk stratification was performed based on thresholds determined from planned receiver operating characteristic (ROC) analyses. Results Postmatching cohorts (56 haemorrhage-free vs 30 haemorrhage cases) showed balanced baseline characteristics. Over a mean follow-up of 22.6 months, the baseline QSM metrics, particularly the median susceptibility (QSMmedian) (HR 58.896, 95% CI 8.544 to 405.989, p<0.001; Bonferroni-adjusted p=0.0001, k=4) and IQR of susceptibility (QSMIQR) (HR 29.754, 95% CI 6.101 to 145.119, p<0.001; Bonferroni-adjusted p=0.0001, k=4) were associated with prospective haemorrhage after adjusting for age, gender, lesion volume and prior haemorrhage. QSMmedian (area under curve (AUC)=0.759) and QSMIQR (AUC=0.740) demonstrated modest predictive performance. Risk stratification based on QSMmedian and QSMIQR demonstrated 2-year haemorrhage-free survival rates of 83.3%, 62.8% and 35.7% for the low-risk, intermediate-risk and high-risk groups, respectively. High-risk patients showed a 7.7-fold greater risk of haemorrhage compared with the low-risk group. Conclusions This study explored the predictive value of QSM metrics for future symptomatic haemorrhage, suggesting that QSM may serve as a complementary imaging biomarker to existing prognostic models. Further validation in larger, independent cohorts is warranted.

BACKGROUND AND OBJECTIVES:Familial cavernous malformations (FCMs) are vascular lesions that pose a lifelong risk of symptomatic hemorrhage (SH) and seizures, yet their natural history remains unclear. This study aims to determine the cumulative lifetime risk of a first SH and/or seizure and assess whether genetic variations influence these risks. METHODS:This international, multicenter retrospective cohort study included data from 16 tertiary referral centers and 1 patient advocacy group. Eligible patients had confirmed or suspected FCM, available magnetic resonance imaging (MRI) data, documented baseline clinical features, and longitudinal follow-up (FU). Functional outcomes were assessed using the modified Rankin Scale (mRS) at last FU. Direct adjusted survival curves and mixed-effects Cox regression analyses were performed to estimate cumulative lifetime risk. The association between genetic variations and SH/seizure rates was evaluated, and mixed-effects logistic regression assessed the effect of SH/seizures on mRS outcomes. RESULTS:A total of 1,592 patients with FCM were included, with a mean age of 37.6 years (SD 17.1) and 55.7% female. The median FU was 42 years (IQR: 27-55), totaling 64,146 person-years. Of these, 869 (54.6%) had confirmed FCM, 775 (48.7%) experienced at least 1 hemorrhage, and 447 (28.1%) had at least 1 seizure. Genetic testing was performed in 47.7%, identifying CCM1 (31.0%), CCM2 (4.8%), and CCM3 (1.9%) variations. The lifetime risk of a first SH was ∼80%, with an event rate that remained constant beyond age 20. The lifetime risk of a first seizure was ∼45%. Patients with CCM3 variations exhibited a more aggressive hemorrhagic course than those with CCM1 (hazard ratio 1.799, 95% CI 1.008-3.208). SH and seizures were independently associated with worse mRS outcomes at last FU. DISCUSSION:The event rate of SH and seizures remained stable over time, leading to high cumulative lifetime risks. Patients with CCM3 variations exhibited a more aggressive disease course. Limitations include the non-population-based design, selection bias from tertiary centers, retrospective data collection, and variability in data extraction across centers. However, this study represents the largest international FCM cohort to date, improving the precision of risk estimates and providing valuable insights into disease progression.

Limited data regarding intracranial adenoid cystic carcinomas (ACCs) were available. The authors aimed to elaborate on the clinical characteristics, treatment strategy, and poor outcomes of the disease. Clinical data from all cases of intracranial ACCs treated at our institute were reviewed retrospectively to evaluate their clinical characteristics, management, and outcomes. our series included 10 males and 9 females, with a mean age of 47.3 years. The most common presentations were cranial neuropathies (11 cases), followed by headache (4 cases), and nasal symptoms (4 cases). The radiologic spectrum for intracranial ACCs was broad. Gross total resection (GTR), subtotal resection, and partial resection were performed in 8 (42.1%), 6 (31.6%) and 5 (26.3%) patients, respectively. After a median follow-up of 35.0 months (range, 3.0-89.0 months), 8 patients (42.1%) died. The 1-, 3-, and 5-year rates of progression-free survival and overall survival were 89.2%, 57.3%, and 20.5% and 89.5%, 57.5%, and 32.9%, respectively. Although the differences were not significantly different, GTR and the use of radiotherapy and chemotherapy tended to improve the prognosis of the patients. Intracranial ACCs are rare neoplasms. GTR alone, if tolerable, is advocated as the optimal treatment for intracranial ACCs. Nevertheless, conservative excision may be preferred to avoid damage to vital structures. Radiotherapy and chemotherapy may be an alternative treatment. Intracranial ACCs tend to recur or metastasis within a few years of the initial surgery.

The role of radiosurgery in preventing haemorrhage in brainstem cavernous malformations remains a subject of debate. This study aimed to evaluate whether radiosurgery provides a protective benefit against haemorrhage in these patients. This multicentre, prospective observational study was conducted in 17 centres and enrolled eligible patients with brainstem cavernous malformations consecutively. Data collected included clinical baseline information, radiosurgery planning details, periodic follow-up evaluations and any adverse radiation effects. The primary outcome of the study was the incidence of first prospective haemorrhage, and the secondary outcome was the development of new or worsening neurological dysfunctions. The impact of radiosurgery was assessed using multivariate Cox regression analysis. From March 2016 to August 2018, the study enrolled 377 patients: 280 in the observation group receiving standard care alone and 97 in the radiosurgery group receiving both radiosurgery and standard care. The overall cohort consisted of 173 females (45.9%) with a mean age of 40.5 years (range, 18-68 years), and there were no significant differences in baseline characteristics between the two groups. After a median follow-up period of 70 months, haemorrhage occurred in 25.0% (n = 70) of patients in the observation group and 10.3% (n = 10) of patients in the radiosurgery group. Multivariate Cox regression analysis identified radiosurgery as an independent protective factor against haemorrhage (hazard ratio 0.379, 95% confidence interval 0.195-0.738, P = 0.004). Following 1:2 propensity score matching, the incidence of prospective haemorrhage was 24.9% (45/181) in the observation group compared with 10.3% (10/97) in the radiosurgery group (hazard ratio 0.379, 95% confidence interval 0.190-0.755, P = 0.006). Adverse radiation effects were observed in 12 patients (12.4%), with none being permanent. Additionally, new or worsening neurological dysfunctions were significantly more common in the observation group (28.9%) compared with the radiosurgery group (16.5%) (P = 0.016). These results suggest that radiosurgery is associated with a low rate of haemorrhage in patients with brainstem cavernous malformations and could provide a benefit in selected patients. However, further research is required to confirm these findings. In a prospective cohort study, Li et al. found that patients with brainstem cavernous malformations who received radiosurgery plus standard medical care had lower rates of haemorrhage, and of new or worsening neurological dysfunction, than patients who received standard medical care alone.

Objective To evaluate the clinical features, surgical outcomes, and predictors of progression-free survival (PFS) in patients with endolymphatic sac tumors (ELSTs). Methods This retrospective study analyzed 15 cases from Beijing Tiantan Hospital and 237 from the literature (1988–2023), focusing on patients with pathologically confirmed intracranial or skull ELSTs who had comprehensive treatment and follow-up records. Univariate and multivariate Cox regression analyses were used to identify factors influencing PFS. Results Patients from our institute comprised 10 males and 5 females, with an average age of 39.1 years. Among these patients, 86.7% underwent gross total resection (GTR). During the follow-up period, 2 patients (13.3%) were lost to follow-up. After a mean follow-up of 74.9 months, 1 patient experienced recurrence and another died from unrelated causes. A review of the literature identified 237 additional patients, including 134 females (56.5%), with an average age of 39.8 years; 22.8% of these patients had von Hippel-Lindau disease. The GTR rate was 69.2%. After a mean follow-up of 53.2 months, 33 recurrences occurred, and the median PFS was 48 months. In addition, 8 patients died during the follow-up period; none of the deaths was attributed to ELSTs. Multivariate analysis identified GTR (hazard ratio, 0.279; 95% confidence interval, 0.086–0.903; P = 0.033) as a significant protective factor against recurrence among the pooled cases. Conclusions GTR is crucial for improving PFS in patients with ELST, emphasizing the need for advanced surgical techniques and long-term follow-up because of potential recurrences.

OBJECTIVE: Based on anatomical magnetic resonance imaging (MRI) sequences, we developed a radiomic signature for brainstem cavernous malformation patients (BSCMs) using radiomic analysis and explore its effectiveness as a prognostic biomarker. METHODS:One hundred and fourteen BSCMs with clinical, and radiomic information were collected and randomly divided into training (n = 68) and validation set (n = 46). Clinical and radiomic nomogram were constructed for the prognosis. Radiomic features were screened with three algorithms (univariate analysis, Pearson analysis, and elastic net algorithm). Cox regression model was used to build the radiomics nomogram. Finally, concordance index (C-index), time-independent receiver operating characteristic (ROC) analysis, and Decision curve analysis (DCA) were utilized to evaluate the clinical application of the radiomics nomogram. RESULTS: The radiomic signature score was calculated with 11 hemorrhage-free survival (HFS) related radiomic features from the training cohort. The patients were divided into high-risk group and low-risk group with the help of radiomic signature and the low-risk group has a better HFS than the high-risk group. In addition, three clinical characteristics including the number of hemorrhages, size, mRS, and radiomics score (Rad-score) were used to develop the radiomics nomogram. The calibration plots showed that the nomogram has good agreement between the predicted and actual survival probabilities. And, the C-index was 0.784 and 0.787 in the training cohort and validation cohort in predicting HFS; the area under curve (AUC) was 72.51 and 76.41 in the training cohort and validation cohort in 3-year survival and 67.62 and 72.57 in 5-year survival. Lastly, the DCA curve showed that the radiomics nomogram has a better clinical application than the clinical model. CONCLUSIONS: Radiomics nomogram integrating radiomics signature and clinical information showed great performance and high sensitiveness in prediction HFS in BSCMs than the clinical model.

Local progression of primary skull base chordoma (PSBC) is a sign of treatment failure. Predicting the postoperative progression of PSBC can aid in the development of individualized treatment plans to improve patients’ progression-free survival (PFS) after surgery. This study aimed to develop a multiparametric MRI-based fusion radiomic model (FRM) and clinicoradiomic model (CRM) via radiomic and clinical analysis and to explore their validity in predicting postoperative progression in PSBC patients before surgery. In this retrospective study, a total of 129 patients with PSBC from our institution, including 57 patients with progression, were enrolled and randomized to the training set (TS) or the validation set (VS) at a 2:1 ratio. Radiomic features were extracted and dimensionally reduced from 3.0 T/axial T2-weighted imaging (T2WI), T1-weighted imaging (T1WI) and contrast-enhanced T1-weighted imaging (CE-T1WI) sequences for each patient, and the features were used for radiomic analysis. Univariate and multivariate Cox regression analyses were used to screen for key clinical factors. We constructed models on the basis of multivariate logistic regression analysis. Receiver operating characteristic (ROC) curve, calibration curve, and decision curve analyses were performed to evaluate the performance of the clinical model (CM), FRM and CRM. Through analysis, we found that blood supply was the only significantly different clinical factor in the CM. For the FRM, the area under the receiver operating characteristic curve (AUC) of the TS was 0.925, and the calibration curves were consistent across the TS. In the CRM, the AUC of the TS was 0.929, the calibration curve analysis was consistent for both the TS and the VS, and the DCA showed that the net benefit was greater at a threshold probability of > 0% for both the TS and the VS. Our proposed FRM can help clinicians better predict PSBC progression preoperatively, and the use of the CRM can lead to the development of more appropriate protocols to improve patients’ PFS after surgery.

OBJECTIVE:To observe the evolution and outcomes of postoperative trigeminal neuropathy following surgery of tumor involving the trigeminal nerve.METHODS:A prospective observational study was conducted between October 2018 and February 2019 involving 25 patients with tumors confirmed to involve the trigeminal nerve during surgery by senior author. Pre- and postoperative trigeminal nerve function status and clinical data were recorded.RESULTS:This study included 18 cases of meningioma and seven of trigeminal schwannoma. Among the meningioma cases, 55.6% of the patients reported facial sensory dysfunction before surgery, 33.3% presented ocular discomfort, and 5.6% had masticatory muscle atrophy. Postoperatively, all patients experienced facial paresthesia, 94.4% complained of eye dryness, and one (5.56%) exhibited keratitis. Additionally, one patient (5.56%) showed new-onset masticatory weakness. During follow-up, 50.0% of patients reported improvement in facial paresthesia, and one (5.56%) experienced deterioration. Eye dryness resolved in 35.3% of patients, and keratitis remission was observed in one patient. However, one patient (5.56%) developed neurotrophic keratitis. Overall, 55.6% of patients displayed mild masticatory weakness without muscle atrophy. In the cases of schwannoma, 28.6% of patients had facial paresthesia before surgery, 42.9% showed ocular discomfort, and one (14.3%) complained of masticatory dysfunction. Postoperatively, 85.7% of patients reported facial paresthesia and eye dryness, with one patient (16.7%) experiencing keratitis. During follow-up, 66.7% of patients demonstrated improvement in facial paresthesia, 28.6% showed eye dryness remission, and one patient (16.7%) recovered from keratitis. However, one patient (16.7%) developed new-onset neurotrophic keratitis. One patient (16.7%) experienced relief of masticatory dysfunction, but 42.9% reported mild deterioration. Another patient (14.3%) had facial anesthesia that had not improved.CONCLUSION:Postoperative trigeminal neuropathy is a common complication with a high incidence rate and poor recovery outcomes after surgery for tumors involving the trigeminal nerve. When trigeminal nerve damage is unavoidable, it is essential to provide a multidisciplinary and careful follow-up, along with active management strategy, to mitigate the more severe effects of postoperative trigeminal neuropathy.

OBJECTIVE:Rosai-Dorfman disease (RDD) is a rare, idiopathic, and non-neoplastic histio-proliferative disease that is uncommon in the central nervous system. Hence, reports of management of RDD in the skull base are scarce and only a few studies on skull base RDD are available. The objective of this study was to analyze the diagnosis, treatment, and prognosis of RDD in the skull base and explore an appropriate treatment strategy thereof. METHODS:Nine patients with clinical characteristics and follow-up data from our department between 2017 and 2022 were included in this study. From this information, the clinical profiles, imaging, treatment, and prognosis data were collected. RESULTS:There were 6 male and 3 female patients with skull base RDD. These patients ranged in age from 13 to 61 years, with a median age of 41 years. The locations included 1 anterior skull base orbital apex, 1 parasellar region, 2 sellar regions, 1 petroclivus, and 4 foramen magnum regions. Six patients underwent total resection and 3 underwent subtotal resection. Patient follow-up lasted 11-65 months, with a median duration of 24 months. One patient died, 2 experienced recurrence, and the other patients' lesions were stable. The symptoms worsened and new complications occurred in 5 patients. CONCLUSIONS:Skull base RDDs are intractable diseases with a high rate of complications. Some patients are at risk of recurrence and death. Surgery may be the basic treatment for this disease, and combined therapy including targeted therapy or radiation therapy may also be a valuable therapeutic strategy.

Primary intracranial Rosai-Dorfman disease (PIRDD) is considered a nonmalignant nonneoplastic entity, and the outcome is unclear due to its rarity. The study aimed to elaborate the clinic-radiological features, treatment strategies, and progression-free survival (PFS) in patients with PIRDD. Patients with pathologically confirmed PIRDD in our institute were reviewed. Literature of PIRDD, updated until December 2019, was systematically searched in 7 databases (Embase, PubMed, Cochrane database, Web of Science, Wanfang Data Knowledge Service Platform, the VIP Chinese Science and Technology Periodical Database (VIP), and the China National Knowledge Infrastructure (CNKI)). These prior publication data were processed and used according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Clinical-radiological characteristics and adverse factors for PFS were evaluated in the pooled cohort. The pooled cohort of 124 cases (81 male and 43 female), with a mean age of 39.7 years, included 11 cases from our cohort and 113 cases from 80 prior studies. Twenty-nine patients (23.4%) had multiple lesions. Seventy-four patients (59.7%) experienced gross total resection (GTR), 50 patients (40.3%) had non-GTR, 15 patients (12.1%) received postoperative adjuvant radiation, and 23 patients (18.5%) received postoperative steroids. A multivariate Cox regression revealed that GTR (HR = 4.52; 95% CI 1.21-16.86; p = 0.025) significantly improved PFS, and multiple lesions (p = 0.060) tended to increase the hazard of recurrence. Neither radiation (p = 0.258) nor steroids (p = 0.386) were associated with PFS. The overall PFS at 3, 5, and 10 years in the pooled cohort was 88.4%, 79.4%, and 70.6%, respectively. The PFS at 5 and 10 years in patients with GTR was 85.4% and 85.4%, respectively, which was 71.5% and 35.8%, respectively, in patients without GTR. Gross total resection significantly improved PFS and was recommended for PIRDD. Radiation and steroids were sometimes empirically administered for residual, multiple, or recurrent PIRDD, but the effectiveness remained arguable and required further investigation.Systematic review registration number: CRD42020151294.

Meningioma was the most primary intracranial tumor, but the molecular characteristics and the treatment of malignant meningioma were still unclear. Nine malignant progression-related genes based prognostic signatures were identified by transcriptome analysis between benign meningioma and malignant meningioma. The external dataset GEO136661 and quantitative Real-time Polymerase Chain Reaction were used to verify the prognostic factors. has-miR-3605-5p, hsa-miR-664b-5p, PNRC2, BTBD8, EXTL2, SLFN13, DGKD, NSD2, and BVES were closed with malignant progression. Moreover, Doxorubicin was identified by Connectivity Map website with the differential malignant progression-related genes. CCK-8 assay, Edu assay, wound healing assay, and trans-well experiment were used to reveal that Doxorubicin could inhibit proliferation, migration and invasion of IOMM-Lee Cells.

PurposeTo investigate the different clinical and cytogenetic features of skull base meningiomas (SBMs) and non-SBMs (NSBMs).MethodsWe conducted a retrospective study on a series of 316 patients with primary intracranial meningiomas. The t-test and the Chi-square test were used to analyze the differences between 194 SBMs and 122 NSBMs. The Cox analysis was used to determine prognostic factors for tumor recurrence.ResultsCompared with NSBMs, on average, the age of patients with SBMs was about 2.88 years younger (p = 0.024); the duration of operation of SBMs was 2.73 h longer (p < 0.001); the duration of hospital stays of patients with SBMs was about 6.76 days longer (p < 0.001); the tumor volume was 7.69 cm(3) smaller (p = 0.025); the intraoperative blood loss was 147.61ml more (p = 0.039); the total cost of SBMs was 1.39 times more (p < 0.001); the preoperative KPS, postoperative KPS, and follow-up KPS of patients with SBMs were all respectively lower (p < 0.001); Gross total resection was less achieved (p < 0.001). SBMs (average of 20.80 per sample) had a smaller total number of copy number variations (CNVs) than NSBMs (29.98 per sample) (p = 0.009). Extremely large CNVs (> 5 Mb) were more likely to present in NSBMs (p < 0.001). Cox analysis showed that subtotal resection (p = 0.002) and the total number of CNVs (p = 0.015) were independent risk factors for tumor recurrence.ConclusionsThe clinical and cytogenetic features of SBMs were different from NSBMs. Moreover, the degree of resection and the total number of whole-genome CNVs were independent prognostic factors for tumor recurrence.