Recurrent high grade glioma surgery with carmustine wafers implantation: a long-term nationwide retrospective study.

PURPOSE: Widespread use of carmustine wafers (CW) to treat high-grade gliomas (HGG) has been limited by uncertainties about its efficacy. To assess the outcome of patients after recurrent HGG surgery with CW implantation and, search for associated factors. METHODS: We processed the French medico-administrative national database between 2008 and 2019 to retrieve ad hoc cases. Survival methods were implemented. RESULTS: 559 patients who had CW implantation after recurrent HGG resection at 41 different institutions between 2008 and 2019 were identified. 35.6% were female and, median age at HGG resection with CW implantation was 58.1 years, IQR [50-65.4]. 520 patients (93%) had died at data collection with a median age at death of 59.7 years, IQR [51.6-67.1]. Median overall survival (OS) was 1.1 years, 95%CI[0.97-1.2], id est 13.2 months. Median age at death was 59.7 years, IQR [51.6-67.1]. OS at 1, 2 and 5 years was 52.1%, 95%CI[48.1-56.4], 24.6%, 95%CI[21.3-28.5] & 8%, 95%CI[5.9-10.7] respectively. In the adjusted regression, bevacizumab given before CW implantation, (HR = 1.98, 95%CI[1.49-2.63], p < 0.001), a longer delay between the first and the second HGG surgery (HR = 1, 95%CI[1-1], p < 0.001), RT given before and after CW implantation (HR = 0.59, 95%CI[0.39-0.87], p = 0.009) and TMZ given before and after CW implantation (HR = 0.81, 95%CI[0.66-0.98], p = 0.034) remained significantly associated with a longer survival. CONCLUSION: OS of patients with recurrent HGG that underwent surgery with CW implantation is better in case of prolonged delay between the two resections and, for the patients who had RT and TMZ before and after CW implantation.

Relative survival after meningioma surgery. A French nationwide population-based cohort study.

BACKGROUND: Survival after meningioma surgery is often reported with inadequate allowance for competing causes of death. METHODS: We processed the Système National des Données de Santé, the French administrative medical database to retrieve appropriate patients' case of surgically treated meningiomas. The Pohar Perme relative survival (RS) method was implement. RESULTS: A total of 28,778 patients were identified between 2007 and 2017 of which 75% were female. Median age at surgery 59 years. Cranial convexity was the most common (24.7%) location and, benign meningioma represented 91.5% of all meningioma. Median follow-up was 3.5 years interquartile range [3.4-3.5]. At data collection, 2,232 patients were dead. The five-year survival relative to the expected survival of an age- and gender-matched French standard population was 96.2% 95% confidence interval (CI)[95.7-96.8]. Meningioma absolute excess risk of death was 973/100,000 person-years 95%CI[887-1068] (p< .001). The related standardised mortality ratio was 1.8 95%CI[1.7-1.9] (p< .001). In the adjusted model, male gender (hazard ratio [HR] =1.39, 95%CI[1.27-1.54], p< .001), age at surgery (HR=0.97, 95%CI[0.97-0.97], p < .001), type 2 neurofibromatosis (HR=2.95, 95%CI[1.95-4.46], p < .001), comorbidities HR=1.39, 95%CI[1.36-1.42], p < .001), location (HR=0.8, 95%CI[0.67-0.95], p= .0111), pre-operative embolization, (HR=1.3, 95%CI[1.08-1.56], p= .00507), cerebro-spinal fluid shunt, (HR=2.48, 95%CI[2.04-3.01], p < .001), atypical (HR=1.3, 95%CI [1.09-1.54], p= .00307) or malignant histology (HR=1.86, 95%CI[1.56-2.22], p< .001), redo surgery (HR=1.19, 95%CI[1.04-1.36], p= .0122) and radiotherapy (HR=1.43, 95%CI[1.26-1.62], p < .001) were established as independent predictors of RS. CONCLUSION: This unique study highlights the excess mortality associated with meningioma disease. Many factors such as gender, age, location, histopathological grading, redo surgery influence the RS.

Cyproterone acetate and meningioma: a nationwide-wide population based study.

BACKGROUND: The study the characteristics of surgical meningiomas in female patients who took CPA and to compare this population to a non-CPA control group. MATERIALS AND METHODS: We processed the French Système National des Données de Santé (SNDS) database to retrieve appropriate cases operated between 2007 and 2017. RESULTS: 1 101 female patients (3.8%) who used to take CPA and underwent a meningioma surgery were extracted from a nationwide population based cohort of 28 924 patients. Median age at CPA prescription was 42 years IQR[36.7-48.9]. The median time between CPA start and surgery was 5.5 years IQR[3.1-7.9]. The median age at surgery was significantly lower in patients who were treated by CPA (47 years, IQR[42-54) compared to the non-CPA population (61 years, IQR[51-70], p < 0.001). Median CPA dose was 40 g, IQR[19-72]. There was a strong correlation between CPA dose and duration (r = 0.58, 95%CI[0.54-0.62], p < 0.001). Middle skull base was the most common (39%) location with a anterior skull base insertion being also far more common compared to the usual population with 21.9% of the tumour. This skull base predominance of CPA-associated meningioma is highly significant (p < 0.001). Increased CPA dose raised the risk of having multiple meningioma surgeries (p < 0.001) and multiple meningioma locations (p < 0.001). Tumour grading was not modified by CPA treatment (p = 0.603). Benign or grade I meningioma accounting for 92%, atypical or grade II for 6.1% and malignant or grade III for 1.9%. CONCLUSION: In the past 10 years, a significant number of CPA-induced meningiomas have been removed, modifying the global pyramid of age at surgery for female patients. These tumours occur well before the usual age and are preferentially located on the anterior and middle skull base.

Failure of Internal Cerebrospinal Fluid Shunt: A Systematic Review and Meta-Analysis of the Overall Prevalence in Adults.

OBJECTIVE: Reported rates of failures of internal cerebrospinal fluid shunt (ICSFS) vary greatly from less than 5% to more than 50% and no meta-analysis to assess the overall prevalence has been performed. We estimated the failure rate after ICSFS insertion and searched for associated factors. METHODS: Six databases were searched from January 1990 to February 2022. Only original articles reporting the rate of adult shunt failure were included. Random-effects meta-analysis with a generalized linear mixed model method and logit transformation was used to compute the overall failure prevalence. Subgroup analysis and meta-regression were implemented to search for associated factors. RESULTS: Of 1763 identified articles, 46 were selected, comprising 70,859 ICSFS implantations and 13,603 shunt failures, suggesting an accumulated incidence of 19.2%. However, the calculated pooled prevalence value and its 95% confidence interval (CI) were 22.7% (95% CI, 19.8-5.8). The CI of the different estimates did not overlap, indicating a strong heterogeneity confirmed by a high I2 of 97.5% (95% CI, 97.1-97.8; P < 0.001; τ2 = 0.3). Ninety-five percent prediction interval of shunt failure prevalence ranged from 8.75% to 47.36%. A meta-regression of prevalence of publication found a barely significant decreasing failure rate of about 2% per year (-2.11; 95% CI, -4.02 to -0.2; P = 0.031). CONCLUSIONS: Despite being a simple neurosurgical procedure, ICSFS insertion has one of the highest risk of complications, with failure prevalence involving more than 1 patient of 5. Nonetheless, all efforts to lower this high level of shunt failure seem to be effective.

Internal Ventricular Cerebrospinal Fluid Shunt for Adult Hydrocephalus: A Systematic Review and Meta-Analysis of the Infection Rate.

BACKGROUND: Hydrocephalus is a common neurological condition that usually requires internal ventricular cerebrospinal fluid shunt (IVCSFS). The reported infection rate (IR) varies greatly from below 1% up to over 50%, but no meta-analysis to assess the overall IR has ever been performed. OBJECTIVE: To determine the IVCSFS overall IR in the adult population and search for associated factors. METHODS: Six databases were searched from January 1990 to July 2022. Only original articles reporting on adult IVCSFS IR were included. Random-effects meta-analysis with generalized linear mixed model method and logit transformation was used to assess the overall IR. RESULTS: Of 1703 identified articles, 44 were selected, reporting on 57 259 patients who had IVCSFS implantation and 2546 infections. The pooled IR value and its 95% CI were 4.75%, 95% CI (3.8 to 5.92). Ninety-five percent prediction interval ranged from 1.19% to 17.1%. The patients who had IVCSFS after intracranial hemorrhage showed a higher IR (7.65%, 95% CI [5.82 to 10], P -value = .002). A meta-regression by year of publication found a decreasing IR (-0.031, 95% CI [-0.06 to 0.003], P -value = .032) over the past 32 years. CONCLUSION: IVCSF is a procedure that every neurosurgeon should be well trained to perform. However, the complication rate remains high, with an estimated overall IR of 4.75%. The IR is especially elevated for hydrocephalic patients who require IVCSFS after intracranial hemorrhage. However, decades of surgical advances may have succeeded in reducing IR over the past 32 years.

Newly Diagnosed High-Grade Glioma Surgery with Carmustine Wafers Implantation. A Long-Term Nationwide Retrospective Study.

BACKGROUND: Widespread use of carmustine wafers (CWs) to treat high-grade gliomas (HGG) has been limited by uncertainties about their efficacy. We sought to assess the outcome of patients after newly diagnosed HGG surgery with CW implantation and search for associated factors. METHODS: We processed the French medico-administrative national database between 2008 and 2019 to retrieve ad hoc cases. Survival methods were implemented. RESULTS: In total, 1608 patients who had CW implantation after HGG resection at 42 different institutions between 2008 and 2019 were identified; 36.7% were female and, median age at HGG resection with CW implantation was 61.5 years, interquartile range (IQR) [52.9-69.1]. A total of 1460 patients (90.8%) had died at data collection at a median age at death of 63.5 years, IQR [55.3-71.2]. Median overall survival (OS) was 1.42 years, 95% confidence interval [CI] 1.35-1.49, i.e., 16.8 months. Median age at death was 63.5 years, IQR [55.3-71.2]. OS at 1, 2, and, 5 years was 67.4%, 95% CI 65.1-69.7; 33.1%, 95% CI 30.9-35.5; and 10.7%, 95% CI 9.2-12.4, respectively. In the adjusted regression, sex (hazard ratio [HR] 0.82, 95% CI 0.74-0.92, P < 0.001), age at HGG surgery with CW implantation (HR 1.02, 95% CI 1.02-1.03, P < 0.001), adjuvant radiotherapy (HR 0.78, 95% CI 0.7-0.86, P < 0.001), chemotherapy by temozolomide (HR 0.7, 95% CI 0.63-0.79, P < 0.001), and redo surgery for HGG recurrence (HR 0.81, 95% CI 0.69-0.94, P = 0.005) remained significantly associated with the outcome. CONCLUSIONS: OS of patients with newly diagnosed HGG who underwent surgery with CW implantation is better in young patients, those of the female sex, and for those who complete concomitant chemoradiotherapy. Redo surgery for HGG recurrence also was associated with prolonged survival.

Magendie's foramen debridement and catheterisation for the treatment of syringomyelia due to diffuse craniocervical junction arachnoiditis. A case report and technical note.

A 36 year old woman was referred to our department for symptomatic lumbar spinal arachnoiditis following an epidural anaesthesia for childbirth. She did not had other known causative factor and she was free of any neurological symptoms before. She rapidly developed lower limbs impairment by compressing intradural lumbar collections and arachnoiditis requiring surgical decompression and subsequently internal cerebrospinal fluid shunting for acute hydrocephalus. Three years and the half later, she developed a severe tetraparesis due to a massive syrinx consecutive to the fourth ventricle outlets obstruction cause by the ongoing diffuse craniocervical junction arachnoiditis. Our aim was to treat all the problems in one step. An open fourth ventriculostomy of the Magendie's foramen with catheter insertion from the fourth ventricle down to the upper cervical subarachnoid space improve both the patient status and imagery.

Epidemiology and Survival after Spinal Meningioma Surgery: A Nationwide Population-Based Study.

STUDY DESIGN: This is a cross-sectional nationwide descriptive observational and analytic retrospective study. PURPOSE: This study aims to describe and assess survival after spinal meningioma (SM) surgery. OVERVIEW OF LITERATURE: A few studies report a reduced survival after SM surgery. METHODS: The current study processed the Système National des Données de Santé (SNDS), the French national administrative medical database, to retrieve appropriate cases. RESULTS: This study identified 2,844 patients (79.1% females) between 2008 and 2017. The median age at surgery was 66 years (interquartile range [IQR], 56-75 years). Moreover, 95.9% of SMs were removed through posterior or posterolateral approaches, and 6.9% were epidural and 0.7% needed an associated spine stabilization. Benign meningioma represented 92.9%, with 5% and 2.1% atypical and malignant, respectively. The median follow-up was 3.3 years (IQR, 3.1-3.5 years). Of the patients, 0.25% and 1.2% expired within a month and a year of surgery, respectively. At data collection, 225 patients (7.9%) expired. The 5-year overall survival (OS) probability was 90.1% (95% CI, 88.6%-91.7%). However, absolute excess risk of mortality after SM surgery was null, and the related standardized mortality ratio was 1 (95% CI, 0.9-1.2; p =0.565). In the adjusted regression, age at surgery (hazard ratio [HR], 1.06; 95% CI, 1.04-1.07; p <0.001), level of comorbidities (HR, 1.44; 95% CI, 1.34-1.54; p <0.001), neurofibromatosis type 2 (NF2; HR, 3.65; 95% CI, 1.28-10.39; p =0.0152), epidural SM (HR, 1.73; 95% CI, 1.09-2.75; p =0.0206), and malignant meningioma (HR, 2.64; 95% CI, 1.51-4.61; p <0.001) remained significantly associated to a reduced OS. CONCLUSIONS: The SNDS is of great value in assessing SM incidence, associated mortality, and its predictors. OS after meningioma surgery is favorable but may be impaired for NF2 or older patients with a high level of comorbidities, epidural tumor, and malignant histopathology. SM surgery is not associated with an increased absolute excess mortality risk despite being performed on even more senior patients compared with intracranial meningioma.

Functional Outcome after Spinal Meningioma Surgery.

STUDY DESIGN: A multicenter cross-sectional analytical retrospective study. PURPOSE: To assess functional outcome (FO) after a spinal meningioma (SM) surgery. OVERVIEW OF LITERATURE: All studies report functional improvement after SM removal. METHODS: We performed an analytical retrospective cohort study at five different institutions. All patients with a diagnosis of SM were included in this study, including those with recurrent tumors. Meningiomas of the foramen magnum were excluded. Useful histopathological characteristics were separately extracted. Surgical resection was evaluated according to the Simpson grading scale. Patient outcomes and clinical states were assessed with the help of their medical records using four different scales: the modified Ranawat score, the Nurick scale, the Prolo score, the Frankel grade, and the Eastern Cooperative Oncology Group-World Health Organization- Zubrod score. RESULTS: Between 1991 and 2018, 417 patients were identified, of which 85.8% were female. The median age at surgery was 67.2 years (interquartile range [IQR], 56.7-76.5). The lesion was located in the thoracic region in 77.9% of the patients, cervical region in 16.8%, and lumbar region in 4.1%. Surgical resection was complete in 95.5% of the cases. Only 0.96% of the patients died within the first postoperative month. Neurological status, which improved in 76.9% of the patients, was unchanged in 17.5% and even worsened in 4.4%. Functional status was assessed using the Ranawat score and Nurick scale, with scores of 1 (IQR, 0-2) (i.e., hyperreflexia and asymptomatic; mean, 1.3±1.3) and 1 (IQR, 0-2) (i.e., signs of spinal cord disease, but no difficulty in walking; mean, 1.2±1.4), respectively. Approximately 10.1% of the patients were not ambulant at the last neurosurgical follow-up visit. Older age at surgery was not significantly associated with a chair-bound status (p =0.427). CONCLUSIONS: This large series confirms the favorable FO after spinal meningioma surgery even in the case of seriously impaired preoperative status. A validated scale is needed to assess the factors predicting a worsening of the functional status and guide the management of patients.

Functional Outcome After Spinal Meningioma Surgery. A Nationwide Population-Based Study.

OBJECTIVE: To describe and analysed the functional outcome (FO) after spinal meningioma (SM) surgery. METHODS: We processed the système national des données de santé (SNDS) i.e. , the French national administrative medical database to retrieve appropriate cases. We analysed the International Classification of Diseases 10 codes to assess the FO. Logistic models were implemented to search for variables associated with a favourable FO i.e. , a patient being independent at home without disabling symptom. RESULTS: A total of 2,844 patients were identified of which 79.1% were female. Median age at surgery was 66 years, interquartile range (IQR) (56-75). Ninety-five point nine percent of the SMs were removed through a posterior ± lateral approach and 0.7% need an associated stabilisation. Benign meningioma represented 92.9% and malignant 2.1%. Median follow-up was 5.5 years, IQR (2.1-8), and at data collection 9% had died. The FO was good and increased along the follow-up: 84.3% of the patients were alive and had not associated symptoms at one year, 85.9% at 2 and 86.8% at 3 years. Nonetheless, 3 years after the surgery 9.8% of the alive patients still presented at least one disabling symptom of which 2.7% motor deficit, 3.3% bladder control problem, and 2.5% gait disturbance. One point seven percent were care-provider dependent and 2.1% chair or bedfast. In the multivariable logistic regression an older age at surgery (odds ratio [OR], 0.37; 95% confidence interval [CI], 0.29-0.47, p < 0.001), a high level of comorbidities (OR, 0.71; 95% CI, 0.66-0.75, p < 0.001), and an aggressive tumor (OR, 0.49; 95% CI, 0.33-0.73; p < 0.001) were associated with a worse FO. CONCLUSION: FO after meningioma surgery is favourable but, may be impaired for older patients with a high level of comorbidities and aggressive tumor.

Outcome After Protontherapy for Progression or Recurrence of Surgically Treated Meningioma.

BACKGROUND: To assess the outcome after meningioma surgery and protontherapy (PT). METHODS: We processed the French Système National des Données de Santé database to retrieve appropriate cases of meningiomas operated and irradiated between 2008 and 2017. Survival methods were implemented. RESULTS: One hundred ninety-three patients who received PT after meningioma surgery over a 10-year period were identified. Of the 193 patients, 75.6% were female. Median age at surgery was 50 years (interquartile range [IQR] 41-62). The median number of PT fractions was 31 (IQR 30-39) given over a median duration of 52 days (IQR 44-69). Fourteen patients (7.3%) also received photon radiotherapy and six patients (3.1%) stereotactic radiosurgery. Median follow-up was 4.4 years (IQR 3.86-4.71). Five-year progression-free survival (PFS) rate was 69% (95% confidence interval [CI] 62.1-76.6). For benign, atypical, and malignant meningioma, 5-year PFS rates were 71.5% (95% CI 64.4-79.4), 55.6% (95% CI 32.5-95), and 35.6% (95% CI 12.8-98.9), respectively (p<0.01). In the adjusted regression, tumour location (hazard ratio [HR]=0.1, 95% CI 0.05-0.22, p<0.001), aggressive meningioma (HR=2.26, 95% CI 1.1-4.66, p=0.027), and the need of cerebrospinal fluid (CSF) insertion for hydrocephalus (HR=3.51, 95% CI 1.32-9.31, p=0.012) remained significantly associated to the PFS. All grades considered, 5-year overall survival (OS) rates was 89.7% (95% CI 84.6-95.1). For benign, atypical, and malignant meningioma, 5-year OS rates were 93% (95% CI 88.7-97.4), 76.4% (95% CI 51.4-100), and 44.4% (95% CI 16.7-100), respectively (p<0.01). In the multivariable regression, an older age above 70 years (HR=5.95, 95% CI 2.09-16.89, p<0.001) associated to a high level of comorbidities (HR=5.31, 95% CI 1.43-19.78, p=0.013) and a malignant meningioma (HR=5.68, 95% CI 1.54-20.94, p=0.009) remained significantly associated to a reduced OS. CONCLUSION: Five-year PFS and OS after meningioma surgery and PT is favourable but impaired for older patients with high level of morbidities, tumour of the convexity, malignant histopathology and for those requiring CSF shunting. Further inclusion and prolonged follow-up is required to assess other predictors such as sex, tumour volume, or given dose.

Tamoxifen. A treatment for meningioma?

BACKGROUND: No large-scale study evaluating the usefulness of tamoxifen after meningioma surgery has been undertaken. METHODS: We processed the French Système National des Données de Santé (SNDS) database using an algorithm combining the type of surgical procedure and the International Classification of Diseases to retrieve cases of meningiomas operated between 2007 and 2017. Survival analyses were performed using a matched cohort study. RESULTS: 251 patients treated by tamoxifen were extracted from a nationwide population-based cohort of 28 924 patients operated on for a meningioma over a 10-year period. 94% were female and median age at meningioma first surgery was 57 years IQR[47-67]. Tamoxifen treatment median duration was 1.4 years IQR[0.4-3.2]. Tamoxifen treatment median cumulative given dose was 11.4 gs, IQR[3.6-24.9]. There was a strong positive correlation between treatment duration and cumulative dose (τ=0.81, p<0.001). 6% of the patient had to be reoperated for a meningioma recurrence and 26.3% had radiotherapy. OS rates at 5 and 10 years were: 92.3%, 95%CI[90.3-94.3] and 81.3%, 95%CI[75.2-88] respectively. These 251 patients were matched by gender, age at surgery and grade with the same number of subjects within the nationwide cohort. Nor overall (HR=1.46, 95%CI[0.86- 2.49], p=0.163) or progression-free survival (HR=1.2, 95%CI[0.89- 1.62], p=0.239) were significantly improved by the tamoxifen treatment. CONCLUSION: Using this unique database, in the setting of breast cancer, we could not conclude on a favourable effect of tamoxifen to prevent recurrence after meningioma surgery or to increase meningioma-related survival even in case of prolonged treatment duration or high cumulative given dose.

Cause-Specific Survival After Meningioma Surgery: A Nationwide Population-Based Competing Risk Study.

BACKGROUND: Survival after meningioma surgery often is reported with inadequate allowance for competing causes of death. METHODS: We processed the French Système National des Données de Santé database using an algorithm combining the type of surgical procedure and the International Classification of Diseases to retrieve appropriate cases of meningiomas. The cumulative incidence of meningioma-related death was the primary end point. A competing risk analysis was performed to identify factors associated with meningioma-specific death of patients who underwent meningioma surgery. RESULTS: The risk of meningioma-related death at 1, 2, and 3 years respectively was 2.4%, 95% confidence interval [CI] 2-2.7; 3%, 95% CI 2.6-3.4; and 3.1%, 95% CI 2.7-3.6. In the adjusted Fine-Gray competing risk regression for meningioma cause-specific survival, age at surgery (subdistribution hazard ratio [SHR] 1.07, 95% CI 1.05-1.09, P < 0.001), mortality-related morbidity index (SHR 1.68, 95% CI 1.07-2.63, P = 0.025), expenditure-related morbidity index (SHR 1.06, 95% CI 1.03-1.09, P < 0.001), spinal location (SHR 0.2, 95% CI 0.08-0.47, P < 0.001), cerebrospinal fluid shunt (SHR 3.13, 95% CI 1.9-5.16, P < 0.001), grade (SHR 1.88, 95% CI 1.13-3.14, P = 0.015) redo surgery for recurrence (SHR 1.6, 95% CI 1.01-2.51, P = 0.043), and progressing meningioma (SHR 2.87, 95% CI 1.23-6.68, P = 0.015) were established as independent prognostic factors of meningioma-related death. CONCLUSIONS: Cause-specific survival after meningioma surgery is greater in younger, low-comorbidity adults with spinal and benign meningioma. Those with an intracranial, progressing malignant tumor requiring cerebrospinal fluid shunting and having a severe global health-state have a significant increased risk of meningioma-related death. Redo surgery failed to improve the outcome. We recommend the use of competing risk model in meningioma studies in which unrelated mortality may be substantial, as this approach results in more accurate estimates of disease risk and associated predictors.

A nationwide population-based study on overall survival after meningioma surgery.

BACKGROUND: There are very few nationwide studies on meningioma outcome, the most common primary intracranial tumour. METHODS: We processed the French Système National des Données de Santé (SNDS) database using an algorithm combining the type of surgical procedure and the International Classification of Diseases to retrieve all cases of meningiomas operated between 2007 and 2017. A survival analysis was performed. RESULTS: This nationwide study found 28 773 patients of which 75 % were female. Median age at surgery was 59 years, IQR[49-68]. Cranial convexity (24.4 %) and middle skull base (21.7 %) were the most common locations. 91.3 % of the tumours were benign and 2.6 % malignant.7.5 % of the patients underwent redo surgery, 9.1 % radiotherapy (RT) and 3.2 % stereotactic radiosurgery for recurrence. Median follow-up was 5.3 years 95 % CI [5.24-5.35]. 0.64 % of the patients died within a month of surgery and 2.1 % within a year. Overall survival (OS) rates at 5 and 10 years respectively were: 92.6 %, 95 %CI[92.3, 93] and 85 %, 95 %CI[84.3, 85.8]. In the multivariable analysis, female gender (HR = 0.64, 95 %CI[0.59, 0.69], p < 0.001), older age at surgery (HR= 1.07, 95 %CI[1.06, 1.07], p < 0.001), type 2 neurofibromatosis (HR= 3.89, 95 %CI[2.62, 5.76], p < 0.001), parasagittal (HR= 1.2, 95 %CI[1.05, 1.37], p = 0.00944) or falx cerebri location (HR= 1.18, 95 %CI[1.01, 1.37], p = 0.0343), atypical or (HR= 1.34, 95 %CI[1.15, 1.56], p < 0.001) malignant histology (HR= 2.34, 95 %CI[2.01, 2.73], p < 0.001), redo surgery (HR=1.81, 95 %CI[1.6, 2.04], p < 0.001), progressing meningioma (HR=1.34, 95 %CI[1.05, 1.71], p = 0.0175) or RT for recurrence (HR=2.17, 95 %CI[1.95, 2.4], p < 0.001) were established as independent prognostic factors of the OS. CONCLUSION: In this registry-based study, OS after meningioma surgery is good and is even better in women, younger adults and those with convexity and benign tumour. We also found that NF2 patients and those required redo surgery or additional treatment for uncontrolled meningioma disease are further at risk of death.

Neurofibromatosis type 2: A nationwide population-based study focused on survival after meningioma surgery.

BACKGROUND: There is no dedicated study on outcome after meningioma surgery in neurofibromatosis type 2 (NF2) patients. METHODS: We processed the French Système National des Données de Santé (SNDS) database using an algorithm combining the type of surgical procedure and the International Classification of Diseases to retrieve cases of meningioma operated in NF2 patients between 2007 and 2017. Descriptive and survival analyses were performed. RESULTS: This nationwide study found 184 patients who were operated on for 315 meningiomas over a 10-year period. 57.6 % were female, median age at first surgery was 40 years IQR[24.8-50.2] and 10.9 % were under 18 years. Cranial convexity (23.4 %) and posterior skull base (16.8 %) were the most common locations. 89.7 % of the tumours were benign and 3.3 % malignant. 16.3 % of the patient received radiotherapy and 13.6 % stereotactic radiosurgery. Median follow-up was 6.3 years, IQR[5.3-7]. At data collection, 28 patients were dead (15.2 %) and median age at death was 41.7 years, IQR [32.7-50.4]. 5 patients died within the year of meningioma surgery. OS rates at 5 and 10 years were: 87.8 %, 95 %CI[82.6-93.3] and 73.2 %, 95 %CI[63.7-84.1] respectively. In univariable Cox regression analysis, Mortality-Related Morbidity Index (MRMI) (HR = 1.57, 95 %CI[1.3-1.9], p < 0.001) Expenditure-Related Morbidity Index (HR1.16, 95 %CI[1.09-1.24], p < 0.001), a malignant meningioma (HR=8.15, 95 %CI[2.78-23.85], p < 0.001), and a diagnosis of deafness or vestibular schwannoma (HR=2.52, 95 %CI[1.02-6.23], p = 0.0447), were associated to the outcome. In multivariable analysis, solely the MRMI and a malignant meningioma remained significant predictors of reduce OS. CONCLUSION: Using this unique database, we found that outcome of NF2 patients after meningioma surgery is impaired, especially for those with significant co-morbidities and affected by a malignant meningioma.