Motor seizures confer overall survival benefit in who grade 2 glioma.

OBJECTIVE: The prevalence of epilepsy in World Health Organization (WHO) grade 2 glioma is high, with seizures being the presenting symptom in 60%-90%. We explore the epidemiology of seizures in this patient population in a regional neurosurgical center. METHODS: Electronic health records of patients with histologically-proven WHO grade 2 glioma (n = 228) were reviewed between 1997 and 2021, with data collected including patient demographics, epilepsy prevalence, and seizure semiology. The influence of seizure type on overall survival was calculated using a Cox proportional hazards model. RESULTS: Overall, 197 of 228 patients (86.4%) were diagnosed with epilepsy-either at presentation or during the course of their disease. Male patients were more likely than female patients to be diagnosed with epilepsy (91.1% vs 77.1%, p = .003) and, in those with epilepsy, more likely to experience at least one focal to bilateral tonic-clonic seizure (69.4% vs 54.1%, p = .05). Patients with left-sided tumors were twice as likely to have experienced a focal to bilateral tonic-clonic seizure (p = .02, odds ratio [OR] = .47). Predominantly experiencing seizures with motor activity appeared to confer better overall survival, with a 65% decrease in the risk of death 10 years post diagnosis (hazard ratio [HR] = .35, p = .02). This is despite accounting for previously described prognostic markers including tumor histology/genetics, time from diagnosis to surgery, and the extent of tumor resection. SIGNIFICANCE: Motor seizure activity is a frequent feature in WHO grade 2 glioma and appears to confer a survival benefit regardless of histology or surgical factors. Seizures due to dominant hemisphere tumors may be more likely to propagate and cause bilateral tonic-clonic activity.

Pediatric-Like Brain Tumors in Adults.

Pediatric brain tumors are different to those found in adults in pathological type, anatomical site, molecular signature, and probable tumor drivers. Although these tumors usually occur in childhood, they also rarely present in adult patients, either as a de novo diagnosis or as a delayed recurrence of a pediatric tumor in the setting of a patient that has transitioned into adult services.Due to the rarity of pediatric-like tumors in adults, the literature on these tumor types in adults is often limited to small case series, and treatment decisions are often based on the management plans taken from pediatric studies. However, the biology of these tumors is often different from the same tumors found in children. Likewise, adult patients are often unable to tolerate the side effects of the aggressive treatments used in children-for which there is little or no evidence of efficacy in adults. In this chapter, we review the literature and summarize the clinical, pathological, molecular profile, and response to treatment for the following pediatric tumor types-medulloblastoma, ependymoma, craniopharyngioma, pilocytic astrocytoma, subependymal giant cell astrocytoma, germ cell tumors, choroid plexus tumors, midline glioma, and pleomorphic xanthoastrocytoma-with emphasis on the differences to the adult population.

Progesterone as a Neuroprotective Agent in Neonatal Hypoxic-Ischaemic Encephalopathy: A Systematic Review.

Hypoxic-ischaemic encephalopathy (HIE) in the newborn baby is a major contributor to neonatal mortality and morbidity across the world. Therapeutic hypothermia (TH) is the current standard treatment for moderate to severe HIE, but not all babies benefit. Potential neuroprotective actions of progesterone (PROG) include anti-apoptotic, anti-inflammatory, and anti-oxidative effects and reduction of energy depletion, tissue/cellular oedema, and excitotoxicity. In pre-clinical studies of neonatal HIE, PROG has neuroprotective properties but has not been the subject of systematic review. Here, our objective was to evaluate the evidence base for PROG as a potential therapeutic agent in HIE. The PICO framework was used to define the following inclusion criteria. Population: human neonates with HIE/animal models of HIE; intervention: PROG +/- other agents; comparison: V.S. control; outcome: pathological, neurobehavioural, and mechanistic outcome measures. Medline, EMBASE, and CINHAL were then searched between August to October 2018 using pre-defined medical subject heading and keywords. Study inclusion, data extraction, and risk of bias (ROB) analysis using the SYRCLE ROB tool were carried out by two authors. 14 studies were included in the review. They typically displayed a high ROB. This systematic review suggests that PROG reduced neuropathology and reduced neurobehavioural deficits post-hypoxic-ischaemic (HI) insult in 8 and 3 studies, respectively. However, there was sex dimorphism in the effects of PROG. In addition, there are limitations and biases in these studies, and there remains a need for well-designed large pre-clinical studies with greater methodological quality to further inform the efficacy, safety, dose, timing, and frequency of PROG administration. With such data, large animal studies could be planned combining PROG administration with and without TH.

Levetiracetam as a first-line antiseizure medication in WHO grade 2 glioma: Time to seizure freedom and rates of treatment failure.

OBJECTIVE: The high seizure burden seen in World Health Association (WHO) grade 2 gliomas is well documented. This study aims to identify factors that influence the probability of seizure freedom (12 months of seizure remission) and treatment failure (antiseizure medication [ASM] cessation or introduction of an alternative) in patients with WHO grade 2 glioma. METHODS: This is a retrospective observational analysis of patients from a regional UK neurosurgical center with histologically proven (n = 146) WHO grade 2 glioma and brain tumor related epilepsy. Statistical analyses using both Kaplan-Meier and Cox proportional hazards models were undertaken, with a particular focus on treatment outcomes when the commonly prescribed ASM levetiracetam (n = 101) is used as first line. RESULTS: Treatment with levetiracetam as a first-line ASM resulted in a significant increase in the probability of seizure freedom (p < .05) at 2 years compared with treatment with an alternative ASM. Individuals presenting with focal seizures without bilateral tonic-clonic progression were between 39% and 42% significantly less likely to reach seizure freedom within 10 years (p < .05) and 132% more likely to fail treatment by 5 years (p < .01) when compared to individuals who had seizures with progression to bilateral tonic-clonic activity. ASM choice did not significantly affect treatment failure rates. SIGNIFICANCE: More than two-thirds of patients with WHO grade 2 glioma related epilepsy treated with levetiracetam first line achieve seizure freedom within 2 years and it is a reasonable first-choice agent. Experiencing mainly focal seizures without progression infers a significant long-term reduction in the chance of seizure freedom. Further studies are needed to inform ASM selection.

Do animal models of brain tumors replicate human peritumoral edema? a systematic literature search.

INTRODUCTION: Brain tumors cause morbidity and mortality in part through peritumoral brain edema. The current main treatment for peritumoral brain edema are corticosteroids. Due to the increased recognition of their side-effect profile, there is growing interest in finding alternatives to steroids but there is little formal study of animal models of peritumoral brain edema. This study aims to summarize the available literature. METHODS: A systematic search was undertaken of 5 literature databases (Medline, Embase, CINAHL, PubMed and the Cochrane Library). The generic strategy was to search for various terms associated with "brain tumors", "brain edema" and "animal models". RESULTS: We identified 603 reports, of which 112 were identified as relevant for full text analysis that studied 114 peritumoral brain edema animal models. We found significant heterogeneity in the species and strain of tumor-bearing animals, tumor implantation method and edema assessment. Most models did not produce appreciable brain edema and did not test for observable manifestations thereof. CONCLUSION: No animal model currently exists that enable the investigation of novel candidates for the treatment of peritumoral brain edema. With current interest in alternative treatments for peritumoral brain edema, there is an unmet need for clinically relevant animal models.

Video article: the subtemporal extradural approach to cavernous sinus tumour.

OBJECTIVE: Here we demonstrate via operative video the subtemporal extradural approach to a tumour in the cavernous sinus. METHODS: The extradural approach is performed here in a paediatric patient (a 15-year-old child) via a right extended pterional osteoplastic craniotomy with removal of the zygomatic arch. The operative microscope is introduced, and the dura is divided at the superior orbital fissure into endosteal and meningeal layers using a diamond knife. The middle cranial fossa floor is drilled flat to increase access, and the plane is further developed towards the cavernous sinus. The tumour is seen bulging from within the cavernous sinus, and the cavernous sinus is opened in the anteromedial triangle between cranial nerves Vi and Vii. After biopsy, the tumour is debulked with an ultrasonic aspirator. Doppler is used to identify the internal carotid artery and preserve it. The bone flap is replaced, and the wound is closed in layers in standard fashion. RESULTS: The patient recovered well and was discharged on post-operative day 3. Persistent sixth nerve palsy (present pre-operatively) was present; however, otherwise, there was good recovery from surgery. Good resection of tumour is demonstrated on post-operative MR imaging. CONCLUSIONS: This approach is uncommon but important as it enables extradural access to the cavernous sinus, minimising the complications associated with an intradural approach such as cortical injury. In this video, we also demonstrate the fundamental anatomy using annotation and cadaveric images to enhance understanding required for the neurosurgeon to successfully complete this approach. The patient consented to the procedure in the standard fashion.

Feasibility and safety of a non-operative clinical strategy for radiologically diagnosed low grade anterior mesial temporal tumours in the absence of a histological diagnosis.

BACKGROUND: Although resection of mesial temporal lobe lesions can be achieved with relatively low morbidity, resective surgery is not without risk. Whilst many lesions found in the anterior mesiotemporal lobe are low-grade entities, transforming and high-grade lesions have also been demonstrated. We investigate the feasibility of utilising serial quantitative volumetric imaging, to determine if a strategy of imaging surveillance can be safely employed for the management of radiologically diagnosed anterior mesial temporal low-grade tumours without a confirmed histological diagnosis. METHODS: A retrospective case-note and radiology review design were utilised. The primary presenting symptomatology was recorded together with the efficacy of symptomatic control. Volumetric analysis of MRI images was performed using Brainlab software. Pre- and post-operative neuropsychological data were analysed. RESULTS: 35 patients were identified with a radiological diagnosis of a low-grade anterior mesial temporal lobe tumour. Of these, 29% (n = 10) underwent surgical resection. For the whole cohort, the mean tumour volume at diagnosis was 6.5cm3, with a mean volumetric expansion of 1.4% per month. A significant difference was found between the volumetric expansion rate of those that underwent surgical treatment and those that did not (4.9% per month vs 0.06% per month, p < .01). Of those cases that did not undergo surgical resection, no significant difference was seen between the initial diagnostic volume and the volume at the time of their most recent interval surveillance scan (p = .97). New onset epilepsy was significantly associated with a requirement for eventual surgical tumour resection; relative risk = 6.25, 95% CI = 1.5-25.9, p = .0114. CONCLUSION: Where medical seizure control is adequate, we suggest that conservative management is feasible even in the absence of a confirmed histological diagnosis. However, in patients aged over 50 years with new onset epilepsy, a lower threshold for intervention should be considered.

Day 2 neutrophil-to-lymphocyte and platelet-to-lymphocyte ratios for prediction of delayed cerebral ischemia in subarachnoid hemorrhage.

OBJECTIVE: Recent evidence has suggested that an admission neutrophil-to-lymphocyte ratio (NLR) of ≥ 5.9 predicts delayed cerebral ischemia (DCI) in aneurysmal subarachnoid hemorrhage (aSAH). The primary aims of this study were to assess reproducibility and to ascertain the predictive ability of NLR on subsequent days postictus. Secondary aims included identification of additional inflammatory markers. METHODS: A single-center, retrospective study of all patients aged ≥ 18 years with aSAH between May 2014 and July 2018 was performed. Patient characteristics, DCI incidence, operative features, and outcomes (on discharge and at 3 months postictus) were recorded. C-reactive protein (CRP) and full blood count differentials were recorded on admission and through day 8 postictus or at discharge. In total, 403 patients were included in the final analysis. RESULTS: Ninety-six patients (23.8%) developed DCI with a median time from ictus of 6 days (IQR 3.25-8 days). A platelet-to-lymphocyte ratio (PLR) cutoff ≥ 157 and CRP cutoff ≥ 27 was used in our cohort. In a multiple binary logistic regression model, after controlling for known DCI predictors, day 2 NLR ≥ 5.9 (OR 2.194, 95% CI 1.099-4.372; p = 0.026), day 1 PLR ≥ 157 (OR 2.398, 95% CI 1.1072-5.361; p = 0.033), day 2 PLR ≥ 157 (OR 2.676, 95% CI 1.344-5.329; p = 0.005), and CRP ≥ 27 on days 3, 4, and 5 were predictive of DCI. CONCLUSIONS: The results of this study have confirmed the association between NLR and DCI and have demonstrated the predictive potential of PLR and CRP, suggesting that NLR and PLR at day 2, and CRP from day 3 onward, may be better predictors of DCI than those measurements at the time of ictus.

ABO blood group in aneurysmal subarachnoid haemorrhage-a pilot study.

PURPOSE: To evaluate the distribution and impact of ABO blood group on the baseline characteristics and clinical outcomes of patients presenting with aneurysmal subarachnoid haemorrhage (aSAH). METHODS: Retrospective, single-centre study of patients admitted to a neurosurgical department in the UK, with a diagnosis of spontaneous subarachnoid haemorrhage between May 2014 and January 2020. Patients were categorised by ABO blood type and by Rhesus status. Clinical outcomes such as initial bleeding, rebleeding, delayed cerebral ischaemia (DIND) and venous thromboembolism were analysed in relation to the size of their association with ABO blood type. Hospital mortality rate, Glasgow Outcome Score (GOS) - at discharge and 3 months post-ictus, requirement for ventriculoperitoneal shunt insertion, discharge destination and inpatient length of stay were also considered. RESULTS: Four-hundred twelve adult patients admitted with aSAH were included in our analysis. The distribution of ABO group or Rhesus status in our cohort did not differ significantly from the general population in the UK. Blood group A patients had a significantly increased risk of developing DIND, compared with non-blood group A patients (OR, 1.88 [95% CI: 1.10-3.21]). CONCLUSIONS: ABO blood type appears to influence aSAH sequelae. Blood group A patients are at highest risk of DIND following aSAH.

'Possible shunt malfunction' pathway for paediatric hydrocephalus-a study of clinical outcomes and cost implications.

BACKGROUND: Shunt insertion for hydrocephalus is a common paediatric neurosurgery procedure. Shunt complications are frequent with an estimated 20-40% failure rate within the first year, and 4.5% per year subsequently. We have an open-door 'possible shunt malfunction' pathway for children treated with a shunt or endoscopic third ventriculostomy, providing direct ward access to ensure rapid assessment and timely management of children. OBJECTIVE: To audit the 'possible shunt malfunction' pathway in terms of clinical outcomes (percentage-confirmed shunt dysfunction and number of re-attendances) and costs. METHODS: Clinical data for patients attending the triage service were prospectively recorded over 7 months-including the number of attendances, previous shunt revisions, shunt type, investigations performed (CT, x-rays), and outcome. Costings (e.g. costs of physician, inpatient stay, investigations) were obtained from the hospital's procurement department. RESULTS: In the study period, there were 81 attendances by 62 patients and only 16% of attendances resulted in surgical management (either shunt revision or ETV). Approximately 17% of patients re-attended at least once. The average cost per attendance in our pathway was £765.57 ($969.63; €858.73). The total expenditure for the pathway over 7 months was £62,011.03 ($78,540.07; €69,556.81), with inpatient stay making up the biggest percentage of cost (49.2%). CONCLUSION: Only 16% (13 attendances) of those attending through our pathway required neurosurgical intervention. Investigations for possible blocked shunt come at significant health, social, and financial cost. High rates of shunt failure, re-attendance, investigations, and inpatient stays incur a sizable financial burden to the healthcare system.

The role of sex genotype in paediatric CNS tumour incidence and survival.

PURPOSE: Evidence exists, in CNS germinomas and medulloblastomas (MB), that patient sex significantly influences incidence and outcome. The role of sex genotype in other paediatric CNS tumours remains unclear. This study sought to examine the role of sex genotype in CNS tumour incidence and overall survival (OS). METHODS: Age-adjusted incidence and OS rates were collected from the Surveillance Epidemiology and End Result (SEER) registry between 2000 and 2011 for common paediatric (<=19 years) CNS tumours: pilocytic astrocytoma (PA), anaplastic astrocytoma, glioblastoma (GBM), medulloblastoma, supratentorial CNS embryonal tumour, ependymoma, and germinoma. All patients with histologically confirmed, ICD-03 coded, first tumours, were included. Kaplan-Meier and Cox regression analyses were used to calculate hazard ratios (HR). RESULTS: The total cases are as follows: males=3018 and females=2276. Highest incidence was seen in PA (n=2103). GBM displayed the worst OS, whilst PA displayed the best. Higher incidence was observed in males for all tumours, except PA. Females with ependymoma had significantly better OS compared to males, whereas males with germinomas had better OS compared to females. Females <1 year with AA had better OS than males. Increasing age significantly improved male and female survival in ependymoma and medulloblastoma. CONCLUSION: Interrogating population-based registries such as SEER minimises bias and provides credible data. Observed differences in incidence and OS between the sexes for different paediatric CNS tumours provide useful prognostic information for clinicians. Sex genotype was a significant independent prognostic factor in ependymomas and germinomas. Further investigation of possible epigenetic and hormonal differences may provide sex-specific vulnerabilities that may be exploitable for targeted therapy.

Intramedullary spinal cord tumours - a single Centre, 10-year review of clinical and pathological outcomes.

BACKGROUND: Intramedullary spinal cord tumours are relatively rare tumours of the central nervous system. Surgical outcomes are affected by many variables, including pre-operative neurological function, tumour histology and extent of resection. Emphasis remains on surgical treatment due to limited adjunctive therapeutic options and poor drug penetration. OBJECTIVE: To identify clinically relevant predictors of progression free survival by retrospectively analysing the extent of resection, pre- and post-operative neurological function and histology in intramedullary spinal cord tumours from a single neurosurgical centre over 10 years. METHODS: Forty-three adult cases were identified from a surgical database. Variables collected included pre-and post-operative Frankel Grade and Modified McCormick Scale assessments, tumour histology, extent of resection and length of follow up. Chi-Squared, Kaplan-Mier Survival and Mann-Whitney U-tests were completed. RESULTS: Ependymoma (41.9%) and haemangioblastoma (14.0%) were the commonest tumour histologies. In total, 17 different histological tumours were identified in the series. There was a statistically significant relationship between identification of the tumour plane and extent of resection (p < 0.01), along with the extent of resection and recurrence (p = 0.04). Compared to the other histological subtypes, ependymoma's demonstrated a significantly greater extent of resection (p = 0.01). There was a significant relationship between the grade of tumour and progression-free survival (p < 0.01). CONCLUSION: Tumour plane and the extent of tumour resection are significant determinants of progression-free survival. Ependymoma, whilst being the commonest histology in our series were also the most resectable. Whilst complete resection reduces the rate of recurrence, tumour grade is the most important predictor of outcome. Given the importance of the extent of resection, and following a similar trend to other low volume pathologies, these tumours should only be tackled by neurosurgeons with experience in their resection.

A comparison of the extent of resection in pineal region tumours via the occipital transtentorial and supracerebellar infratentorial approaches.

PURPOSE: To perform a single unit review of surgical approaches to the pineal region, looking to ascertain if trends were identifiable regarding the extent of resection and the rate of post-operative complications between approaches. We hypothesised that each approach would offer different exposure of the pineal region which may result in poor access to certain areas of the tumour. This may lead to residual tumour in reliable and predictable locations, and an awareness of these regions could help with pre-operative planning and lead to higher levels of suspicion when inspecting these regions intraoperatively. MATERIALS AND METHODS: We performed a single centre, retrospective review of all adult and paediatric patients who underwent surgical debulking of pineal region tumours between 2008 and 2019. Patient demographics, pre- and post- operative radiological tumour volume data, histology and complication rates were compared between the two groups. RESULTS AND CONCLUSIONS: The occipital transtentorial approach resulted in a significantly lower extent of resection when compared to the supracerebellar infratentorial approach (p = 0.04), even after multivariate analysis (p = 0.006). There was no significant difference between the location of residual tumour relative to the superior colliculi between the two approaches (p = 1.00). There was a significant incidence of radiological occipital lobe ischaemia from the occipital transtentorial approach (p = 0.04). Within our series, we did not demonstrate a consistent location of residual tumour relative to the surgical approach chosen. Whilst there was a significant difference with regards to the extent of resection between approaches, in the context of small comparative groups this is difficult to draw far-reaching conclusions from.

Fronto-orbital advancement and reconstruction using reverse frontal bone graft without the use of orbital bar: a technical note.

INTRODUCTION: We describe our technique of using reverse frontal bone graft for FOAR for patients with metopic or coronal synostosis and present our complications using the Leeds classification system for complications in craniosynostosis surgery. METHODS: Since April 2015, seventeen patients have been operated using this technique. We perform a frontal bone graft that is then reversed, and supraorbital margins are drilled out. The orbital bar is then removed and drilled down to make bone dust and on-lay bone grafts which are then used to fill gaps on exposed dura and fill in around the temporal region. RESULTS: All 17 patients who underwent this technique have good cosmetic results. We report 5 (29%) complications and 8 (47%) blood transfusions (7 exposures, 1 cell salvage).

Recovery of major cognitive deficits following awake surgery for insular glioma: a case report.

BACKGROUND: Resection of insular tumours utilising modern neurosurgical techniques has become commonplace since its safety and reduced morbidity was first established. Interest has grown in the cognitive consequences of insula neurosurgery and studies have largely shown postoperative stability or minor decline. Major or widespread improvements in cognitive functioning following resection of insular tumours have not previously been reported. CASE DESCRIPTION: A 34-year-old, left-handed man with a right insular low-grade glioma (LGG) presented with seizures, nausea, altered sensation, poor balance and extensive cognitive decline. Comprehensive neuropsychological assessment highlighted a striking left hemispatial neglect and impairments in attention, working memory, verbal learning and fluency. During an awake craniotomy with functional cortical mapping, he reported intraoperative improvements in hand function and processing speed. Resolution of the neglect and significant improvements in cognition, mood and functioning were observed at follow-up and sustained over several years. CONCLUSIONS: This case highlights that right insular LGGs can cause significant cognitive and functional deficits and that neurosurgery has the potential to alleviate these difficulties to an extent beyond those documented in the extant literature.

Can quantifying the extent of 'high grade' features help explain prognostic variability in anaplastic astrocytoma?

PURPOSE: Both phenotypic and genotypic variations now underpin glioma classification, thus helping to more accurately guide their clinical management. However, WHO Grade III anaplastic astrocytoma (AA) remains an unpredictable, heterogeneous entity; displaying a variable prognosis, clinical course and treatment response. This study aims to examine whether additional tumour characteristics influence either overall survival (OS) or 3-year survival in AA. MATERIALS AND METHODS: Data were collected on all newly diagnosed cases of AA between 2003 and 2014, followed up for a minimum of 3 years. Molecular information was obtained from case records and if missing, was re-analysed. Histological slides were independently examined for Ki-67 proliferation index, cellularity and number of mitotic figures. Kaplan-Meier and Cox regression analyses were used to assess OS. RESULTS: In total, 50 cases were included with a median OS of 14.5 months (range: 1-150 months). Cumulative 3-year survival was 31.5%. Median age was 50 years (range: 24 - 77). Age, IDH1 mutation status, lobar location, oncological therapy and surgical resection were significant independent prognostic indicators for OS. In cases demonstrating an OS ≥ 3 years (n = 15), Ki-67 index, number of mitotic figures and percentage areas of 'high cellularity' were significantly reduced, i.e. more characteristic of lower-grade/WHO Grade II glioma. CONCLUSIONS: IDH1 status, age, treatment and location remain the most significant prognostic indicators for patients with AA. However, Ki-67 index, mitotic figures and cellularity may help identify AA cases more likely to survive < 3 years, i.e. AA cases more similar to glioblastoma and those cases more likely to survive > 3 years, i.e. more similar to a low-grade glioma.

Incidence of childhood CNS tumours in Britain and variation in rates by definition of malignant behaviour: population-based study.

BACKGROUND: Intracranial and intraspinal tumours are the most numerous solid tumours in children. Some recently defined subtypes are relatively frequent in childhood. Many cancer registries routinely ascertain CNS tumours of all behaviours, while others only cover malignant neoplasms. Some behaviour codes have changed between revisions of the International Classification of Diseases for Oncology, including pilocytic astrocytoma, downgraded to uncertain behaviour in ICD-O-3. METHODS: We used data from the population-based National Registry of Childhood Tumours, which routinely included non-malignant CNS tumours, to document the occurrence of CNS tumours among children aged < 15 years in Great Britain during 2001-2010 and to document the descriptive epidemiology of childhood CNS tumours over the 40-year period 1971-2010, during which several new entities were accommodated in successive editions of the WHO Classification and revisions of ICD-O. Eligible cases were all those with a diagnosis included in Groups III (CNS tumours) and Xa (CNS germ-cell tumours) of the International Classification of Childhood Cancer, Third Edition. The population at risk was derived from annual mid-year estimates by sex and single year of age compiled by the Office for National Statistics and its predecessors. Incidence rates were calculated for age groups 0, 1-4, 5-9 and 10-14 years, and age-standardised rates were calculated using the weights of the world standard population. RESULTS: Age-standardised incidence in 2001-10 was 40.1 per million. Astrocytomas accounted for 41%, embryonal tumours for 17%, other gliomas for 10%, ependymomas for 7%, rarer subtypes for 20% and unspecified tumours for 5%. Incidence of tumours classified as malignant and non-malignant by ICD-O-3 increased by 30 and 137% respectively between 1971-75 and 2006-10. CONCLUSIONS: Total incidence was similar to that in other large western countries. Deficits of some, predominantly low-grade, tumours or differences in their age distribution compared with the United States and Nordic countries are compatible with delayed diagnosis. Complete registration regardless of tumour behaviour is essential for assessing burden of disease and changes over time. This is particularly important for pilocytic astrocytoma, because of its recent downgrading to non-malignant and time trends in the proportion of astrocytomas with specified subtype.

Chiari I malformation and altered cerebrospinal fluid dynamics-the highs and the lows.

PURPOSE: This paper reviews the altered cerebrospinal fluid dynamics that can be associated with paediatric Chiari I malformation and we present our own institutional experience. METHODS: We conducted a thorough review of the literature and retrospectively analyzed all cases of operatively managed paediatric Chiari 1 malformation at our institution between February 2006 and February 2019. RESULTS: Acquired Chiari malformation (ACM) can radiologically mimic Chiari I and has been associated with both intracranial hypotension (either secondary to lumboperitoneal shunting or spontaneous CSF hypotension) and idiopathic intracranial hypertension (IIH). At our institution, 61 paediatric cases (range, 2-15 years) underwent foramen magnum decompression (FMD) for Chiari I malformation. Whilst 80% (50/61) of cases underwent FMD with no preceding or post-operative problems of CSF dynamics, 8% (5/61) of cases had hydrocephalus at initial presentation requiring CSF diversion followed by FMD for persistent Chiari, and 10% (6/61) developed hydrocephalus following FMD and required long-term CSF diversion. CONCLUSIONS: In paediatric ACM, the management of intracranial hypotension involves thorough radiological assessment and inclusion/adjustment of a valve in the case of lumboperitoneal shunting or epidural blood patch or interventional techniques in the case of spontaneous CSF leak. Thereby, unwarranted posterior fossa decompression surgery is avoided. In the case of IIH and Chiari I malformation, children who have recurrent symptoms despite adequate posterior fossa decompression surgery (failed Chiari), there is a strong role for intracranial pressure monitoring as raised intracranial pressure may indicate long-term CSF diversion.

Outcome and prognostic features in paediatric pineoblastomas: analysis of cases from the Surveillance, Epidemiology, and End Results registry (1990-2007).

BACKGROUND: Paediatric pineoblastomas are rare central nervous system tumours. Patient and treatment factors associated with outcome are poorly defined and limited to small retrospective case series and single case reports. METHODS: Using the Surveillance, Epidemiology, and End Results (SEER) cancer registry, we investigated clinical and pathological factors associated with outcome in paediatric pineoblastomas. Paediatric patients (< 16 years old) with pineoblastomas diagnosed between 1990 and 2007 were identified from the SEER database. Kaplan-Meier survival analysis and Cox models were used to examine the effect of variables on overall survival. The variables analysed included patient's age at diagnosis, gender, race, tumour spread and size, surgical resection and the use of adjuvant radiotherapy. RESULTS: Seventy-eight patients were identified from the database. Twelve patients were excluded as 11 had no surgery and one patient was excluded as the surgical status was unknown. Analysis of the remaining 66 patients revealed a median age at diagnosis of 5.5 years. Three patients underwent biopsy. Seventeen patients underwent full and partial resection, respectively. A further 46 patients underwent surgery the nature of which was not recorded. Thirty-nine patients (59.1%) received adjuvant radiotherapy. Eight patients (12.1%) had both surgery (full or partial resection) and radiotherapy. The median overall survival was 40.5 months. Univariate analysis demonstrated that older age at diagnosis was the only positive predictor of overall survival. CONCLUSION: This study represents the largest analysis of paediatric pineoblastomas to date. The only clinically relevant prognostic factor was older age at diagnosis. The role of surgery and adjuvant radiotherapy on overall survival remains to be defined.

From above or from below? That is the question. Comparison of the supraorbital approach with the endonasal approach. A cadaveric study.

BACKGROUND: Nowadays the endoscopic endonasal approach is increasingly being used to remove craniopharyngiomas, tuberculum sellae meningiomas and other presellar and parasellar lesions and its value in anterior skull base surgery is undisputed. Herein, we assess the relative advantages, disadvantages and feasibility of the keyhole eyebrow approach and the endonasal endoscopic approach in four cadaveric heads for the removal of presellar and parasellar lesions. METHODS: We used four cadaveric heads for 12 surgical dissections. The specimens were embalmed with two different techniques. Two bilateral supraorbital endoscopic assisted approaches and one transnasal expanded endonasal approach were performed for each head. We evaluated the feasibility, maneuverability and safety of each approach. We measured the operating room obtained with each approach and the distance from the main structures we reached. RESULTS: The technical feasibility of the endoscopic endonasal transphenoidal approach and the supraorbital eyebrow approach was reproduced in all four cadaveric heads. The transnasal approach gave us a good operating field medial to the two optic nerves and the two carotid arteries anteriorly until the frontal sinus and, posteriorly, the basilar artery, the emergence of the superior cerebellar arteries and posterior cerebral arteries. After performing the supraorbital approach, we viewed a wider field of the anterior skull base and we were able to reach the ipsilateral carotid artery, the optico-carotid recess, the pituitary stalk, the lamina terminalis until the contra lateral optic nerve and carotid artery, keeping a wider angle of maneuverability. CONCLUSIONS: Although the endoscopic transnasal approach has developed in leaps and bounds in the last decade, other transcranial approaches maintain their value. The supraorbital endoscopic approach is a minimally invasive approach and seems to be optimal for those lesions wider than 2 cm in the lateral extension and for all the paramedian lesions.