Search

Background Neurocognitive impairments are common among brain tumor patients, and may impact patients’ awareness of performance in instrumental activities in daily life (IADL). We examined differences between patient- and proxy-reported assessments of the patient’s IADL, and whether the level of (dis)agreement is associated with neurocognitive impairments. Methods Brain tumor patients and their proxies completed the phase 3 version of the EORTC IADL-BN32 questionnaire measuring IADL, and patients completed six neurocognitive measures. Patient-proxy difference scores in IADL were compared between patients who were defined as neurocognitively impaired (≥2 neurocognitive measures ≥2.0 standard deviations below healthy controls) and non-neurocognitively impaired. With multinomial logistic regression analyses we examined if neurocognitive variables were independently associated with patient-proxy disagreement in IADL ratings. Results Patients (n = 81) did not systematically (P < .01) rate IADL outcomes different than their proxies. Proxies did report more problems on 19/32 individual items and all five scales. This effect was more apparent in dyads with a neurocognitively impaired patient (n = 37), compared to dyads with non-neurocognitively impaired patients (n = 44). Multinomial logistic regression analyses showed that several neurocognitive variables (e.g., cognitive flexibility and verbal fluency) were independently associated with disagreement between patients and proxies on different scales. Conclusion Neurocognitive deficits seem to play a role in the discrepancies between brain tumor patients and their proxies assessment of patient’s level of IADL. Although replication of our results is needed, our findings suggests that caution is warranted in interpreting self-reported IADL by patients with neurocognitive impairment, and that such self-reports should be supplemented with proxy ratings.

To provide an exhaustive review of the neuropsychological examination as conducted in brain tumor clinical trials over the last 30 years and to provide objective ratings about the reliability and suitability of such tests in neurooncological research and clinical practice. Methodologies and tools provided by the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) were exploited in order to assess the measurement properties of questionnaires and performance-based instruments used to evaluate cognitive functioning in brain tumor clinical trials from 1997 to 2017. Twenty-six brain tumor clinical trials were analyzed and an overall set of 10 neuropsychological tests was identified. A list of 24 studies concerning the reliability of such tests was analyzed. Reliability and level of evidence scores for each study and for each test were obtained. The results revealed relevant faults about the quality of measurements and the suitability of the neurocognitive assessment batteries most commonly used in brain tumor clinical trials. Our findings suggest that the cognitive assessment in brain tumor clinical trials should be implemented according to specific endpoints and should be addressed to investigate all the cognitive domains known to be affected by brain tumor and treatment.

BACKGROUND Neurocognitive deficits are common among brain tumour patients, and may impact on patient awareness of deficits in instrumental activities in daily life (IADL). This study aimed to examine differences between patient-reported and proxy-reported assessments of the patient’s performance of IADL, and whether the level of (dis)agreement is associated with neurocognitive deficits. MATERIAL AND METHODS A phase III EORTC questionnaire measuring IADL in brain tumour patients (EORTC IADL-BN32) and six neurocognitive test measures were administered as part of a larger multicentre international study designed to develop a brain tumour specific IADL questionnaire. Bland-Altman plots and Mann-Whitney U tests were used to evaluated patient- and proxy-reported IADL on a group level. Subsequently, Mann-Whitney U tests were performed to compare patient-proxy difference scores (patient IADL score - their proxy IADL score) between patients who were considered clearly neurocognitively impaired (≥2 neurocognitive test measures; ≤2.0 SD below healthy controls) and patients who were not. Furthermore, multinomial logistic regression analyses were performed to examined which sociodemgraphic, clinical, and particularly neurocognitive variables were independently associated with patients and proxies differing in their evaluation of patient’s IADL. RESULTS Patients (N=81) and proxies (N=81), on group level, did not significantly differ on either the IADL individual item or scale scores. However, significant differences were found on patient-proxy difference scores between patients who were (N=37) and were not (N=44) considered clearly neurocognitively impaired for 10/32 individual items and one of the scales (i.e. Scale 4: Administrative tasks), all showing that the proxies of clearly neurocognitively impaired patients reported more problems relative to the patients themselves, compared to proxies of patients not clearly neurocognitively impaired. Furthermore, for each scale, a neurocognitive variable, either impaired information processing speed, cognitive flexibility, verbal fluency or the number of neurocognitive test measures impaired, was found to be independently associated with proxies reporting more problems. For 4/5 scales, a clinical variable was additionally independently associated with proxies reporting more problems. Only one variable was independently associated with patient reporting more problems, namely being in active treatment was found to be associated with patients reporting more problems on Scale 4: Administrative tasks. CONCLUSION Results imply a consistent trend of clearly neurocognitively impaired patients underreporting problems with IADL compared to their proxies. It would therefore be advised to administer both the patient- and proxy-version of the EORTC IADL-BN32, particularly if neurocognitive deficits are presumed.

Objective: Prognostic value of health-related quality of life (HRQoL) data may be important to inform patients in clinical practice and to guide clinical decision-making. Our study investigated the added prognostic value of HRQoL for overall survival (OS) and progression-free survival (PFS) in a large heterogeneous sample of glioma patients, besides known prognostic factors.Methods: We included individual baseline data from previously published randomised controlled trials (RCTs) in glioma patients in which HRQoL was assessed through the European Organisation for Research and Treatment of Cancer QLQ-C30 and QLQ-BN20 questionnaires. Multivariable Cox regression models (stratified for newly diagnosed versus recurrent disease) were constructed, first with clinical variables (age, sex, tumour type, performance status, allocated treatment and extent of resection) only and subsequently with HRQoL variables added, separately for OS and PFS. The added prognostic value of HRQoL was calculated using C-indices.Results: Baseline HRQoL and clinical data from 15 RCTs were included, comprising 5217 patients. In the model including both clinical and HRQoL variables, better cognitive and role functioning and less motor dysfunction were independently associated with longer OS, whereas better role and cognitive functioning, less nausea and vomiting and more appetite loss were independently associated with prolonged PFS. However, C-indices indicated only a small prognostic improvement of the models for OS and PFS when adding HRQoL to the clinical prognostic variables (+1.1% for OS and +.7% for PFS).Conclusion: Our findings demonstrate that several baseline HRQoL variables are independently prognostic for OS and PFS, yet the added value of HRQoL to the known clinical prognostic variables was small. (C) 2019 Elsevier Ltd. All rights reserved.

Purpose Being able to function independently in society is an important aspect of quality of life. This ability goes beyond self-care, requires higher order cognitive functioning, and is typically measured with instrumental activities of daily living (IADL) questionnaires. Cognitive deficits are frequently observed in brain tumour patients, however, IADL is almost never assessed because no valid and reliable IADL measure is available for this patient group. Therefore, this measure is currently being developed. Methods This international multicentre study followed European Organisation for Research and Treatment of Cancer (EORTC) Quality of Life Group module development guidelines. Three out of four phases are completed: phases (I) generation of items, (II) construction of the item list, and (III) pre-testing. This paper reports the item selection procedures and preliminary psychometric properties of the questionnaire. Brain tumour patients (gliomas and brain metastases), their informal caregivers, and health care professionals (HCPs) were included. Results Phase I (n = 44 patient-proxy dyads and 26 HCPs) generated 59 relevant and important activities. In phase II, the activities were converted into items. In phase III (n = 85 dyads), the 59 items were pre-tested. Item selection procedures resulted in 32 items. Exploratory factor analysis revealed a preliminary dimensional structure consisting of five scales with acceptable to excellent internal consistency (α = 0.73–0.94) and two single items. For three scales, patients with cognitive impairments had significantly more IADL problems than patients without impairments. Conclusion A phase IV validation study is needed to confirm the psychometric properties of the EORTC IADL-BN32 questionnaire in a larger international sample.

Multilingualism has become a worldwide phenomenon that poses critical issues about the language assessment in patients undergoing awake neurosurgery in eloquent brain areas. The accuracy and sensitivity of multilingual perioperative language assessment procedures is crucial for a number of reasons: they should be appropriate to detect deficits in each of the languages spoken by the patient; they should be suitable to identify language-specific cortical regions; they should ensure that each of the languages of a multilingual patient is tested at an adequate and comparable level of difficulty. In clinical practice, a patient-tailored approach is generally preferred. This is a necessary compromise since it is impossible to predict all the possible language combinations spoken by individuals and thus the availability of standardized testing batteries is a potentially unattainable goal. On the other hand, this leads to high inconsistency in how different neurosurgical teams manage the linguistic features that determine similarity or distance between the languages spoken by the patient and that may constrain the neuroanatomical substrate of each language. The manuscript reviews the perioperative language assessment methodologies adopted in awake surgery studies on multilingual patients with brain tumor published from 1991 to 2021 and addresses the following issues: (1) The language selected for the general neuropsychological assessment of the patient. (2) The procedures adopted to assess the dimensions that may constrain language organization in multilingual speakers: age and type of acquisition, exposure, proficiency, and use of the different languages. (3) The type of preoperative language assessment used for all the languages spoken by the patient. (4) The linguistic tasks selected in the intraoperative setting. The reviewed data show a great heterogeneity in the perioperative clinical workup with multilingual patients. The only exception is the task used during language mapping, as the picture naming task is highly preferred. The review highlights that an objective and accurate description of both the linguistic profile of multilingual patients and the specific properties of the languages under scrutiny can profitably support clinical management and decision making in multilingual awake neurosurgery settings.

BACKGROUND Maintenance of functioning and wellbeing during the progression-free survival (PFS) period is important for glioma patients. This study aimed to determine whether health-related quality of life (HRQoL) can be maintained during progression-free time, and factors associated with HRQoL deterioration in this period. MATERIAL AND METHODS We included longitudinal HRQoL data from previously published clinical trials in glioma. The percentage of patients with stable HRQoL until progression was determined per scale and at the individual patient level (i.e. considering all scales simultaneously). We assessed time to a clinically relevant deterioration in HRQoL, expressed in deterioration-free survival and time-to-deterioration (the first including progression as an event). We also determined the association between sociodemographic and clinical factors and HRQoL deterioration in the progression-free period. RESULTS 5539 patients with at least baseline HRQoL scores had a median time from randomization to progression of 7.6 months. Between 9%-29% of the patients deteriorated before disease progression on the evaluated HRQoL scales. When considering all scales simultaneously, 47% of patients deteriorated on ≥1 scale. Median deterioration-free survival period ranged between 3.8–5.4 months, and median time-to-deterioration between 8.2–11.9 months. For most scales, only poor performance status was independently associated with clinically relevant HRQoL deterioration in the progression-free period. CONCLUSION HRQoL was maintained in only 53% of patients in their progression-free period, and treatment was not independently associated with this deterioration in HRQoL. Routine monitoring of the patients’ functioning and well-being during the entire disease course is therefore important, so that interventions can be initiated when problems are signalled.

Background Two methods combining survival and health-related quality of life (HRQoL) data in glioma trials to calculate the “net clinical benefit” were evaluated: Quality-adjusted effect sizes (QASES) and joint modeling (JM). Methods The net clinical benefit in two trials was calculated as proof of concept for other trials. With the QASES method, effect sizes for differences in progression-free survival (PFS) or overall survival (OS) and HRQoL between the experimental arm and standard treatment arm were calculated, while the relative emphasis placed on survival/HRQoL varied. JM allows simultaneous modeling of HRQoL and OS/PFS. Results In the EORTC 26951 trial, combined radiochemotherapy significantly prolonged OS (difference 11.7 months), but also resulted in more patients experiencing clinically relevant worsening (≥10 points) in appetite loss and nausea/vomiting shortly after treatment. Using QASES, the survival benefit of additional procarbazine, lomustine, and vincristine (PCV) decreased from 42.3 months to 29.5 and 28.2 months when accounting for appetite loss and nausea/vomiting, respectively. JM analyses resulted in a loss of the beneficial effect of additional PCV between 13% and 24% when adjusting for different HRQoL parameters. The EORTC 22033 trial showed no significant PFS difference between radiotherapy or temozolomide alone (46 vs 39 months), nor clinically relevant differences in HRQoL. JM analyses also showed no significant association between PFS and HRQoL scales/items, whereas QASES showed that temozolomide alone was more favorable when considering symptom burden (47–49 instead of 39 months). Conclusions Both methods resulted in different outcomes, but adjusting for the impact of treatment on HRQoL resulted in theoretically reduced survival benefits.

BACKGROUND: Health-related quality of life (HRQoL) is an important outcome in glioma research, reflecting the impact of disease and treatment on a patient’s functioning and wellbeing. Data on changes in HRQoL scores provide important information for clinical decision-making, but different analytical methods may lead to different interpretations of the impact of treatment on HRQoL. This study aimed to study whether different methods to evaluate change in HRQoL result in different interpretations. Methods: HRQoL and sociodemographical/clinical data from 15 randomized clinical trials were combined. Change in HRQoL scores was analyzed: (1)at the group level, comparing mean changes in scale/item scores between treatment arms over time, (2)at the patient level per scale/item by calculating the percentage of patients that deteriorated, improved or remained stable on a scale/item per scale/item, and (3)at the individual patient level combining all scales/items. Results: Data were available for 3727 patients. At the group scale/item level (method 1), only the item ‘hair loss’ showed a significant and clinically relevant change (i.e. ≥10 points) over time, whereas change scores on the other scales/items showed a statistically significant change only (all p< .001, range in change score:0.1–6.2). Analyses on the patient level per scale (method 2) indicated that, while a large proportion of patients had stable HRQoL over time (range:27–84%), many patients deteriorated (range:6–43%) or improved (range:8–32%) on a specific scale/item. At the individual patient level (method 3), the majority of patients (86%) showed both deterioration and improvement, while only 1% of the patients remained stable on all scales. Conclusion: Different analytical methods of changes in HRQoL result in distinct interpretations of treatment effects, all of which may be relevant for clinical decision-making. Additional information about the joint impact of treatment on all outcomes may help patients and physicians to make the best treatment decision.

INTRODUCTION The impact of treatment on both the quality and the quantity of life, i.e. the ‘net clinical benefit’, should be considered to facilitate shared decision making. Two methods that combine survival and health-related quality of life (HRQoL) data: Quality Adjusted Effect Sizes (QASES) and Joint Modelling (JM) were applied to gain insight in the net clinical benefit. METHODS The net clinical benefit in one RCT (EORTC 26951 comparing radiotherapy (RT) + PCV chemotherapy versus RT alone) was calculated as a proof of concept for other trials. With the QASES method, effect sizes for differences in survival and HRQoL between treatment arms were calculated. JM allows simultaneous modeling of a longitudinal outcome (HRQoL), and a time-to event outcome (survival). HRQoL scales/items that were selected for primary analysis in the main study were also selected for this analysis: fatigue, global health, social functioning, communication deficit, seizures, physical functioning, and nausea/vomiting. RESULTS 288/386 patients completed baseline HRQoL forms and were included in the analysis. Overall survival (OS) was significantly longer with combined treatment (42.3 vs. 30.6 months). The percentage of patients who experienced a clinically relevant deterioration (≥10 points) in nausea/vomiting, fatigue, social functioning and global health up to one year after treatment compared to baseline was larger in the RT+PCV arm. QASES corresponded to a reduction in the median OS difference from 9.7 months up till 5.5 months, given equal weights to OS and HRQoL. JM analyses resulted in a theoretical loss of treatment effect in OS of 2–6% when adjusting for HRQoL. CONCLUSION Both methods showed that adjusting for the impact of treatment on a relevant HRQoL parameter reduced the survival benefit in the experimental treatment arm compared to standard treatment arm. Applying these methods may facilitate communicating the impact of treatment to patients in clinical practice.

BACKGROUND: The relationship between spontaneous speech and formal language testing in people with brain tumors (gliomas) has been rarely studied. In clinical practice, formal testing is typically used, while spontaneous speech is less often evaluated quantitatively. However, spontaneous speech is quicker to sample and may be less prone to test/retest effects, making it a potential candidate for assessing language impairments when there is restricted time or when the patient is unable to undertake prolonged testing.AIM: To assess whether quantitative spontaneous speech analysis and formal testing detect comparable language impairments in people with gliomas. Specifically, we addressed (a) whether both measures detected comparable language impairments in our patient sample; and (b) which language levels, assessment times, and spontaneous speech variables were more often impaired in this subject group.METHOD: Five people with left perisylvian gliomas performed a spontaneous speech task and a formal language assessment. Tests were administered before surgery, within a week after surgery, and seven months after surgery. Performance on spontaneous speech was compared with that of 15 healthy speakers.RESULTS: Language impairments were detected more often with both measures than with either measure independently. Lexical-semantic impairments were more common than phonological and grammatical impairments, and performance was equally impaired across assessment time points. Incomplete sentences and phonological paraphasias were the most common error types.CONCLUSIONS: In our sample both spontaneous speech analysis and formal testing detected comparable language impairments. Currently, we suggest that formal testing remains overall the better option, except for cases in which there are restrictions on testing time or the patient is too tired to undergo formal testing. In these cases, spontaneous speech may provide a viable alternative, particularly if automated analysis of spontaneous speech becomes more readily available in the future. These results await replication in a bigger sample and/or other populations.

Background Symptom management in glioma patients remains challenging, as patients suffer from various concurrently occurring symptoms. This study aimed to identify symptom clusters and examine the association between these symptom clusters and patients’ functioning. Methods Data of the CODAGLIO project was used, including individual patient data from previously published international randomized controlled trials (RCTs) in glioma patients. Symptom prevalence and level of functioning were assessed with European Organisation for Research and Treatment of Cancer (EORTC) quality of life QLQ-C30 and QLQ-BN20 self-report questionnaires. Associations between symptoms were examined with Spearman correlation coefficients and partial correlation networks. Hierarchical cluster analyses were performed to identify symptom clusters. Multivariable regression analyses were performed to determine independent associations between the symptom clusters and functioning, adjusted for possible confounders. Results Included in the analysis were 4307 newly diagnosed glioma patients from 11 RCTs who completed the EORTC questionnaires before randomization. Many patients (44%) suffered from 5–10 symptoms simultaneously. Four symptom clusters were identified: a motor cluster, a fatigue cluster, a pain cluster, and a gastrointestinal/seizures/bladder control cluster. Having symptoms in the motor cluster was associated with decreased (≥10 points difference) physical, role, and social functioning (betas ranged from −11.3 to −15.9, all P < 0.001), independent of other factors. Similarly, having symptoms in the fatigue cluster was found to negatively influence role functioning (beta of −12.3, P < 0.001), independent of other factors. Conclusions Two symptom clusters, the fatigue and motor cluster, were frequently affected in glioma patients and were found to independently have a negative association with certain aspects of patients’ functioning as measured with a self-report questionnaire.

Objective Owing to their rarity and proteiform pathologic features, the clinical behavior of atypical meningiomas is not yet well characterized. Though the extent of resection is believed to be a key determinant of prognosis, limited data exist regarding optimal management of patients with recurrent disease. Methods In this 20-year retrospective case series, we reviewed the medical records of 46 patients with recurrent atypical meningomas (185 lesions, 89 of which were local, 78 marginal, and 18 distant recurrences); treatment was radiosurgery ( n = 60), surgery ( n = 56), or both ( n = 8). The median follow-up period was 53 months. Outcome measures were length of overall survival and disease-free intervals and prognostic factors for survival. Results Overall, the median progression-free survival was 26 months at the first recurrence and 100 months thereafter (the sum of the later intervals). Multivariate analysis showed that no treatment-related factors influenced prognosis, whereas recurrence at the skull base was a significant tumor-related factor limiting further treatment. Irrespective of treatment type, the recurrence-free interval was increasingly shorter during the clinical course, with a higher occurrence of marginal and distant lesions migrating to the midline and to the skull base. In sporadic cases, disease-free intervals were longer after wide craniotomy, tumor and dural resection with tumor-free margin. Conclusions The disease-free interval was substantially similar after surgery and radiosurgery for treating recurrent disease in patients with atypical meningiomas. Surgery is the mainstay for prolonging survival, while radiosurgery can be an adjuvant strategy to gain time for clinical observation and planning aggressive surgical treatment.

Background Falcotentorial meningiomas (FTM) stand out for their rarity, inconsistent definition, and surgical complexity. It is appropriate to deal with them in the context of medial tentorial meningiomas (TMs). Methods Clinical and radiologic characteristics of medial TMs, comprising the typical features of FTM and TM, along with surgical management and short-term and long-term outcomes, are reported. Results FTM (n = 16) were typically supratentorial, large, edematous tumors that caused mainly headache and hemianopia; TM (n = 12) were infratentorial, smaller not edematous tumors that caused mainly headache and gait ataxia. The most frequent venous pattern was straight sinus infiltration in one third of cases of FTM and occlusion in one half of cases of TM. Total removal (Simpson grade I–II) was obtained in 46.4% of cases and subtotal removal (Simpson grade III–IV) in 53.6%. Suprainfratentorial extension in FTM and incomplete venous invasion in TM were the factors most likely opposing complete removal. The overall acute complications rate was 32.1% (higher for FTM), transient for most cases. Patients with supratentorial meningiomas performed significantly worse preoperatively (Karnofsky Performance Status ≤70 in 75% of cases); patients with infratentorial symptoms/signs recovered worse postoperatively. Stereotactic radiosurgery with subtotal removal was used as adjuvant treatment in 8 cases. Only 2 recurrences, both atypical tumors, occurred at 57.6 months (mean) follow-up. Conclusions As a general rule, careful venous management, tailored surgical approach for FTM, and cautious tumor removal for TM can yield good and stable results. Total removal accounts for half the cases in both groups, whereas FTM was associated with worse postoperative complications.

Meningioma arising in the inner third of the sphenoidal wing has been well recognized since the origin of neurosurgery, yet it still poses a formidable challenge for the surgeon. Treatment strategies can be optimized through a tailored approach to surgical timing and use of a non-surgical armamentarium. The aim of this study was to evaluate the long-term effect of different strategies on progression-free survival and overall survival. We examined the clinical records of brain tumor patients to assess determinants for surgery (extent of tumor removal, postoperative complications) and for progression-free survival and overall survival in relation to timing of surgery eventually followed by stereotactic radiosurgery (SRS). The records of 60 patients were retrospectively reviewed, from preoperative assessment to a median follow-up of 104 months. All were symptomatic with prevalently visual symptoms (42.2%), large tumors (median diameter 3.44 cm), extension into the cavernous sinus (38.3%), and severe vascular involvement of one or more encased or narrowed vessels (50%). Subtotal removal was achieved in 40% of cases, mainly determined by cavernous sinus and vascular involvement; neurological complications occurred in 18.3% (persistent in 6.7% due to oculomotor and vascular injury). The overall rate of symptom improvement was 32.3% at 3 months and 49.5% at 12 months. Radiological monitoring prevented clinical progression; tumor progression occurred in 11.7% of cases. There were significant differences in progression-free survival between patients with (median 46 months) and those without (median 104 months) recurrence (p = 0.002): 12.5% after total removal, 6.2% after subtotal removal and adjuvant SRS, and 28.5% after subtotal removal and observation. The related Kaplan-Meier survival curve showed no significant difference between the three strategies. Further, disease progression after recurrence was noted in 28.6% of cases, but overall survival was not influenced by either tumor recurrence or type of treatment. Treatment failure was recorded in four cases (6.7%): one perioperative death and three later on. Surgery is the mainstay for the treatment of symptomatic meningioma and to restore neurological function; however, resectability is limited by vascular and cavernous sinus involvement. Careful postoperative monitoring prevented clinical progression and adjuvant or adjunctive SRS proved effective in tumor control. A low surgical complication rate and excellent long-term outcomes were achieved with this strategy.

Background Tentorial meningiomas are a broad and consistent category of tumors but their definition is still unclear and their classification uncertain. Objective To report the clinical and surgical characteristics of tentorial hiatus meningiomas based on a revised classification of tentorial meningiomas. Methods We reviewed the records of 14 patients who had undergone microsurgical removal of incisural tentorial meningioma. Two tumor subgroups, anterolateral (AL) and posteromedial (PM), were distinguished according to their site of attachment: the middle third and the posterior third of the tentorial free margin, respectively. Clinical presentation, radiological findings, surgical approaches, extent of resection, and outcome were compared. Results The 2 subgroups differed by tumor size (larger in PM), incidence of the direction of growth (infratentorial in PM), and hydrocephalus (only in PM), as well as by some clinical aspects. Surgical approach depended on tumor location: lateral (pterional, subtemporal, and retromastoid) for AL lesions; medial (occipital or supracerebellar infratentorial) for PM lesions. Total removal (Simpson grade I-II) was performed in 64% of cases and complications occurred in 14%. Stereotactic radiosurgery was performed in cases of incomplete resection. At a mean follow-up of 104.5 mo, clinical improvement with low recurrence (9%) was achieved. Conclusion Reallocation of tentorial edge meningiomas is the premise to compare treatment and further improve the approach case-by-case. In spite of their deep site, good outcomes can be achieved in both AL and PM tentorial meningiomas. Also of note is the indolent behavior of residual tumor.

Neurosurgical mapping studies with nouns and finite verbs are scarce and subcortical data are nonexistent. We used a new task that uses finite verbs in six Italian-speaking patients with gliomas in the left language-dominant hemisphere. Language-relevant positive areas were detected only with nouns in four patients, with both tasks yet in distinct cortical areas in one patient, and only with finite verbs in another patient. Positive areas and types of errors varied across participants. Finite verbs provide complementary information to nouns, and permit more accurate mapping of language production when nouns are unaffected by electrical stimulation.

Background The aim of this study was to explore the nociceptive system of patients affected by trigeminal neuralgia (TN) secondary to documented vascular contact who underwent microvascular decompression. For that purpose, we used the classical trigeminal reflexes and the trigeminal laser-evoked potentials (tLEPs) before and after surgery, in order to verify any possible change after decompression and determine if there was any correlation between the neurophysiological parameters and the clinical outcome. Methods Eleven patients affected by TN caused by trigeminovascular contact and 10 age-matched controls underwent conventional trigeminal reflexes (bilateral Blink Reflex/BR and Masseter Inhibitory Reflex stimulating infraorbital and mental nerves/MIR V2 and V3) and tLEPs. Patients repeated neurophysiological tests one week after surgery. Results Short-latency BR and MIR were normal in all patients before surgery and there was no statistical difference before and after surgery. Conversely, in patients before surgery, tLEPs' amplitudes were significantly lower in the affected than in the healthy side (p = 0.017 for V2 and 0.037 for V3 branches). After surgery, on the affected side, tLEP amplitude increased and the pre/post-operative difference was significant (p = 0.017 for V2 and 0.028 for V3 divisions). Nine patients referred satisfactory pain relief and the favourable clinical outcome correlated with the neurophysiological recovery. Conclusions This study demonstrates that TN caused by trigeminovascular compression may be related to Aδ fibres impairment, and tLEPs are more sensitive than conventional trigeminal reflexes to reveal small fibre dysfunction and to monitor the post-surgical outcome in these patients.

BACKGROUND In brain tumour patients, impairments in every day functioning can impact quality of life, and are therefore an important outcome in both clinical trials and practice. One way to measure every day functioning is with an activities of daily living (ADL) questionnaire. Instrumental ADL (IADL) are the cognitively more complex activities, that are essential to function autonomously within society. Cognitive decline may therefore negatively impact IADL, making these activities particularly relevant to brain tumour patients. The aim of this study is to develop a reliable and valid questionnaire to measure IADL in primary malignant and metastatic brain tumour patients. MATERIAL AND METHODS The questionnaire development study followed the standard European Organisation for Research and Treatment of Cancer (EORTC) four phase methodology: (I) generation of activities list, (II) construction of item list, (III) pre-testing, and (IV) field testing. This report covers phases I-III. To ensure cross-cultural validity, participants were recruited from different countries (The Netherlands, United Kingdom, Italy, Austria and Japan). In phase I, potential activities were identified based on a literature review and in-depth interviews with patients, proxies and healthcare professionals. In phase II, activities were turned into items, and translated into all required languages by the EORTC Translation Unit. In phase III, the item list was pre-tested in patient-proxy dyads. In accordance with predetermined decision rules to reduce items, final items were selected, and preliminary psychometric properties (i.e. factor structure, validity, reliability) were assessed. RESULTS Phase I (N=44 dyads) resulted in 59 IADL activities which were converted into 59 items in phase II. In phase III, N=85 dyads completed and reviewed this item list. The item list was subsequently reduced to 32 items. An exploratory factor analysis indicated several items measuring similar underlying constructs (e.g. domestic life and using computer/smartphone) showing acceptable to good (α≥0.7) internal consistency (range α=0.69–0.89). Seven items were less related to these underlying constructs (e.g. work or managing your own medication), and therefore single items. Construct validity, measured with known-group comparisons analyses between cognitively impaired and unimpaired patients, showed significant differences in scores between the two groups on some scales and several single items. CONCLUSION The currently developed EORTC IADL-BN32 questionnaire can be a valuable asset in assessing IADL functioning in brain tumour patients, but further validation in phase IV is required and is planned.

Morphological assessment using Computed Tomo-graphy (CT) or Magnetic Resonance Imaging (MRI) is still the workhorse of tumor detection and diagnosis. In particular, MRI provides detailed information about cerebral tumor anatomy, cellular metabolism and hemodynamic features, making it a fundamental tool for a correct diagnosis, treatment and monitoring of the disease. Various new functional imaging modalities assessing tissue microstructure and physiology have increased the scope of neuro imaging and raised expectations among clinicians. This article provides an overview of the most advanced MR imaging techniques (functional MRI, perfusion-weighted imaging, diffusion-weighted imaging and MR spectroscopy) now available for neurosurgical planning and their role in brain tumors assessment. Their pros and cons are analyzed in order to find out which one may be chosen as best diagnostic pre-surgical protocol. At the moment none of the single techniques can be considered the golden standard; only the integration of advanced and conventional MR imaging proves to be a reliable tool in the hands of the neuro-radiologist and neurosurgeon, thus maximazing tumor resection and function preservation.

We included patients who received temozolomide concurrent with and adjuvant to radiotherapy after diagnosis of GBM and had a second surgery performed at least 3 months after radiotherapy completion. Other eligibility criteria were age ≥18 years and Eastern Cooperative Oncology Group performance status 0-2. We evaluated theFrom our institutional data warehouse, 295 patients with recurrent GBM who underwent second surgery were evaluated.

Syringomyelia is a misleading disease since the problem always lies elsewhere. Arachnoiditis, because it is radiographically difficult to discern, is an especially insidious cause. To better guide selection from among surgical treatment options for syringomyelia, we reviewed our case series of patients without Chiari malformation or spinal injury. Excluding syringomyelia due to Chiari malformation, spinal cord injury, and tumors, 32 patients (mean age 44 years) were operated on between 1995 and 2013 and followed up for a mean of 53.8 months. Presumed causes at diagnosis, clinical and radiological findings, type of operation, clinical and radiological outcome were reviewed. Duration of clinical history varied widely (range 6–164 months). Clinical assessment was based on the McCormick classification (15 independent, 17 dependent). Causes included birth trauma, pyogenic meningitis, tuberculous meningitis, postoperative scarring, dysraphism, and basilar impression. Treatment was local decompression with arachnoid lysis and shunts. Hindbrain-related syringomyelia was differentiated from non hindbrain-related syringomyelia. Hindbrain arachnoiditis was significantly associated with radiological findings at the foramen magnum (p = 0.01) and craniocervical decompression (p

Objective Tentorial meningiomas represent a heterogeneous group of tumors. Most of the published series deal with either a small number of patients or consider different locations as a whole, making indications for treatment and prognosis difficult to drawn. We analyzed the surgical management of the lateral tentorial meningiomas, a homogenous and rare subgroup. Methods Fifty-two later tentorial meningiomas were operated on between 1990 and 2010. Clinical and radiologic features and surgical management of these patients were reviewed. Tumors were further subcategorized in to posterior/intermediate and in to supra-/infratentorial subgroups. Surgical outcome, long-term results, and prognostic factors are described. Results Mean age was 57 years (41 female, 11 male). Mean tumor size was 46 mm; most had an infratentorial location (36 vs. 16). Prevailing presenting symptoms were headache ( n = 28), vertigo/gait disturbances ( n = 25), and confusion and visual disturbances ( n = 16). The infratentorial group presented with a poorer clinical condition before as well as after operation. Extent of tumor resection was Simpson I in 10 patients, II in 26, III in 6, and IV in 10. Subtotal resection was statistically correlated with sinus invasion and tumor size. There was no surgical mortality. Permanent complications occurred in 3 patients. At latest follow-up (mean, 119 months) 42/46 had resumed their normal daily activity. Six cases recurred and remained stable after radiosurgery. Conclusions Lateral tentorial meningiomas are a homogeneous entity characterized by simple surgical approaches and favorable outcome (no mortality and low overall morbidity). Infratentorial location was more frequent and was characterized by poorer outcome. The limiting factors for surgical removal were tumor size and sinus invasion. The latter point strengthens the rationale for their classification into posterior and intermediate.

Background: The most appropriate management of recurrent glioblastoma is still controversial. In particular, the role of surgery at recurrence remains uncertain. Patients & methods: From our Institutional data warehouse we analyzed 270 consecutive patients who received second surgery for recurrent glioblastoma, to assess survival after second surgery, and to evaluate prognostic factors. Results: Complete resection was found in 128 (47.4%) and partial resection in 142 patients (52.6%). Median survival from second surgery was 11.4 months (95% CI: 10.0–12.7). Multivariate analysis showed that age (p = 0.001), MGMT methylation (p = 0.021) and extent of surgery (p < 0.001) are associated with better survival. Conclusion: A complete resection should be the goal for second resection and younger age and MGMT methylation status might be considered in the selection of patients.

Object The ventriculus terminalis, an embryological remnant consisting of the ependymal-lined space of the conus medullaris, can occasionally become symptomatic after cystic dilation. In the existing literature, consisting of 32 cases, the preferred type of management (conservative vs surgical) is still debated. The object of this study was to report the surgical results in a consecutive series of 10 adult patients with cystic dilation of the ventriculus terminalis (CDVT), to match them with data retrieved from the relevant literature, and specifically to validate a new recent clinical classification. Methods The authors reported 13 new cases of CDVT treated in the Department of Neurosurgery at University Hospital in Verona, Italy. Treatment modalities and clinical and radiological outcomes, both early and at follow-up, were analyzed and compared with a preoperative classification of clinical presentation, as established by de Moura Batista and colleagues (2008). Results Surgical treatment seemed to guarantee the resolution of CDVT. Dorsolumbar laminotomy, myelotomy, and cystic drainage were performed in 10 patients. Patients with Type I symptoms (nonspecific complaints) often presented with comorbidities (herniated disc or facet hypertrophy) confusing their clinical status. The surgical treatment of patients with Type I symptoms promoted good results only if the diagnosis of CDVT was definitive and symptoms had rapidly evolved. In patients with Type II (focal neurological deficits) and III (sphincter disturbances) symptoms, surgical treatment sustained improvement even at the late follow-up. Conclusions While confirming the usefulness of de Moura Batista and colleagues' classification in its impact on prognosis, the authors propose a revision of the classification with subgroups Type Ia (nonspecific symptoms without clear relation to CDVT), which is best treated conservatively, and Type Ib (rapid onset and invalidating unspecific complaints without comorbidities), which may benefit from surgical evacuation.

Although generally well tolerated, awake craniotomy is burdened by non-negligible failure rates. The aim of this pilot study was to verify the feasibility of a wider research scope to define objective criteria for patient exclusion and the risk of intraoperative mapping failures.Twenty-one patients with brain tumors were subjected to a procedure in 3 steps: neuropsychological criteria for both cognition and language; psychological questionnaires for anxiety, attitude to pain and depression, and psychophysiological monitoring for the candidate's capacity for self-control; and an intraoperative interview for the patient's perception during awake procedure. Outcome measures were as follows: (1) patient compliance, defined as patient response to the intraoperative procedure and measured by psychological scale scores for fear and pain, and (2) failure, defined as the impossibility to complete brain mapping (minor) or conversion to general anesthesia (major). Data analysis included the description of preoperative and intraoperative assessments and their evaluation (Spearman ρ test), and the prognostic factors for intraoperative compliance and procedure failure (Mann-Whitney test).Three patients were considered ineligible after the first step. In the remaining 18, the responses of 10 patients fell within the normal range and 8 showed some degree of impairment on at least 1 preoperative evaluation, but not enough to be excluded from awake surgery. The data analysis also showed that fear of pain correlated with pain felt during the operation and preoperatively with depression and psychophysiological changes, the latter of which was associated with fear felt during craniotomy. Minor failures occurred in 2 patients.From these preliminary results, we observed that warning signs for minor failure were fear of pain and anxiety, as revealed by psychological questionnaire responses, and the incapability of self-control at psychophysiological monitoring. This assessment may serve to fit mapping modality to the single patient and to avoid complications.

In surgery for tumors of the dominant hemisphere, the attention devoted to quality of resection and preservation of language function has not been accompanied by comparable interest in preservation of cognitive abilities which may affect quality of life. We studied 22 patients undergoing awake surgery for glioma removal in the language areas of the brain. Besides monitoring tumor variables (size, location, histology, edema), we used a multifaceted battery of tests to investigate mood, cognition, and language in an attempt to assess the burden of disease and treatment, and the relationships between these three dimensions. Baseline assessment showed that 45% of the patients were depressed and 23% anxious; some cognitive and language impairment was noted for 59 and 50%, respectively. A general decline in postoperative cognitive performance (significant for memory and attention only) and language function (significant for picture naming) was observed, whereas depression was unchanged and anxiety decreased. Tumor histology, but not demographic variables or extent of resection, correlated with postoperative cognitive changes: patients undergoing surgery for high-grade tumors were more likely to improve. No correlation was observed between scores for mood, cognition, and language function. A subset of patients with low-grade glioma was followed up for 3-6 months; although some improvement was observed they did not always regain their preoperative performance. In conclusion, we believe that cognitive assessment performed in conjunction with language testing is a necessary step in the global evaluation of brain tumor patients both before and after surgery.

Neuropsychological testing is not a luxury in the management of patients with brain tumors. Regardless of the therapeutic approach selected in each case (but, especially in the case of awake surgery), it provides information on the status of language and other cognitive functions. This information can be used preoperatively to identify eloquent areas and to provide a baseline against which further examinations can be evaluated, intraoperatively to identify eloquent areas, and post-operatively and at follow-up to ascertain the short-term and long-term consequences of surgery, as well as those of other treatments (chemotherapy and radiotherapy), and to plan and monitor rehabilitative treatments.

INTRODUCTION: Symptom management in glioma patients remains a challenge, as patients suffer from various concurrent occurring symptoms. This study aimed to identify symptom clusters and to examine the relation between these symptom clusters and functioning. METHODS: We prospectively included individual patient data from previously published international randomized controlled trials (RCTs) in glioma patients. Symptom prevalence and level of functioning were assessed with EORTC QLQ-C30 and QLQ-BN20 questionnaires. The magnitude of the associations between symptoms were examined with Spearman correlation coefficients and correlation matrices. Hierarchical cluster analyses (looking at euclidean distances) were performed to identify symptom clusters. Multivariable regression analyses were performed to analyze associations between the symptom clusters and functioning, adjusted for confounding variables. RESULTS: Data of 3595 newly diagnosed glioma patients who completed the questionnaires before randomization indicated that many patients (44%) suffered from 5–10 symptoms simultaneously. The most prevalent symptoms were fatigue, drowsiness and motor dysfunction, experienced by 86%, 59% and 55% of the patients respectively. Five symptom clusters were identified with hierarchical cluster analyses: motor cluster, fatigue cluster, headache cluster, gastrointestinal/seizures cluster and hair loss/itchy skin cluster. Having symptoms in the motor cluster had a clinically relevant and significant negative influence (≥10points difference) on the global health status/quality of life scale, and physical, role, cognitive and social functioning (Beta’s ranged from -10.9 to -18.6, all p

In the last few years much has been published to validate new technology in brain mapping for clinical purposes, but there have been few clinical results. In this report we describe our five-year experience in the surgical management of malignant gliomas around motor areas with an evaluation of the impact of functional magnetic resonance imaging (fMRI) plus navigator and intraoperative neurophysiology (IN). End-points were extent of removal, morbidity, and survival. Variables describing patient and tumor characteristics and treatment modalities were statistically weighted in relation to treatment outcome. Tumor depth (P = 0.01), midline shift >/=1 cm. (P = 0.05), and insular location (P = 0.001) negatively affected extent of removal, whereas IN (P < 0.001) and fMRI plus navigator (P = 0.02) contributed to increasing the rate of total removal (73\%, 71\% vs. 40\%). Postoperative motor impairment was mild and transient in a minority of cases (20\%). General complications, as defined by the Glioma Outcome Project, occurred in 23\% of cases. IN was the only factor associated with acute postoperative motor deterioration (P < 0.001). IN and age >65 years (P = 0.01) were associated with the occurrence of complications. Overall survival was significantly higher in patients operated with IN or fMRI plus navigator (P < 0.01). Comparing different surgical strategies used in the same period, we observed that supportive technologies in glioma surgery have their primary impact on the quality of resection and survival. IN led to transient motor impairment and some additional complications which did not affect functional outcome.

Purpose We performed a new phase II trial enrolling patients with newly diagnosed high-grade glioma (HGG) to test the efficacy of a weekly alternating temozolomide (TMZ) schedule after surgery and concomitant chemoradiotherapy. Methods From January 2005 to January 2007, 34 patients (21 men, 13 women; age range 30–70, mean age 53) were enrolled. There were 32 glioblastoma multiforme and two anaplastic astrocytoma. Each patient after surgery received standard concurrent chemoradiotherapy. After a 4-week break, patients were then to receive 12 cycles of 1-week-on/1-week-off TMZ, with 75 mg/m2 for the first cycle, 100 mg/m2 for the second, 125 mg/m2 for the third, and 150 mg/m2 from the fourth to the 12th. Hematological toxicity was monitored every week during concomitant chemoradiotherapy and then every 4 weeks. Results After 12 months from the end of radiotherapy, the overall survival (OS) rate was 59% (20/38), distributed as follows: 60% (18/30) for recursive partitioning analysis (RPA) class 4 patients and 33% (1/3) for RPA class 6 patients; the only RPA class 1 patient was alive and disease free at the time of writing. Median OS was 13 months [95% confidence interval (CI) 11.02–14.98 months]. Hematological toxicity was seen in six patients (18%): grade 1 neutropenia in four, grade 2 thrombocytopenia in one, and grade 4 thrombocytopenia plus grade 1 neutropenia in one. There was one case of opportunistic infection (Pneumocystis carinii pneumonitis). Conclusion The toxicity of the TMZ dose-dense regimen was very low. Results seem to be encouraging for RPA lower classes (patients with good prognostic factors).

BACKGROUND No data on the role of chemotherapy in recurrent ependymal tumors are available in adults. The aim of the current study was to investigate outcomes after salvage chemotherapy in this setting. METHODS A retrospective review was made of the charts of 28 adults (≥ 18 years) with progressive or recurrent ependymal tumors after surgery and radiotherapy, who received chemotherapy between 1993 and 2003 in 3 institutions of the Gruppo Italiano Cooperativo di Neuro-Oncologia network. RESULTS Thirteen patients (46.3%) received cisplatin-based chemotherapy (Group A) and 15 (53.7%) received regimens without cisplatin (Group B). Platinum-based chemotherapy yielded 2 complete responses (CR) (15.4%) and 2 (15.4%) partial responses (PR), whereas 7 patients (53.8%) remained stable (SD). After regimens without cisplatin, there were no CR, 2 PR (13.3%), and 11 SD (73.3%). The overall median time to progression was 9.9 months (95% confidence interval [95% CI], 7.5–21.7 months), 9.9 months (5.2–not reached) for Group A and 10.9 months (95% CI, 7.17–23.9 months) for Group B. The overall median survival (OS) was 40.7 months (95% CI, 16–not reached), 31 months (21–not reached) for Group A and 40.7 months (13.4–not reached) for Group B. CONCLUSIONS Cisplatin-based chemotherapy achieved a higher response rate, but did not prolong disease progression-free survival or OS. More active regimens for the salvage treatment of ependymal tumors have yet to be found. Cancer 2005. © 2005 American Cancer Society.

Introduction MicroRNAs (miRNAs) are small non-coding RNAs which are known to play an important role in proliferation, differentiation, invasion and angiogenesis of different malignant tumors. The role of tissue and blood level of miRNAs as useful bio-markers of gliomagenesis is also increasing. Interest is growing on tissue and plasma miR-222 level as prognostic marker in first line chemo- and radiotherapy treated patients with glioma, the mechanisms of action and the target genes of this miRNA in gliomagenesis being under extensive investigation. Furthermore, some evidence exist that miR-221 and miR-222 are upregulated in glioblastoma (GBM) patients and that these paralogues target O(6)-methylguanine-DNA methyltransferase (MGMT) mRNA. Here we focused on the prognostic role of tissue miR-222 in GBM patients. Experimental procedures 63 GBM patients were included in the analysis (overall survival (OS) range: 8-122 months). For each patient formalin-fixed paraffin-embedded tissue samples at first surgery were collected. miR-222 expression level was analyzed by qRT-PCR with TaqMan probe, normalizing miR-222 level on miR-U6 level. OS was analyzed by Kaplan-Meyer (K-M) survival curves. MGMT methylation analysis by pyrosequencing on tumoral DNA is on-going. Results In silico analysis of The Cancer Genome Atlas (TCGA) for GBM defined a median OS of 13.6 months in the whole sample population (n=199). Comparison of median OS, done stratifying two groups according to median tissue miR-222 expression level (low 222 vs high 222), showed a significant increased OS in low 222 patients (14.3 vs 13.1 months, p=0.017). Interestingly, K-M curves overlapped for the first 14 months and then diverged after. In particular, K-M analyses starting from 14 months revealed a significantly increased hazard ratio for high 222 patients (HR 1.96, 95% CI = 1.25 - 3.09, p Conclusions Quantitative analysis of miR-222 expression in the tumor tissue obtained at first surgery might provide a relevant prediction of prolonged survival in GBM patients. Further conclusions will be presented on the relationship between miR-222 and MGMT which will shed light on a possible role of miR-222 on gene methylation and GBM epigenetics. Citation Format: Laura Lattanzio, Marzia Borgognone, Rossella Merli, M. Cristina Dechecchi, Gianluigi Dorelli, Cristina Mocellini, Andrea Talacchi, Daniela Vivenza, Federica Tonissi, Alessandra Santangelo, Fabrizio Giordano, Caludio Ghimenton, Albino Eccher, Sergio Pericotti, Luisa Zanolla, Claudio Bernucci, Marco Merlano, Cristiana Lo Nigro, Giulio Cabrini. Low miR-222 expression levels predict long-term survival of patients affected by glioblastoma [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2017; 2017 Apr 1-5; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2017;77(13 Suppl):Abstract nr 4432. doi:10.1158/1538-7445.AM2017-4432

BACKGROUND Choroid plexus papillomas (CPPs), of the cerebellopontine angle (CPA), are a rare entity and no surgical series have been reported so far. We reviewed all the pertinent literature of 12 patients operated on in the last 20 years at our institution. METHODS All the patients were adults, ranging from 19 to 61 years. The group included 6 males and 6 females. Preoperatively, on computerized tomography (CT) ( n = 10) or magnetic resonance imaging (MRI) ( n = 4), differential diagnosis was difficult to achieve, especially with meningiomas. Hydrocephalus was disclosed in 8 cases. Angiography ( n = 11) showed tumor blush with typical vascular supply in almost half the cases. RESULTS In 6 patients a midline approach via the cerebellomedullary fissure was used; in the remaining 6 patients the retromastoid route was adopted. We found 2 "unconnected" tumors; no hilum was identified at surgery. Total tumor removal was achieved in 6 patients, predominantly in the last cases. Two patients died in the postoperative period, 3 patients had mild additional deficits, whereas 7 patients were stable or improved. All the patients were followed up for a mean period of 8.2 years. Conventional radiotherapy was carried out in 5 patients; 1 of them after tumor recurrence. Stereotactic radiotherapy was performed in 3 patients; 2 of them after recurrences. Two patients showed tumor progression and died during the follow-up. One of them presented a suprasellar metastasis and died much earlier (2 versus 7 years). CONCLUSION Careful assessment and surgical planning is accomplished with the combined information from CT, MRI, and angiography. Typical characteristics are the following: vascular supply from the choroidal arteries, ventral extension, adhesion to the brainstem, and lower cranial nerves. Nowadays, total removal of CPPs of the CPA can be achieved with acceptable morbidity and mortality. In our experience, conventional radiotherapy did not prove effective.

Brain tumors in children under 3 years of age differ in clinical presentation and pathological behavior from those in older patients. In this study, we reviewed data from 39 children (24 males and 15 females) under 3 years of age who were treated for intracranial brain tumors since the introduction of magnetic resonance imaging. The purpose was to assess correlations between clinicoradiological and treatment-related factors, and their impact on prognosis. The following factors were analyzed: sex, age, duration of symptoms, intracranial hypertension on admission, tumor location, surgical removal and histology. Associations between these factors and correlations with prognosis were determined using bivariate analyses (2 test) and Kaplan-Meier survival curves. Collins' concept of a period of risk recurrence was tested. Mean follow-up was 41 months (range 0-136). In March 1998, 20 children were still alive (51.2%) with a mean survival time of 65 months (range 2-136). The incidence of supratentorial tumors was significantly higher in children less than 1 year old (p = 0.027). Lateral tumors were 9/10 (90%) supratentorial versus only 7/26 (27%) midline tumors (p = 0.001). Outcome (dead or alive) was significantly better (p = 0.037) for low-grade astrocytomas (9/12 = 75% survival) when compared to ependymomas (2/6 = 33%) and primitive neuroectodermal tumors (3/12 = 25%). Total tumor removal was achieved in 20 cases and was associated with a better outcome (65 vs. 33% survival; p = 0.049). Survival analysis confirmed a worse prognosis for children with ependymomas and primitive neuroectodermal tumors (p = 0.011) and revealed a worse survival for children with intracranial hypertension on admission (p = 0.047). Total tumor removal was associated with a longer survival, although not significantly (p = 0.077). Finally, we found no exceptions to Collins' law.

A case of dumbbell-shaped hypoglossal neurinoma with intra- and extracranial extension is reported. The tumour was surgically completely removed in a one-stage operation via a dorsolateral sub-occipital transcondylar approach. Clinical presentation and the role of high-resolution CT-scan, MRI and angio-MRI in diagnosis and surgical planning are discussed. We include a review of the literature concerning these rare tumours of the foramen magnum region.

Spheno-orbital meningioma (SOM) is an intriguing tumor because of the many different factors that can influence clinical and oncological outcome after treatment. Reasoning that outcome indicator measurement is key to improving therapy, we retrospectively evaluated the management of proptosis and other ocular symptoms in 47 patients surgically treated for SOM at our department in the last 10 years. This patient series was characterized by a high rate of tumor infiltration of the extradural cranial base. Clinical outcome was assessed by comparing preoperative and postoperative ophthalmological and neurological signs. Acute postoperative complications were reported, and clinical and radiological outcome was assessed at 4–6 months, 12 months, and the last follow-up. Proptosis (measured by Hertel exophthalmometry), visual acuity, visual field defect (measured by Goldmann perimetry), diplopia (measured by the Hess-Lancaster test), and other disturbances were rated as normalized, improved, or unchanged/worsened. The most common presenting symptoms were proptosis (95.7 %), visual impairment (51 %), and cranial nerve deficit (38.2 %). Surgery via the frontotemporal approach was performed in all 47 cases, with the primary aim to relieve symptoms/signs and maximize tumor resection. Bony orbital reconstruction was never performed. Complete resection was achieved in 51 % of cases (Simpson grades I and II) with minimal morbidity. At a mean follow-up of 52 months (range, 12–112), proptosis normalized in 90.9 % and improved in the remaining patients, visual acuity normalized in 20.8 % and improved in 45.8 % patients, cranial nerve deficit subsided in all but two cases. The recurrence rate was 29.7 %. One of the gold standards of surgical treatment, normalization of proptosis, can be achieved by accurate resection of the superior and lateral orbital walls. In this setting, careful reconstruction of the frontobasal dura is far superior to bony reconstruction. Complete tumor resection should not be pursued at the expense of increased morbidity.

Objective Posterior gyrus cinguli tumors are a well-defined group of tumors that pose considerable challenges in creating surgical access and manipulating adjacent eloquent areas (visual and motor). Here we report our 5-year experience in the surgical treatment of these tumors and describe tumor characteristics, surgical steps, critical aspects, and prognostic factors. Methods This series comprises 37 patients operated on for glioma (high-grade in 28, low-grade in 9), often presenting with motor impairment (n = 20), intracranial hypertension (n = 15), seizures (n = 11), and/or hemianopia (n = 9). Preoperative assessment was performed with magnetic resonance imaging. Half of the tumors were more than 4 cm in size, and the majority presented secondary extension into the fronto-parieto-occipital area, the temporo-mesial area, and/or the corpus callosum. Positioning and assisted surgery were optimized in each patient based on preoperative planning. Results The ipsilateral interhemispheric approach was elected in all cases. Tumor size and extension were significantly associated with the degree of tumor removal. Total removal was achieved in 25 patients (65%); 4 (10%) had persistent morbidity (visual or motor deficits). The occurrence of local and systemic complications was negligible. Conclusions Surgical treatment of posterior gyrus cinguli tumors can be safely approached via the interhemispheric route as it permits several beneficial operative maneuvers in selected cases.

Direct cortical and subcortical stimulation has been claimed to be the gold standard for exploring brain function. In this field, efforts are now being made to move from intraoperative naming-assisted surgical resection towards the use of other language and cognitive tasks. However, before relying on new protocols and new techniques, we need a multi-staged system of evidence (low and high) relating to each step of functional mapping and its clinical validity. In this article we examine the possibilities and limits of brain mapping with the aid of a visual object naming task and various other tasks used to date. The methodological aspects of intraoperative brain mapping, as well as the clinical and operative settings, were discussed in Part I of this review.

Electrocortical stimulation remains the standard for functional brain mapping of eloquent areas to prevent postoperative functional deficits. The aim of this study was to investigate whether the short-train technique (monopolar stimulation) and Penfield's technique (bipolar stimulation) would induce different effects on brain oscillatory activity in awake patients, as quantified by electrocorticography (ECoG). The study population was seven patients undergoing brain tumor surgery. Intraoperative bipolar and monopolar electrical stimulation for cortical mapping was performed during awake surgery. ECoG was recorded using 1 × 8 electrode strip. Spectral estimation was calculated using a parametric approach based on an autoregressive model. Wavelet-based time-frequency analysis was then applied to evaluate the temporal evolution of brain oscillatory activity. Both monopolar and bipolar stimulation produced an increment in delta and a decrease in beta powers for the motor and the sensory channels. These phenomena lasted about 4 s. Comparison between monopolar and bipolar stimulation showed no significant difference in brain activity. Given the importance of quantitative signal analysis for evaluating response accuracy, ECoG recording during electrical stimulation is necessary to characterize the dynamic processes underlying changes in cortical responses in vivo. This study is a preliminary approach to the quantitative analysis of post-stimulation ECoG signals.