Targeted sequencing of cancer-related genes reveals a recurrent TOP2A variant which affects DNA binding and coincides with global transcriptional changes in glioblastoma.

High-grade gliomas are aggressive, deadly primary brain tumors. Median survival of patients with glioblastoma (GBM, WHO grade 4) is 14 months and <10% of patients survive 2 years. Despite improved surgical strategies and forceful radiotherapy and chemotherapy, the prognosis of GBM patients is poor and did not improve over decades. We performed targeted next-generation sequencing with a custom panel of 664 cancer- and epigenetics-related genes, and searched for somatic and germline variants in 180 gliomas of different WHO grades. Herein, we focus on 135 GBM IDH-wild type samples. In parallel, mRNA sequencing was accomplished to detect transcriptomic abnormalities. We present the genomic alterations in high-grade gliomas and the associated transcriptomic patterns. Computational analyses and biochemical assays showed the influence of TOP2A variants on enzyme activities. In 4/135 IDH-wild type GBMs we found a novel, recurrent mutation in the TOP2A gene encoding topoisomerase 2A (allele frequency [AF] = 0.03, 4/135 samples). Biochemical assays with recombinant, wild type (WT) and variant proteins demonstrated stronger DNA binding and relaxation activity of the variant protein. GBM patients carrying the altered TOP2A had shorter overall survival (median OS 150 vs 500 days, P = .0018). In the GBMs with the TOP2A variant we found transcriptomic alterations consistent with splicing dysregulation. luA novel, recurrent TOP2A mutation, which was found exclusively in four GBMs, results in the TOP2A E948Q variant with altered DNA binding and relaxation activities. The deleterious TOP2A mutation resulting in transcription deregulation in GBMs may contribute to disease pathology.

Freezing: how do water mites (Acari: Hydrachnidia) survive exposure to sub-zero temperatures?

Until now, very little is known about the ability of adult and deutonymph water mites (Acari, Hydrachnidia) to survive in sub-zero temperatures. Information concerns mainly water mites from vernal astatic waters, and the knowledge has never been experimentally verified. To determine the sensitivity of water mites to freezing, experiments were conducted on (1) the impact of acclimatization, (2) temperature, and (3) duration of freezing on survival, (4) the survival rate of water mites from various types of water bodies, and (5) the survival rate of water mites from different climatic zones. The experiments were carried out in a phytotron chamber, and water mites were placed in containers (10 × 10 × 5 cm) filled with 4/5 of water for 10 specimens each. Water mites were identified to the species level after finishing the experiments. The temperature was lowered 1 °C every hour until the target temperature was reached. After a certain period of freezing (depending on the treatment) the temperature was raised by 1 °C every hour until it reached 4 °C. The time of the experiment was measured from the moment the desired temperature was reached (below 0 °C) until the ice thawed and the temperature of 4 °C was reached again. The highest survival rates had Limnochares aquatica, Piona nodata, Sperchon clupeifer and Lebertia porosa, followed by L. insignis, Hygrobates longipalpis, H. setosus, Limnesia undulatoides, Piona pusilla, Arrenurus globator, Hydrodroma despiciens, Piona longipalpis, Sperchonopsis verrucosa, Unionicola crassipes and Mideopsis crassipes; no specimens of Torrenticola amplexa survived. The following conclusions were drawn: (1) water mites can survive freezing to -2 °C, lower temperatures are lethal for them; (2) they survived better the short period of freezing (24-48 h) than the long period (168 h); (3) resistance to freezing seems to be an evolutionary trait of individual species, only partly related to the living environment; and (4) freezing survival rates are linked to the region of Europe and are much lower in Southern than in Central Europe.

Predictors of Class I epilepsy surgery outcome in tumour-related chronic temporal lobe epilepsy in adults.

OBJECTIVE: Temporal lobe tumours, especially low-grade gliomas and glioneuronal tumours, are common causes of seizures in patients referred for epilepsy surgery. We here present our experience of surgical treatment of patients with intractable chronic epilepsy associated with temporal lobe tumours, focusing on the long-term surgical outcomes and the features associated with better seizure control. METHODS: In this study, we retrospectively analysed 44 consecutive patients from a total of 182 with refractory temporal lobe epilepsy presenting with long-term intractable epilepsy due to a temporal lobe tumour who were surgically treated at our institution between 2005 and 2015 with post-surgical follow-up of at least two years. All patients underwent a standard pre-surgical evaluation that included: history and physical examination with a description of the seizure semiology, serial scalp EEG recording, brain MR imaging, and a detailed neuropsychological evaluation. Our surgical strategy comprised tumour resection, and combined mesial temporal and neocortical resection in most cases. RESULTS: No patient died during surgery or the postoperative course. Seven patients had postoperative complications, of whom two had permanent hemiparesis due to ischaemic stroke. At the final follow-up, a favourable seizure outcome (Engel Class I) was found in 37 patients (84%), including 31 (70.5%) in Engel Class IA (excellent result). Two (4.5%) patients presented with an Engel Class II outcome (unfavourable outcome). Five patients (11.5%) were in Engel Classes III or IV (surgical failure). We found that complete resection of the hippocampus along with tumour and temporal pole removal was strongly associated with seizure freedom (p = 0.015). Pathological diagnosis was also a significant prognostic indicator of tumour-related seizure freedom. Patients with a diagnosis of a glioneuronal tumour benefited from more seizure freedom after resection compared to those who had a low-grade glioma (p = 0.024). CONCLUSION: The most appropriate management of tumour-related chronic temporal lobe epilepsy in adults appears to be tai-lored temporal lobe resection including tumour and hippocampal complex removal. Surgical treatment of tumoural temporal lobe epilepsy demonstrates excellent results in terms of seizure improvement, especially in patients with glioneuronal tumours.

Trigone ventricular meningiomas - clinical characteristics, histopathology and results of surgical treatment.

AIM OF THE STUDY: Intraventricular meningiomas (IVMs) are rare tumours accounting for 0.5-3.0% of all meningiomas. IVMs require different surgical approaches and preparation in deep brain areas. The aim of our study was to present the clinico- -histopathological characteristics and treatment outcomes of trigone IVMs in a series of 15 patients. MATERIALS AND METHODS: Eight women and seven men (mean age 52) with 15 trigone IVMs were retrospectively analysed. Patients presented with headache (47%), psychoorganic syndrome (40%), hemianopsia (33%) or paresis (20%), including three (20%) patients with Karnofsky Performance Scale (KPS) < 80. Mean tumour size was 55.2 mm (range: 30-100 mm). RESULTS: Gross total tumour resection was performed in 14 (93%) cases, and subtotal in one (7%). A new deficit appeared in 83% (5/6) following a transparietal approach, in 14% (1/7) following a transtemporal approach, and in none of two patients following a transoccipital approach. Postoperative complications occurred in six (40%) patients; no patient died, but in two (13%) the new deficit was permanent. Tumour re-growth was found in two (13%) patients after 14 and 31 months. Meningiomas of WHO grade I occurred in 12, grade II in three, and grade III in one tumour recurrence. In long-term follow-up (mean: 60.8 months), including the results of revision operations, KPS: 80-100 was in 13 (87%) patients, KPS: 50 in one (severe hemiparesis after revision) and one patient was lost to follow-up (KPS: 100 on discharge). CONCLUSIONS: 20% of IVMs in our series were atypical. The results of surgery for IVMs, although satisfactory in general, require further improvement by reducing the rate of focal deficits resulting from a surgical approach.

Diffusion tensor tractography of pyramidal tracts in patients with brainstem and intramedullary spinal cord tumors: Relationship with motor deficits and intraoperative MEP changes.

PURPOSE: To evaluate whether pyramidal tracts course alterations observed in diffusion tensor tractography (DTT) in cases of brainstem and intramedullary spinal cord tumors reflect patient clinical status and prognosis. MATERIALS AND METHODS: For this purpose, we assessed in 17 patients relationships between pyramidal tracts course alterations observed in DTT (classified into four categories: unaffected; displaced or interspaced; partially disintegrated and completely disintegrated) performed on a 1.5 Tesla scanner and the presence of preoperative motor deficits, changes observed in motor evoked potentials (MEPs) records at the beginning of the operation, deterioration of the MEPs records during the operation, and perioperative deterioration of muscle strength. RESULTS: We found that, if the picture of pyramidal tracts in DTT was worse, motor deficit was more common (P = 0.062). This observation was even more evident (P = 0.027), when cases with at least partially destroyed pyramidal tracts were compared with cases with normal or at most displaced or interspaced by tumor but still preserved pyramidal tracts. Significant relationships were also found between changes in DTT and abnormal MEP records at the beginning of the operation (P = 0.032) and perioperative deterioration of muscle strength (P = 0.0058). CONCLUSION: A close relationship was found between pyramidal tracts course alterations in DTT imaging and preoperative motor status and especially with changes in the MEP records at the beginning of the operation. DTT may be a method that allows the better planning of brainstem and intramedullary spinal cord tumors operations and may help in the risk assessment of postoperative motor deficits. LEVEL OF EVIDENCE: 3 Technical Efficacy: Stage 4 J. MAGN. RESON. IMAGING 2017;46:715-723.

Surgical treatment for spinal dural arteriovenous fistulas: Outcome, complications and prognostic factors.

BACKGROUND AND PURPOSE: Spinal dural arteriovenous fistulas (SDAVFs) are rare, acquired pathology and they inevitably lead to severe disability if untreated. The aim of this study is to present the outcome and complications, and to find factors that may affect the outcome after surgical treatment. METHODS: Seventeen consecutive patients (men - 14, women - 3, age: 41-79) were retrospectively analyzed. The patients presented with paraparesis (88%), bladder symptoms (71%) and/or sensory disturbances (65%). The fistula was found in the upper thoracic spine in 2 cases, in the lower thoracic (T7-Th12) in 11 cases, and in the lumbar spine in 4 cases. Microsurgical shunt interruption was performed in all, followed by epidural arteries coagulation in 12 cases. RESULTS: In the long term, improvement or achievement of a good stable condition was observed in 13 patients (76%), and no patient deteriorated. All 5 paraplegic patients improved by at least 1 grade in MCS. Satisfactory results (modified McCormick Scale grades I-II) were found in 10 patients (59%), and 15(88%) were independent. Postoperative complications occurred in 4 patients (24%), two of them (12%) required revision surgery for epidural hematoma. The success rate was 94%; one patient required revision surgery for recurrent SDAVF. Better neurological condition on admission (p=0.0098) and age >60 years (p=0.0498) were the factors associated with satisfactory outcome. CONCLUSIONS: Microsurgical closing of a SDAVF brings good and stable results over time. Aggressive treatment should be attempted even in cases of total loss of spinal cord function. Neurological condition before surgery and age may influence the outcome.

Epidermoid cysts of the cerebellopontine angle: Clinical features and treatment outcomes.

OBJECTIVE: To report clinical characteristics, treatment outcomes and risk of recurrence in patients with surgically treated cerebellopontine angle epidermoids. METHODS: In 1994-2013, we operated 17 patients, including 7 with tumor limited to the cerebellopontine angle, 7 with cerebellopontine angle tumor penetrating supratentorially, and 3 with cerebellopontine angle tumor extending along skull base to contralateral cerebellopontine angle. All patients were followed-up for the mean duration of 126 months. RESULTS: On admission cranial nerve symptoms predominated. Total tumor removal was achieved in 5 patients, and incomplete removal (with small tumor remnants left on vessels, nerves, or brainstem) in 12 patients. Postoperatively, preoperative deficits worsened in 2 and new postoperative deficits occurred in 10 patients. The extent of tumor expansion had no effect on postoperative morbidity and risk of recurrence. During long-term follow-up, improvement or resolution of preoperative deficits was seen in 11 of 17 patients, and new postoperative deficits in 8 of 10 patients. Symptomatic recurrences after an average of more than 9 years were noted in 5 patients, 3 of whom were reoperated. Recurrences occurred in some younger patients and always in area of primary tumor. No effect of extent of tumor removal on risk of recurrence was found. CONCLUSIONS: The extent of tumor removal had no effect on the risk of recurrence, and thus it may be acceptable to leave tumor capsule fragments adhering closely to nerves, vessels, or brainstem. During long-term follow-up, resolution or improvement of present preoperatively and new postoperative neurological deficits may be expected in most patients.

Surgery for sporadic vestibular schwannoma. Part II. Complications (not related to facial and auditory nerves).

INTRODUCTION: The aim of this study was to analyze the frequency and consequences of postoperative complications (PC) after surgery for sporadic vestibular schwannoma and to find factors that increase the risk of PC occurrence. MATERIALS AND METHODS: The study included 220 consecutive patients (134 women, 86 men; age ranged from 18 to 74) operated on with the retrosigmoid (217) or translabyrinthine (3) approach. Complicated postoperative period was defined as an occurrence of at least one of: cerebrospinal fluid (CSF) leakage, hematoma in the tumor bed, intracerebellar hematoma, cerebellar swelling, brainstem stroke, hydrocephalus (HCP), healing problems, meningitis and cranial nerves (excluding VII-VIII) palsies or cerebellar symptoms. Correlation studies and multivariate regression analysis were performed. RESULTS: PC occurred in 55 patients (25%). PC included lower cranial nerve (LCN) palsy (8.2%), cerebellar symptoms (7.3%), CSF leakage (5.9%), HCP (5%), CNVI palsy (3.1%), meningitis (1.8%), cerebellar swelling (1.4%), CNV dysfunction (0.9%), intracerebellar hematoma (0.5%) and lethal brainstem stroke (0.5%). In long term follow-up, LCN deficit was present in 2 patients (0.9%), cerebellar syndrome in 4(1.8%) and facial hypoesthesia in 2(0.9%). One patient (0.5%) developed bilateral blindness, secondary to preoperative optic nerve atrophy. As a result of PC, 10 patients (4.5%) required 11 additional surgical procedures. In statistical analysis, PC were independently related to preoperative cerebellar syndrome (p=0.002) and tumor size (>30 mm vs.<30 mm, p<0.05). The risk of PC diminished significantly with the increased number of performed procedures from 40% at the beginning to 16.4% in the last 55 cases. CONCLUSIONS: Tumor size, cerebellar syndrome at presentation and experience of the team were the three most important risk factors for PC occurrence. Permanent deficit secondary to PC remained in only 4% of the patients.

Surgery for sporadic vestibular schwannoma. Part I: General outcome and risk of tumor recurrence.

BACKGROUND: Vestibular schwannomas are slow growing, benign tumors. There are three possible management options: surgery, radiation treatment or active surveillance. The aim of this study was to assess the general outcome and risk of tumor recurrence. MATERIALS AND METHODS: The study included 220 consecutive patients (134 women, 86 men; the age ranged from 18 to 74) operated with the retrosigmoid transmeatal approach. The largest extrameatal diameter of the tumor ranged from 8 to 72mm (mean 30mm). According to the Samii grading scale, the tumors were classified as follows: T2-12 (6%), T3-51 (23%) and T4-157 (71%). Gross total resection was performed in 217 patients and neartotal in 3. RESULTS: Two hundred and eighteen (99.1%) patients were discharged home in a satisfactory neurological condition (GR or MD in GOS). One (0.5%) patient died due to brainstem infarction. One (0.5%) patient had unchanged severe cerebellar syndrome in comparison to the preoperative period (SD in GOS). In long-term follow-up, one patient went blind within a few months after surgery. Including the results of further neurosurgical procedures for CSF leak, shunt implantation, tumor regrowth and facial nerve reanimation, 98.6% of the patients were fully independent but with different neurological deficits. Tumor recurrence was observed in 5 (2.3%) patients during the follow-up period (mean term: 6.4 years). The average time to recurrence diagnosis was 8.8 years. All those patients were operated on again without any adjuvant therapy and there was no further re-growth at mean follow-up of 5.2 years. CONCLUSIONS: Complete removal of VS is usually curative and poses very low risks of severe disability (if audio-facial sequels are not included), mortality and long-term recurrence. For recurrent tumors, carefully tailored revision surgery without irradiation offers a high efficacy with low risk of complications.

Management of spinal tumors in neurofibromatosis type 2 patients.

OBJECTIVE: We sought to determine clinical characteristics of NF2 patients with spinal lesions and to define when and like the spinal tumors are a major problem in the treatment of patients with NF2. METHODS: The authors retrospectively reviewed the clinical records, neuroimaging studies, and follow-up data of the 34 patients with neurofibromatosis type 2, who were treated at our institution between 1998 and 2014. 23 patients harbored one or multiple spinal tumors. RESULTS: Patients with spinal tumors had a lower age at first symptoms of the disease, a higher number of intracranial meningiomas and non-vestibular schwannomas. 11 patients had one or more intramedullary tumors with MRI characteristics of spinal ependymomas. 22 patients had intradural extramedullary tumors. 7 patients presented with symptomatic spinal tumors on admission or developed symptoms during the follow-up. Only two intramedullary and four extramedullary tumors demonstrated growth in the mean radiological follow-up period of over 6 years. It was found that symptomatic both intra- and extramedullary tumors were associated with younger age at the onset of NF2-related symptoms. 2 patients with intramedullary tumors and 12 patients with extramedullary tumors underwent their tumors resection. In case of symptomatic tumors partial recovery was observed in two patients. CONCLUSION: It seems that close surveillance with MR imaging is a reasonable option for asymptomatic spinal tumors. Nevertheless, intramedullary tumor removal in non-growing and asymptomatic cases may be an option when ABI implantation is considered. Symptomatic tumors and those of documented growth should be eligible for surgical intervention.

Sensorimotor C5 and C6 radiculopathy caused by thrombosed vertebral artery dissection and successfully treated with limited oblique corpectomy - Case report.

The authors report the case of an exceptional presentation of vertebral artery dissection. A 44-year-old man who presented with left shoulder weakness, radicular pain and numbness of the left forearm and thumb was admitted to our hospital with an initial diagnosis of cervical disc herniation. Due to the inconsistency between the levels of radiculopathy (C5 and C6) and discopathy (C6-C7), neuroimaging examinations were extended. Based on MRI, MRA, CTA and DSA, left vertebral artery dissection with intramural hematoma was diagnosed. The patient underwent surgical decompression of the affected nerve roots using the anterolateral approach described by Bernard George. The radicular pain resolved immediately and sensorimotor deficit completely disappeared within 4 months. MRI/MRA performed 6 months after surgery showed the normal image of the vertebral artery. There were no ischemic events within 2.5 years of follow-up.

Oblique corpectomy for treatment of cervical spine epidural abscesses: Report on four cases.

BACKGROUND: Spinal epidural abscesses (SEAs) in cervical locations are particularly life-threatening. Currently, SEAs are widely treated with bony decompression, followed by internal stabilization in purulent osteomyelitis. However, recently, a growing number of studies have reported minimally invasive approaches without internal fixation. PURPOSE: We describe four patients with cervical SEAs that were evacuated by oblique corpectomy (OC) without fusion. METHODS: This study included two women and two men (aged 44-90) that received operations for removing ventral cervical SEAs. All patients presented with progressively increasing myelopathy, and 3 had severe comorbid conditions. In all cases, a multilevel OC without fusion was performed. The amount of bone resection was tailored to fit the needs of granulation removal, with an effort to retain as much of the vertebral bodies as possible. Then, pus was evacuated and debridement of granulation was performed, followed by rinsing and drainage. RESULTS: The neurological status of 3 patients improved significantly after surgery. At the last follow-up examination, one showed full recovery, and in two a minor residual deficit persisted. During mean follow-up of 5.5 years, no internal stabilization was necessary. The oldest patient was tetraplegic, and had several concomitant diseases. That patient died from sudden cardiac arrest on the third postoperative day. Oblique corpectomy did not affect the anterior or posterior column. Additionally, it provided a broad view of the ventral aspect of the spinal canal. CONCLUSIONS: Oblique corpectomy allows appropriate spinal cord decompression and granulation removal in the case of cervical spine epidural abscess, without sacrificing spinal stability.

Surgery for sporadic vestibular schwannoma. Part IV. Predictive factors influencing facial nerve function after surgery.

OBJECTIVE: To analyze the impact of various clinical, radiological and perioperative factors that could influence the facial nerve intraoperative disruption risk (CNVII-IDR) and its long-term function (CNVII-LTF) after vestibular schwannoma (VS) surgery. MATERIAL AND METHODS: The study included 212 patients operated on for sporadic VS with no history of previous treatment for VS or CNVII palsy. The mean size of the tumor was 30 mm. Gross (210) or near-total (2) resections were carried out using the retrosigmoid (210) or translabyrinthine (2) approach. Correlation studies and multivariate regression analysis (RA) were performed. RESULTS: In correlation studies, the CNVII-IDR was increased by: headaches and cerebellar ataxia if one of them was the first symptom of the tumor (33% and 29%, respectively, p=0.008); preoperative hydrocephalus (40% vs. 9%, p=0.01), tumor size >3 cm (18% vs. 5%, p<0.01), tumor volume >10 cm(3) (19% vs. 4%, p<0.01), right-sided location 15% vs. 6%, p=0.047), lateral "park-bench" position (19% vs. 5% for supine position, p<0.01) and the procedure order (16% for the first 106 procedures vs. 6% for the last 106 procedures, p<0.05). In RA the tumor volume (p=0.012), side of the tumor (p=0.028) and patient's position during surgery (p=0.016) independently affected the CNVII-IDR. The following factors correlated significantly with satisfactory CNVII-LTF (HB grades I-III): tumor stage

Clinical course and management of intracranial meningiomas in neurofibromatosis type 2 patients.

OBJECTIVE: The aim of this study is to evaluate our surgical experience with intracranial meningiomas in NF2 patients and provide knowledge of the natural history of these lesions. METHODS: We included in the natural growth study patients with the diagnosis of NF2 who harbored intracranial meningiomas and were observed for at least 1 year. Tumors that were resected before achieving long-term follow-up were excluded from this analysis. RESULTS: We found 118 intracranial meningiomas in 34 patients in our series. 8 meningiomas in 7 patients were symptomatic. It was found that with an increase in tumor volume, brain edema and with the tumor location at the skull base, meningiomas are more likely to be symptomatic. Univariate analysis revealed that tumor growth was associated with a younger age at the onset of NF2-related symptoms, greater initial tumor volume, brain edema and with the presence of intracranial non-vestibular schwannoma. Multivariate analysis showed that the probability of tumor growth is associated with prolonged follow-up time. De novo meningiomas exhibited a significantly higher growth rate than other meningiomas. These tumors were more frequent in patients with intracranial non-vestibular schwannoma and with increasing length of meningioma observation. CONCLUSION: Meningiomas occur in about half NF2 patients. Many of them exhibit slow growth and long remain asymptomatic, however, those associated with early onset of NF2 symptoms and other features of the disease severity should be monitored in case of clinical and radiological progression that may require surgical treatment.

Results of surgical treatment of anterior clinoidal meningiomas - our experiences.

OBJECTIVE: Presentation of our experience in the treatment of anterior clinoidal meningiomas, including evaluation of factors that may affect early and long-term treatment outcomes. METHODS: Thirty patients were operated with strategy of complete tumor resection using fronto-orbito-zygomatic approach. Outcomes were assessed by Glasgow Outcome Scale at discharge and by Karnofsky Performance Scale at follow-up. RESULTS: There were 6 tumors in group I, 20 in group II, and 4 in group III according to Al-Mefty classification. Complete tumor resection (Simpson I or II) was achieved in 19 patients, incomplete resection (Simpson IV) in 11: due to strict tumor adhesion to cerebral arteries in 5 and tumor extension to cavernous sinus in 6 cases. Operative mortality was 6.7%. Visual acuity improved in six among nine patients with impaired vision but in no one among nine patients with blindness. Normal life activity (80-100 KPS) could be carried out by 88% patients at follow-up. Recurrence was observed in two (11.8%) patients after radical removal and progression of residual tumor in two (25%) after subtotal resection. CONCLUSIONS: Complete tumor removal is possible with an acceptable risk of death and severe neurological deficits, except for cases with tumor extension to the cavernous sinus or strict tumor adhesion to cerebral arteries. Visual acuity improvement may be expected in two thirds of patients with impaired vision, but not in cases of blindness. In cases of incomplete tumor removal, use of stereotactic radiosurgery immediately after surgery seems justified.

Surgery for sporadic vestibular schwannoma. Part III: Facial and auditory nerve function.

OBJECTIVE: The aim of this analysis was to assess short-term and long-term outcomes with respect to the preservation of facial and auditory nerve function following surgery for sporadic vestibular schwannomas. MATERIAL AND METHODS: The study included 220 consecutive patients operated on with the retrosigmoid (217) or translabyrinthine (3) approach. The mean extrameatal diameter of the tumor was 30mm. In 217 patients, gross total resection was performed and near-total in 3. Before surgery, the facial nerve (CNVII) weakness was found in 18% of patients and only 20% had serviceable hearing. Intraoperative neurophysiological CNVII monitoring was routinely used (the last 211 procedures). Intraoperative monitoring of the cochlear nerve function was used when the preservation of hearing was attempted (45 procedures). RESULTS: The rate of CNVII continuity loss during surgery was 11%, however, this decreased to 6% in the second half of the series. Facial nerve function deteriorated, in 88% of the patients shortly after surgery. However, it improved in 87% in follow-up. Delayed CNVII palsy was found in 5% of the patients and had a good prognosis in 88%. Final satisfactory CNVII function (CNVII-SF, HB grades I-III) was achieved in 76% of the patients when excluding the anastomosis results, and 87% when including them. In recent years, the rate of CNVII-SF has risen to 94%. Non-serviceable hearing was preserved in 49% of the patients, on whom it was attempted. CONCLUSION: Considering the size of the tumors and extent of the resections, the preservation of CNVII function is currently very high. A close surveillance of CNVII function evolution following surgery is mandatory, as 2/3 of the patients discharged with deep paresis will need different face reanimation procedures. The preservation of useful hearing is still problematic, especially in patients with large tumors.

Benign versus atypical meningiomas: risk factors predicting recurrence.

OBJECTIVE: The aim of the study is to determine which clinic, radiologic, and surgical characteristics of benign and atypical meningioma are associated with tumor progression. METHODS: 335 patients who underwent gross-total resection of intracranial benign and atypical meningiomas between 2000 and 2009 were followed during the period of at least 3 years. Clinical, radiological and surgical features possibly associated with progression-free survival and influencing tumor recurrence were assessed. RESULTS: 291 lesions were benign (WHO Grade I) and 44 were atypical (WHO Grade II). In the median follow-up period of 82 months 34 meningiomas recurred. The 3-, 5- and 10-year progression-free survival (PFS) rates for benign and atypical tumors were 99.7 and 81.4%, 97.5 and 69.7%, 87.5 and 69.7%, respectively. In a Kaplan-Meier analysis subpial plane of surgical dissection (pial invasion) was associated with increased tumor progression both in benign (p=0.0084) and atypical cohort (p=0.0104), and bone involvement (p=0.0033) and peritumoral brain edema (p=0.0073) were associated with increased tumor progression only in atypical meningiomas. In a multivariate analysis pial invasion and WHO Grade II type were significantly associated with tumor recurrence. All recurrences in atypical meningioma group occurred within 4 years of the surgical resection. CONCLUSION: Pial invasion is an important predictor of tumor recurrence in benign and atypical meningiomas. In atypical meningiomas bone involvement and large peritumoral brain edema are associated with increased tumor progression.

Strategy for the surgical treatment of vestibular schwannomas in patients with neurofibromatosis type 2.

OBJECTIVE: Guidelines for appropriate management of vestibular schwannomas in NF2 patients are controversial. In this paper we reviewed our experience with patients with NF2 for the results of surgical treatment with particular reference to hearing and facial nerve preservation. METHODS: We included in the study 30 patients (16 women and 14 men) with the diagnosis of NF2 treated in our department between 1998 and 2014 who underwent surgery for vestibular schwannoma removal with a follow-up for at least 1 year. In 3 cases, the vestibular schwannomas were unilateral. Six patients with bilateral vestibular schwannomas underwent unilateral procedure. Therefore, 51 acoustic tumors were studied in 30 patients. RESULTS: No operative death we noted. Significant deterioration to the non-functional level occurred in 19 out of 22 cases with well-preserved preoperative hearing. Only three ears maintained their preoperative good hearing. Hearing was preserved in cases of small schwannoma not exceeding 2 cm. Among 21 patients who underwent bilateral operations hearing was preserved in 3 out of 7 cases when smaller tumor or better hearing level side was attempted at first surgery. In contrary none of the 14 patients retained hearing when the first operation concerned the worse-hearing ear. Among 14 tumors up to 2 cm there was only one case of moderately severe facial nerve dysfunction (House-Brackmann Grade IV) in the long follow-up. CONCLUSION: Early surgical intervention for vestibular schwannoma in NF2 patient is a viable management strategy to maintain hearing function and preserve facial nerve function.

Surgical treatment of parasagittal and falcine meningiomas invading the superior sagittal sinus.

OBJECTIVE: We present our experience with surgery of parasagittal and falcine meningiomas invading the superior sagittal sinus with special consideration of the surgical complications and the incidence of tumour recurrence. MATERIALS AND METHODS: The analysis included 37 patients with parasagittal and falcine meningiomas invading the superior sagittal sinus. In 13 cases, the sinus was ligated and resected with tumour. In 14 cases, the sinus was entered with the goal of tumour resection and the sinus was reconstructed, while in 10 patients the sinus was not entered and the remaining residual tumour was observed for growth. RESULTS: Out of 13 patients after radical resection of the tumour and invaded part of sinus, 9 revealed haemodynamic complications: venous infarction (4), significant brain oedema (3) and hypoperfusion syndrome (2). 2 out of 14 patients after resection of the tumour from the lumen of the superior sagittal sinus with subsequent sinus repair developed venous infarction after surgery. Among 27 patients after radical tumour excision the remote follow-up revealed recurrence in 2 patients. There were no significant haemodynamic complications in none of 10 cases, in which the residual tumour was left after surgery in the superior sagittal sinus. In this group, 3 cases were subjected to early post-operative radiotherapy and local recurrence was observed in 4 patients. CONCLUSIONS: The aggressive surgical treatment of meningiomas infiltrating the superior sagittal sinus is associated with a high surgical risk. The incidence of recurrence of these tumours increases significantly in the case of non-radical excision of the tumour.

Surgical treatment of jugular foramen schwannomas.

OBJECTIVE: We present our experience with surgery of jugular foramen schwannomas with special consideration of clinical presentation, surgical technique, complications, and outcomes. METHODS: This retrospective study includes ten patients with jugular foramen schwannomas treated by the senior author between January 2007 and December 2012. Three patients had undergone partial tumour resection elsewhere. The initial symptom for which they sought medical help was hearing loss, dysphagia, hoarseness, and shoulder weakness. Preoperative glossopharyngeal and vagal nerve deficits were the most common signs. In our series, tumour extension was classified according to Kaye-Pellet grading system. In two cases the tumours were classified into type A and 8 patients presented with type D tumours. A retromastoid suboccipital craniotomy was performed for type A tumours and modifications of cranio-cervical approach were suitable for type D. RESULTS: No death occurred in this series. Four patients deteriorated after surgery: in two patients preoperative cranial nerve deficits deteriorated after surgery while new cranial nerve palsy occurred in 2 other patients. In four patients, the cranial nerve dysfunction had improved at the last follow-up examination. In all other patients, the cranial nerve dysfunction remained the same. One patient experienced tumour recurrence over a follow-up period of 40 months. This patient underwent a successful second surgery without further evidence of tumour growth. CONCLUSIONS: Jugular foramen schwannomas can be radically managed with the use of skull base surgery techniques. However, the surgical treatment of jugular foramen schwannomas carries a significant risk of the lower CN deficits.