Clinical characteristics and long-term surgical outcome of spinal myxopapillary ependymoma: a French cohort of 101 patients.

PURPOSE: Myxopapillary ependymoma (MPE) is the most frequent tumor affecting the medullary conus. The surgical therapeutic management is still debated and only few studies have focused on the postoperative clinical outcome of patients. This study aimed to demonstrate long-term postoperative outcome and to assess the predictive factors of recurrence as well as the clinical evolution of these patients. METHODS: From 1984 to 2019, in four French centers, 101 adult patients diagnosed with MPE were retrospectively included. RESULTS: Median age at surgery was 39 years. Median tumor size was 50 mm and lesions were multifocal in 13% of patients. All patients benefited from surgery and one patient received postoperative radiotherapy. Gross total resection was obtained in 75% of cases. Sixteen percent of patients presented recurrence after a median follow-up of 70 months. Progression free survival at 5 and 10 years were respectively estimated at 83% and 79%. After multivariable analysis, sacral localization, and subtotal resection were shown to be independently associated with tumor recurrence. 85% of the patients had a favorable evolution concerning pain. 12% of the patients presented a postoperative deterioration of sphincter function and 4% of motor function. CONCLUSION: Surgery alone is an acceptable option for MPE patients. Patients with sacral location or incomplete resection are at high risk of recurrence and should be carefully monitored.

Cerebellar high-grade gliomas do not present the same molecular alterations as supratentorial high-grade gliomas and may show histone H3 gene mutations.

Numerous molecular alterations have been described in supratentorial high-grade gliomas (1p19q co-deletion, IDH1/2, histone H3, hTERT promotor mutations, loss of ATRX) which have led to a new histomolecular classification of diffuse gliomas. We aimed at describing these alterations in a series of 19 adults with pure cerebellar high-grade gliomas. Systematic immunohistochemical analyses, including that of IDH1R132H, ATRX, p53, PTEN, EGFR, p16, FGFR3, BRAFV600E, mismatch repair proteins, H3K27me3, H3K36me3, and H3K27M; molecular analyses of IDH1/2, hTERT, BRAF, H3F3A, and HIST1H3B mutation hotspots; and EGFR, PTEN FISH were retrospectively performed in a multicentric study. We histopathologically identified 14 glioblastomas, 4 grade III astrocytomas and 1 gliosarcoma. Two cases showed a H3F3A K27M mutation. Only one case harbored a classical profile of glioblastoma with hTERT mutation, EGFR gain and 10q loss. The most frequent alteration was the absence of p16 immunoexpression. We report a histomolecular analysis of pure cerebellar high grade gliomas. The histomolecular profile appears to be different from that of supratentorial gliomas, with no IDH1/2 gene mutations and only 1 case with a classic profile of de novo glioblastoma. In 2 cases, we identified H3F3A K27M mutation, classically described in pediatric midline gliomas.
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Predictive factors of neurological recovery after chronic craniovertebral brainstem compression.

BACKGROUND: Neurologic manifestations of craniovertebral junction (CVJ) disease may generate dramatic brainstem damage, which could evolve to paralysis. In most cases, patients are referred with advanced neurological symptoms such as tetraplegia/paresis. The aim of this study was to identify predictive factors of favorable neurological evolution after non-traumatic brainstem compression. METHODS: A prospective study evaluated 143 consecutive patients who had undergone CVJ anterior brainstem decompression. The mean age was 45.1 ± 19.1 years. The study analyzed clinical, surgical, and imagery characters to determine predictive factors of neurological improvement. RESULTS: The mean follow-up of our series was 10.2 years (range 0.5-23.9). Seventy-one (49.6%) presented initial tetrapalsies resulting from spinal cord compression. Multivariable analysis revealed that Frankel score [odds ratio (OR) 5.7, CI 95% 1.01-31.8; p < 0.04] and preoperative symptoms < 6 months [OR 0.33, CI 95% 0.125-0.9; p < 0.025] were independently associated with partial neurological improvement, while the only independent factor associated with total neurologic recovery was the preoperative symptom evolution <6 months [odd ratio (OR) 4.3, CI 95% 1.6-11.4; p < 0.003]. None of the following were identified as predictive factors: demographic characteristics, medical history, the etiology of compression, or initial spinal cord MRI. CONCLUSION: The earlier the decompression is performed, the better the neurological improvement. Whatever the initial Frankel score, if neurological palsy or disorders evolved for less than 6 months, complete recovery is possible.

A Staged Approach for Surgical Management of Basilar Invagination.

BACKGROUND AND OBJECTIVES: Patients with basilar invagination (BI) can be treated with several surgical options, ranging from simple posterior decompression to circumferential decompression and fusion. Here, we aimed at examining the indications and outcomes associated with these surgical strategies to devise a staged algorithm for managing BI. METHODS: We conducted a retrospective cohort study in 2 neurosurgical centers and included patients with a BI, as defined by a position of the dens tip at least 5 mm above the Chamberlain line. Other craniovertebral junction anomalies, such as atlas assimilation, platybasia, and Chiari malformations, were documented. C1-C2 stability was assessed with a dynamic computed tomography scan. RESULTS: We included 30 patients with BI with a mean follow-up of 56 months (min = 12, max = 166). Posterior decompression and fusion (n = 8) was only performed in cases of obvious atlanto-axial instability (eg, increased atlanto-dental interval or hypermobility on flexion/extension), while anterior decompression (transoral or transnasal) was reserved to patients with lower cranial nerves deficits (eg, swallowing dysfunction) and irreducible anterior compression (n = 9). Patients with posterior signs (eg, Valsalva headaches) or myelopathy but without C1-C2 instability nor anterior signs were managed with an isolated foramen magnum decompression, with or without duraplasty (n = 13). Complications were more frequent for combined procedures, including neurological deterioriation (n = 4) and tracheostomy (n = 2), but reinterventions were more likely in patients undergoing posterior decompression alone (n = 3). CONCLUSION: Patient selection is key to determine the appropriate surgical strategy for BI: In our experience, combined approaches are only needed for patients with irreducible and symptomatic anterior compression, while fusion should be restricted to patient with obvious signs of atlanto-axial instability. Other BI patients can be managed by foramen magnum decompression alone to minimize surgical morbidity.

Efficacy and Safety of Proton Beam Therapy for Primary Optic Nerve Sheath Meningioma.

PURPOSE: Management of optic nerve sheath meningiomas (ONSM) remains challenging. Photon radiation therapy (PhRT) is the most common treatment for sight-threatening ONSM. Proton beam therapy (PBT) is less commonly used because it is more expensive and because there are questions about its efficacy specifically in relation to ONSM. PBT has the theoretical advantage of reducing radiation exposure to adjacent structures. We report the visual outcome of patients with primary ONSM managed at the Fondation Ophtalmologique Adolphe de Rothschild, Paris, France, and treated with PBT at the Centre de Protonthérapie, Institut Curie, Orsay, France. METHODS: We conducted a retrospective review of all patients with primary ONSM who received PBT (either by itself or following surgery) between January 2006 and January 2019. Neuro-ophthalmic examinations were performed at presentation and after radiotherapy, and, when applicable, after surgery. Meningiomas were measured at the time of diagnosis and at each follow-up MRI examination. RESULTS: Sixty patients (50 women, 10 men; mean age, 45.2±11.1y) were included, of whom 29 underwent surgery. At presentation, 52 (87%) of them had decreased vision (average visual acuity: 0.6 logMAR). Fundus examination showed optic disc swelling (n=27; 46.5%), optic disc pallor (n=22; 37.9%), optic disc cupping (n=2; 3.4%), opto-ciliary shunt (n=8; 13.8%), or choroidal folds (n=5; 8.6%). Otherwise, it was unremarkable (n=7; 12.1%). After treatment, visual function was stable overall. Fundus examination showed pallor (n=47; 83.9%), swelling (n=3; 5.4%), or cupping (n=2; 3.4%) of the optic disc, or was unremarkable (n=5; 8.9%). The visual field of 8 patients worsened, while 3 developed asymptomatic retinal hemorrhages. Tumor shrunk significantly in 8 patients at 1 year after PBT and remained stable in size in all others. Patients with opto-ciliary shunts had significantly worse visual outcome than other patients. Retinal abnormalities were observed in 11 patients during follow-up. CONCLUSION: PBT alone or in association with surgery appears to be a safe and efficient treatment for ONSM, reducing the tumor size and stabilizing visual function. The risk of developing radiation retinopathy seems to be higher when patients had upfront surgery.

Microsurgical Transcranial Approach of 112 Paraoptic Meningiomas: A Single-Center Case Series.

BACKGROUND: Predictors of visual outcomes after optic nerve decompression are controversial. OBJECTIVE: To identify the predictors of poor visual outcomes after surgery of meningiomas responsible of a compressive optic neuropathy. METHODS: We focused on paraoptic meningiomas (POMs), which gathered tuberculum sellae meningiomas (TSMs) and anterior clinoid meningiomas (ACMs) responsible for visual impairment or threatening visual function, that underwent surgery at our institution between January 2009 and December 2015 and analyzed the clinical and radiological findings of our patients. RESULTS: Among 112 patients who underwent surgery for a POM, a preoperative visual deficit was present in 108 patients (96.4%). Six months after surgery, 79 patients (70.5%) had a visual improvement, 15 patients (13.4%) had an unchanged vision, and 18 patients (16.1%) had deteriorated vision. A preoperative visual deficit of 6 mo or more was a strong predictor of poor visual outcome after surgery (P = .034). Poor visual outcome after surgery was not significantly related to the size of the tumor (P = .057), the age of the patient (P = .94), or the tumor extension into the optic canal (P = .47). CONCLUSION: The duration of preoperative visual deficit was found to be a strong predictor of poor visual outcomes after surgery in POMs Other predictors of poor visual outcomes are still needed and are currently under evaluation in a prospective study at our institution.

French Spine Surgery Society guidelines for management of spinal surgeries during COVID-19 pandemic.

Since the outbreak of coronavirus disease 2019 (COVID-19) in December 2019 in China, various measures have been adopted in order to attenuate the impact of the virus on the population. With regard to spine surgery, French physicians are devoted to take place in the national plan against COVID-19, the French Spine Surgery Society therefore decided to elaborate specific guidelines for management of spinal disorders during COVID-19 pandemic in order to prioritize management of patients. A three levels stratification was elaborated with Level I: Urgent surgical indications, Level II: Surgical indications associated to a potential loss of chance for the patient and Level III: Non-urgent surgical indications. We also report French experience in a COVID-19 cluster region illustrated by two clinical cases. We hope that the guidelines formulated by the French Spine Surgery Society and the experience of spine surgeons from a cluster region will be helpful in order optimizing the management of patients with urgent spinal conditions during the pandemic.

Conservative management of 386 cases of unilateral vestibular schwannoma: tumor growth and consequences for treatment.

OBJECT: The object of this study was to evaluate the natural history, pattern, and occurrence of tumor growth and its consequences for treatment of small-sized vestibular schwannomas (VSs). METHODS: From 1990 to 2005, 386 patients underwent conservative management for VS because of the following: age > 60 years, poor health/medical risks, risk of deterioration of good hearing, small tumor size, minimal or no incapacitating symptoms, and/or patient preference. Tumor size was measured by MR imaging according to the guidelines of the Committee on Hearing and Equilibrium. The first MR imaging study was performed 1 year after diagnosis, and subsequent imaging was performed yearly or every 2 years depending on the appearance of new symptoms, tumor growth, or both. RESULTS: Sixty-one patients were lost to follow-up the first year after presentation. Of the 325 patients for whom 1-year follow-up data were available, 39 showed tumor growth > or = 3 mm. Conservative management was discontinued for these 39 patients. The patients who returned for follow-up were evaluated at 1- or 2-year intervals depending on tumor growth. The authors extrapolated to obtain data for 2-year intervals, yielding data for 160, 56, 21, and 8 patients at 3, 5, 7, and 9 years after initial presentation, respectively. The overall mean tumor growth rate (+/-standard deviation) was 1.15 +/- 2.4 mm/year. This rate was estimated by pooling all values of tumor growth that had been determined for all patients and dividing by the total number of "events," with each assessment constituting an event. In 58.6% of patients, the annual tumor growth rate was < 1 mm/year; in 29.2%, 1-3 mm/year; and in 12.2%, > or = 3 mm/ year. The growth rates of intrameatal (1.02 +/- 1.8 mm/year) and extrameatal (1.40 +/- 3.1 mm/year) tumors did not differ significantly. No significant association was found between tumor growth rate and sex, age, initial hearing status, or initial tumor grade. Delay in diagnosis was the only significant factor associated with tumor growth rate. During follow-up, conservative management was discontinued for 77 (23.7%) of the 325 patients for whom at least 12-month follow-up data were available; surgery was performed in 60 (77.9%) and radiation therapy in 17 (22.1%). CONCLUSIONS: The results of this study support the role of a conservative "wait-and-scan" policy of management for small-sized VSs because most have a slow growth rate. Long-term neuroimaging follow-up is needed even with non-growing tumors.

Locally aggressive monostotic fibrous dysplasia of the cervical spine mimicking malignancy: a case report and literature review.

We report the case of a 30-year-old woman with histologically proven monostotic fibrous dysplasia of C2 revealed by a pathological fracture of the odontoid process. Radiological investigations showed a ground-glass mineralization of the vertebral body, a centimetric lytic area with poorly defined margins involving the inferior part of the vertebral body and inferior endplate and a fracture through an osteolytic area in the base of the odontoid process. Owing to the vertebral instability, a surgical procedure combining C0-C5 fixation and posterior bone grafting was performed. The surgical biopsy was inconclusive and pathological confirmation was finally obtained through a percutaneous needle biopsy under fluoroscopic guidance. At 26-month follow-up, the patient still experienced mild persistent cervical posterior neck pain and stiffness possibly related to a C5-6 laxity below the intervertebral fixation. This case combines three radiological findings, which are unusual in fibrous dysplasia: monostotic presentation involving the spine, some aggressive radiographic features, and a pathological fracture.

Craniovertebral Junction Transoral Approach: Predictive Factors of Complications.

BACKGROUND: The transoral approach provides the most direct exposure to extradural lesions of the ventral craniovertebral junction. The morbidity and mortality from this approach greatly limits its use because they are still feared and debated. Using univariable and multivariable logistic regression analyses, this study aims to identify the factors associated with short-term complications in patients undergoing the transoral approach. METHODS: A consecutive cohort analysis of prospectively collected data in several neurosurgery spine departments evaluated 143 consecutive patients who had undergone craniovertebral junction transoral approach surgery. The mean age at the time of surgery was 45.1 ± 19.1 years. The study analyzed the comorbidities, the operative procedure, and postoperative morbidity and mortality. RESULTS: Seventeen patients (11.9%) had a postoperative complication in the first month. In our univariable analysis: age, smoking, tumor etiology, preoperative posterior fixation, posterior bone graft, preoperative external lumbar shunt, and the transoral approach pathway were significantly associated with postoperative mortality and morbidity. In our multivariable analyses, preoperative external lumbar shunt was significantly associated with complication risks (odds ratio [OR] 6.7; 95% confidence interval [CI] 2.1-21.7, P < 0.001), whereas preoperative posterior fixation (OR 0.28; 95% CI 0.08-1.1, P < 0.04) and posterior bone graft (OR 0.14; 95% CI 0.03-0.6, P < 0.008) were significantly associated with lower complication risks. CONCLUSIONS: To reduce complications, it is essential to be aware of and to manage these preoperative risk factors. In such manner, we insinuate that postoperative complications depend on the surgeon's familiarity with the transoral approach.

Loss of SMARCE1 expression is a specific diagnostic marker of clear cell meningioma: a comprehensive immunophenotypical and molecular analysis.

Clear cell meningioma (CCM) is a rare grade II histopathological subtype that usually occurs in young patients and displays high recurrence rate. Germline SMARCE1 mutations have been described in hereditary forms of this disease and more recently in small syndromic and sporadic CCM series. The diagnostic value of SMARCE1 in distinguishing between CCM and other meningioma variants has not been yet established. The aim of our study was to investigate the status of SMARCE1 in a series of CCMs and its morphological mimickers. We compared the performance of an anti-SMARCE1 antibody and the molecular analysis of the SMARCE1 gene in a retrospective multicenter series of CCMs. All CCMs lossed SMARCE1 immunoexpression. Bi-allelic inactivating events were found by NGS-based sequencing in all of these cases, except for one, which was incompletely explored, but had a wild-type sequence. We then validated the anti-SMARCE1 antibody specificity by analyzing additional 305 pediatric and adult meningiomas of various subtypes and 15 non-meningioma clear cell tumors by SMARCE1 immunohistochemistry. A nuclear immunostaining was preserved in all other meningioma variants, as well as non-meningioma clear cell tumors. In conclusion, our series showed, for the first time, that SMARCE1 immunostaining is a highly sensitive biomarker for CCM, useful as a routine diagnostic biomarker.

Transoral robotic surgery for sellar tumors: first clinical study.

OBJECTIVE The aim of this study was to confirm the feasibility of an innovative transoral robotic surgery (TORS), using the da Vinci Surgical System, for patients with sellar tumors. This technique was designed to offer a new minimally invasive approach, without soft-palate splitting, that avoids the rhinological side effects of classic endonasal approaches. METHODS The authors performed a prospective study of TORS in patients with symptomatic sellar tumors. Specific anatomical features were required for inclusion in the study and were determined on the basis of preoperative open-mouth CT scans of the brain. The main outcome measure was sellar accessibility using the robot. Resection quality, mean operative time, postoperative changes in patients' vision, side effects, and complications were additionally reported. RESULTS Between February and May 2016, 4 patients (all female, mean age 49.5 years) underwent TORS for resection of sellar tumors as participants in this study. All patients presented with symptomatic visual deficits confirmed as bitemporal hemianopsia. All tumors had a suprasellar portion and a cystic part. In all 4 cases, the operation was performed via TORS, without the need for a second surgery. Sella turcica accessibility was satisfactory in all cases. In 3 cases, tumor resection was complete. The mean operative time was 2 hours 43 minutes. Three patients had a significant visual improvement at Day 1. No rhinological side effects or complications in patients occurred. No pathological examination was performed regarding the fluid component of the tumors. There was 1 postoperative delayed CSF leak and 1 case of transient diabetes insipidus. Side effects specific to TORS included minor sore throat, transient hypernasal speech, and 1 case of delayed otitis media. The mean length of hospital stay and mean follow up were 8.25 days and 82 days, respectively. CONCLUSIONS To our knowledge, this is the first report of the surgical treatment of sellar tumors by means of a minimally invasive TORS. This approach using the da Vinci Surgical System seems feasible and constitutes an innovative neurosurgical technique that may avoid the adverse side effects and technical disadvantages of the classic transsphenoidal route. Moreover, TORS allows an inferosuperior approach to the sella turcica, which is a key point, as the tumor is approached in the direction of its growth.

Poor Prognosis and Challenging Treatment of Optic Nerve Malignant Gliomas: Literature Review and Case Report Series.

BACKGROUND: Malignant optic glioma of adulthood is a rare, invasive neoplasm of the anterior visual pathway. In this article, the clinical features of a case series of 3 malignant optic nerve glioblastomas (World Health Organization grade IV) are presented, and the modalities of treatment and their associated survivals are discussed through a review of the existing literature to date. METHODS: A retrospective case series study was led for 3 patients diagnosed with primary optic nerve and chiasm glioblastoma, coming from 2 referral neurosurgical centers. An electronic search was conducted on MEDLINE via PUBMED, COCHRANE, from October 1973 to April 2016. Cohort, case reports, and case series were screened for investigating treatment and overall survival (OS) of malignant optic nerve gliomas. Pooled means and 95% confidence intervals of OS for each treatment were generated. RESULTS: From our retrospective case series, all patients had initial visual impairment (2 women and 1 man). The histologic diagnosis was done by biopsy. The patients' mean age was 67.3 years (standard deviation [SD] 18.5). The disease was rapidly lethal for all patients: median OS was 5 months (SD: 15.1). Two patients underwent chemotherapy by single cure of temozolomide, while the third one was treated with a radiochemotherapy protocol. Due to the fact that there is no gold standard treatment as first-choice treatment, a large heterogeneity in first-choice oncologic treatment is observed. However, we did not find any significant differences for OS between World Health Organization grade III and grade IV optic gliomas. CONCLUSION: Malignant optic glioma is a rare and fatal disease in adults. Despite the modalities of treatment, the treatment outcomes remain unsatisfactory. There is no significant difference in the median OS of patients with malignant optic nerve, as compared with those diagnosed with other supratentorial glioblastoma. Chemoradiotherapy with temozolomide currently remains the best treatment in terms of OS. Advances in the understanding of tumor biology have yet failed to translate into effective treatment regimens.

Posterior Fossa Craniectomy with Endovascular Therapy of Giant Fusiform Basilar Artery Aneurysms: A New Approach to Consider?

BACKGROUND: The prognosis of unruptured giant basilar artery (BA) aneurysms is very poor. No treatment has shown efficacy in survival. This pilot case-control study examines the overall survival (OS) benefit of combined surgical and endovascular management of giant BA aneurysms. METHODS: Combined treatment including posterior fossa craniectomy followed by endovascular treatment was performed in 3 patients with giant BA aneurysms. OS of the 3 patients was compared with a control group of 6 patients (ratio 1:2) treated with the endovascular procedure only. RESULTS: The mean survival time was 32.6 months in the craniectomy group (SD 9.01, 95% confidence interval [14.9, 50.3]) and 3.5 months in the control group (SD = 2.08, 95% confidence interval [0.001, 7.6]; Mantel-Cox test P < 0.04). At mean follow-up of 36.5 months (SD 10.2), 2 of 3 patients had a favorable outcome with a Glasgow Outcome Scale score of 5. Univariate analysis determined that women had a statistically higher OS than men (33.7 months vs. 3.058 months for men; log-rank test P = 0.011). A similar outcome was obtained in the presence of a circulating posterior communicating artery (P = 0.03) and in the presence of an endovascular right vertebral artery occlusion (P = 0.022). CONCLUSIONS: Our study suggests that preventive posterior fossa craniectomy increases significantly OS of patients with giant BA aneurysms.

Natural course and prognosis of anaplastic gangliogliomas: a multicenter retrospective study of 43 cases from the French Brain Tumor Database.

Background: Anaplastic gangliogliomas (GGGs) are rare tumors whose natural history is poorly documented. We aimed to define their clinical and imaging features and to identify prognostic factors. Methods: Consecutive cases of anaplastic GGGs in adults prospectively entered into the French Brain Tumor Database between March 2004 and April 2014 were screened. After diagnosis was confirmed by pathological review, clinical, imaging, therapeutic, and outcome data were collected retrospectively. Results: Forty-three patients with anaplastic GGG (median age, 49.4 y) from 18 centers were included. Presenting symptoms were neurological deficit (37.2%), epileptic seizure (37.2%), or increased intracranial pressure (25.6%). Typical imaging findings were unifocal location (94.7%), contrast enhancement (88.1%), central necrosis (43.2%), and mass effect (47.6%). Therapeutic strategy included surgical resection (95.3%), adjuvant radiochemotherapy (48.8%), or radiotherapy alone (27.9%). Median progression-free survival (PFS) and overall survival (OS) were 8.0 and 24.7 months, respectively. Three- and 5-year tumor recurrence rates were 69% and 100%, respectively. The 5-year survival rate was 24.9%. Considering unadjusted significant prognostic factors, tumor midline crossing and frontal location were associated with shorter OS. Temporal and parietal locations were associated with longer and shorter PFS, respectively. None of these factors remained statistically significant in multivariate analysis. Conclusions: We report a large series providing clinical, imaging, therapeutic, and prognostic features of adult patients treated for an intracerebral anaplastic GGG. Our results show that pathological diagnosis is difficult, that survivals are only slightly better than for glioblastomas, and that complete surgical resection followed with adjuvant chemoradiotherapy offers longer survival.

Suboccipital and cervical chordomas: the value of aggressive treatment at first presentation of the disease.

OBJECT: Chordoma is a locally invasive tumor with a high tendency for recurrence for which radical resection is generally recommended. To assess the benefits of aggressive treatment of chordomas, the authors compared results in patients treated aggressively at the first presentation of this disease with results in patients who were similarly treated, but after recurrence. METHODS: Among 36 patients with cervical chordomas who were treated at the authors' institution, 22 underwent primary aggressive treatment (Group A) and 14 were treated secondarily after tumor recurrence (Group B). Two cases were excluded from Group A because of unrelated early deaths and three from Group B because of insufficient pre- or postoperative data. Most tumors were located at the suboccipital level and only eight cases at a level below C-2. Radiotherapy and proton therapy were similarly conducted in both groups of patients. The actuarial survival rates were 80 and 65% at 5 and 10 years, respectively, in Group A patients and 50 and 0% at 5 and 10 years, respectively, in Group B patients (p = 0.049, log-rank test). The actuarial recurrence-free rates were 70 and 35% at 5 and 10 years, respectively, in Group A and 0% at 3 years in Group B (p < 0.0001, log-rank test). The numbers of recurrences per year were 0.15 in Group A and 0.62 in Group B (p > 0.05). All other parameters that were analyzed (patient age, delay before diagnosis, clinical symptoms, chondroid type of lesion, and histological features) did not prove to influence prognosis in a statistically significant manner. CONCLUSIONS: Aggressive therapy, combining as radical a resection as possible with radiotherapy, seems to improve the prognoses of suboccipital and cervical chordomas when applied at the patient's first presentation with the disease.

Transcranial approach for venous embolization of dural arteriovenous fistulas.

OBJECT: Transvenous embolization is effective in the treatment of an intracranial dural arteriovenous fistula (DAVF). Access to the fistula via the internal jugular vein (IJV) may be limited by associated dural sinus thrombosis; a transcranial approach has been developed for venous embolization in such a situation. The authors report their experiences with the use of a transcranial approach for venous embolization of DAVFs. METHODS: Ten patients with DAVFs underwent craniectomy and embolization procedures in which direct sinus puncture was performed. The DAVFs were located inside the dura mater that constituted the walls of the transverse sinus in five cases, the superior sagittal sinus in four cases, and the superior petrosal sinus in one case. All DAVFs drained directly into a sinus with secondary reflux into leptomeningeal veins. In all cases, the fistula could not be accessed from the IJVs. Craniectomy was performed in an operating room and, in seven cases, subsequent enlargement of the craniectomy was required. Sinus catheterization was performed after the patient had been transferred to the angiography room. The DAVFs were embolized using coils only in five patients, glue only in two patients, and both coils and glue in three patients. Angiographic confirmation that embolization of the fistula was successful was obtained in all cases. A transient complication occurred during the first case after sinus catheterization was attempted in the operating room. CONCLUSIONS: The transcranial approach allows straightforward access to DAVFs located on superficial dural sinuses that are inaccessible from the IJVs. The effectiveness of this approach is similar to that of the standard retrograde venous approach. The correct location and adequate extent of the craniectomy are essential for success to be achieved using this technique.