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This work aims to prospectively record all primary central nervous system tumors (PCNST), in France, for which histological diagnosis is available. The objectives are to create a national registry and a network to perform epidemiological studies, to implement clinical and basic research protocols, and to harmonize the healthcare of patients affected by PCNST. Following a feasibility study, including an estimate of the gross incidence of PCNST (15.8/100,000 person-years) in France, all French neuropathology and neurosurgery departments decided to participate in the program. For each patient, the neurosurgeon and the neuropathologist complete a data file containing socio-demographic, clinical, radiologic and anatomopathologic information. The Tumor Registry from Herault is authorized to compile the data files with personal identifiers. In 2.5 years, 10,093 cases of newly diagnosed PCNST have been recorded. Tumor resections were performed in 75.3%, while biopsies accounted for 24.7%. Histological diagnoses included glioma (49.6%), other neuroepithelial tumors (3.8%), meningioma (30.9%), neurinoma (8.7%), lymphoma (2.9%) and others (4.1%). Cryopreservation was reported for 2,261 PCNST specimens. Clinical and radiological aspects were also recorded. Preliminary results are encouraging and stimulating for the long-term goal of creating a National Registry and a National Network for patients affected by PCNST. To our knowledge, this is the first European databank dedicated to PCNST, with collection of clinical, radiological and histological data (including cryopreservation of the specimen). The creation of this registry and this database may have major clinical and fundamental implications.

Although advances in diffusion tensor imaging have enabled us to better study the anatomy of the inferior longitudinal fasciculus (ILF), its function remains poorly understood. Recently, it was suggested that the subcortical network subserving the language semantics could be constituted, in parallel with the inferior occipitofrontal fasciculus, by the left ILF, joining the posterior occipitotemporal regions to the temporal pole, then relayed by the uncinate fasciculus connecting the anterior temporal pole to the frontobasal areas. Nevertheless, this hypothesis was solely based on neurofunctional imaging, allowing a cortical mapping but with no anatomofunctional information regarding the white matter. Here, we report a series of 12 patients operated on under local anaesthesia for a cerebral low-grade glioma located within the left temporal lobe. Before and during resection, we used the method of intraoperative direct electrostimulation, enabling us to perform accurate and reliable anatomofunctional correlations both at cortical and subcortical levels. In order to map the ILF. Using postoperative MRI, we correlated these functional findings with the anatomical locations of the sites where language disturbances were elicited by stimulations, both at cortical and subcortical levels. Our goal was to study the potential existence of parallel and distributed language networks crossing the left dominant temporal lobe, subserved by distinct subcortical pathways--namely the inferior occipitofrontal fasciculus and the ILF. Intraoperative stimulation of the anterior and middle temporal cortex elicited anomia in four patients. At the subcortical level, semantic paraphasia were induced in seven patients during stimulation of the inferior occipitofrontal fasciculus, and phonological paraphasia was generated in seven patients by stimulating the arcuate fasciculus. Interestingly, subcortical stimulation never elicited any language disturbances when performed at the level of the ILF. In addition, following a transient postoperative language deficit, all patients recovered, despite the resection of at least one part of the ILF, as confirmed by control MRI. On the basis of these results, we suggest that the "semantic ventral stream" could be constituted by at least two parallel pathways within the left dominant temporal lobe: (i) a direct pathway, the inferior occipitofrontal fasciculus, that connects the posterior temporal areas and the orbitofrontal region, crucial for language semantic processing, since it elicits semantic paraphasia when stimulated; (ii) and also possibly an indirect pathway subserved by the ILF, not indispensable for language, since it can be compensated both during stimulation and after resection.

Object: While scarcely reported in low-grade glioma (LGG), accurate assessment of higher functions is essential to evaluate then preserve the quality of life. We assessed verbal working memory (vWM) in patients with LGG in language areas, before and after surgery, to evaluate the effect of glioma and resection on cognition, respectively., Methods: About 23 patients harboring a LGG in language areas underwent awake surgery. All patients had a vWM assessment, before and immediately after the resection, in addition to KPS. vWM was also evaluated 3 months after surgery in eight patients (KPS in all cases), who performed postoperative rehabilitation., Results: Preoperatively, 91% of patients had vWM disorders (KPS > or =90 in 22 patients). Immediately after surgery, 96% of patients had vWM worsening (50% of KPS > or =90). At 3 months, among the eight patients examinated, five recovered their preoperative vWM score, and three significantly improved it (KPS > or =90 in 23 patients)., Conclusion: In LGG, neuropsychological assessment is encouraged in addition to KPS. vWM evaluation before treatment showed that most patients had a cognitive deficit. Moreover, surgery induced a transient vWM worsening, which nevertheless recovers within 3 months. Specific rehabilitation might help to recover and even to improve the preoperative cognitive status.

While surgery is proned in low-grade glioma (LGG), the invasion of functional areas frequently prevents a complete resection. We report the first case of a patient operated on for a left frontal LGG, diagnosed because of seizures, with partial resection due to an invasion of the controlateral hemisphere. Chemotherapy enabled a regression of this controlateral extension. Postchemotherapy surgery performed with intraoperative functional mapping then allowed a complete resection, without sequelae. The patient has a normal socio-professional life, with no seizure. No other treatment was given. There was no recurrence, with a follow-up of 2 years since the second surgery (3.5 years since the first symptom). We propose a new therapeutic strategy in unresectable LGG, with preoperative chemotherapy, to make a radical surgery possible in a second step, while preserving the quality of life.

Object: Low grade gliomas are both proliferative and diffusive tumors, as recently modelized. When proliferation is predominant, the tumor is rather bulky and its main locations are the supplementary motor area and the paralimbic system. Diffusion occurs preferentially along white matter tracts. Recent anatomo-functional studies, performed both in vitro and in vivo, have described the fiber tracts centered around the insula. We thus propose to analyze the extension of paralimbic low grade gliomas in terms of invaded subcortical pathways., Methods: We retrospectively reviewed the MRIs of patients followed for a WHO grade II glioma at the Salpêtrière Hospital between 1991 and 2003. We selected patients with tumors centered on the insula and extending in temporal and frontal lobes (Type 2b-2c-3 of Yasargil's classification). We then analyzed on FLAIR sequences the extension (tracked on successive examinations before any treatment) along two main fasciculi in that area: the uncinate and arcuate fasciculi., Results: A total of 40 patients fulfilled the inclusion criteria. The uncinate fasiculus was invaded in 28 cases, the arcuate fasciculus in 9 cases, and both fasciculi in 3 cases. Longitudinal follow-up was available in 16 cases, and confirmed the preferential extension along these fasciculi., Conclusion: This kinetic analysis of extension of paralimbic low grade gliomas confirms that these tumors spread along distinct subcortical fasciculi. Due to the functional role of these pathways, this classification could be useful to elaborate therapeutic strategy (prognosis index, pre- and intra-operative neuropsychological testing, functional outcome).

Despite a better understanding of the organization of the cortical network underlying the semantic system, very few data are currently available regarding its anatomo-functional connectivity. Here, we report on a series of 17 patients operated on under local anaesthesia for a cerebral low-grade glioma located within the dominant hemisphere. Prior to and during resection, intraoperative electrical stimulation was used to map sensorimotor and language structures so that permanent neurological deficits could be avoided. In a number of cases, cortical and subcortical stimulation caused semantic paraphasias. Using postoperative MRI, we correlated these functional findings with the anatomical locations of the sites where semantic errors were elicited by stimulation, especially at the subcortical level, with the aim of studying the connectivity underlying the semantic system. In temporal gliomas, cortical sites involved in semantic processing were found around the posterior part of the superior temporal sulcus, with subcortical pathways reproducibly located under the depth of this sulcus. In insular gliomas, although stimulation elicited no semantic disturbances at the cortical level, such semantic paraphasias were generated at the level of the anterior floor of the external capsule. In frontal tumours, cortical regions implicated in semantics were detected in the lateral orbitofrontal region and dorsolateral prefrontal cortex, with subcortical fibres located under the inferior frontal sulcus. All these eloquent structures were systematically preserved, thereby avoiding permanent postoperative deficits. Our results provide arguments in favour of the existence of a main ventral subcortical pathway underlying the semantic system, within the dominant hemisphere, joining the two essential cortical epicentres of this network: the posterior and superior temporal areas, and the orbitofrontal and dorsolateral prefontal regions. Such a ventral stream might anatomically partly correspond to the inferior fronto-occipital fasciculus.

Background and Purpose: Hemangioblastomas (HBs) are rare lesions accounting for 1-5% of all spinal cord tumors. Seventy-five percent of spinal HBs are intramedullary. Lesions of the conus medullaris and the cauda equina are uncommon, and the filum terminale location is very rare. HBs of the lower spinal region are highly vascular tumors requiring surgery that is potentially complicated by excessive bleeding. In the literature, there are few reports concerning preoperative embolization of HBs, and only few cases are reported in spinal location. Presurgical embolization of HBs located in the lower spinal region has not been described. Although lower spinal HBs are predominantly fed by the anterior spinal artery (ASA), embolization of these lesions is possible and can reduce tumor vascular supply, thus facilitating surgery. We report our experience in four rare cases of solitary HBs occurring in the lower spinal region., Methods: Clinical charts and radiologic studies of four patients with a preoperatively embolized HB of the lower spinal region were retrospectively reviewed. The lesions were located in the conus medullaris in one case, at the level of the cauda equina in another, and in the filum terminale in two. In the conus medullaris case, the neoplasm was associated with a syrinx. In three patients, the HB was sporadic, while the patient with the HB of the cauda equina had Von Hippel-Lindau disease. Devascularization of the tumor was performed through the ASA in all cases and also through the posterior spinal artery in one by using non reabsorbable calibrated microspheres in three cases and polyvinyl alcohol particles in the other one., Results: Embolization caused no permanent complications, although one patient with a cauda equina HB mildly worsened after the endovascular procedure but recovered before surgery. At surgery, the tumor was completely removed in all cases. Blood loss was reported to be less than usually observed (100, 200, 200, and 400 mL). In addition, manipulation and removal of the tumor was reported to be easy in three of four tumors. Histologic examination confirmed the diagnosis. At 1-year postsurgical follow-up, two patient recovered completely from neurologic deficits, and two showed significant recovery. No tumor recurred during a follow-up period of 1-6 years (mean, 3.5 years)., Conclusion: Our results indicate that preoperative embolization of HBs of the lower spinal region is an useful procedure in aiding surgical resection of these highly vascular tumors. With a meticulous technique, embolization can be performed through the ASA.

Objective: This study was a randomized, multicenter Phase II trial comparing the effect of perioperative implantation of 5-fluorouracil-releasing microspheres followed by early radiotherapy (Arm A) and early radiotherapy alone (Arm B) in patients with gross total resection of high-grade glioma., Methods: Patients were randomized on clinical and radiological assumption of supratentorial high-grade glioma. All patients underwent surgery, and after resection and histological confirmation, patients randomized to Arm A received multiple injections of microsphere suspension (130 mg of 5-fluorouracil). Conventional fractionated radiotherapy (59.4 Gy) was initiated between the second and the seventh day after surgery for both arms., Results: A total of 95 patients were randomized. Seventy-seven patients were treated and analyzed in intention to treat for efficacy and safety. Overall survival was 15.2 months in Arm A and 13.5 months in Arm B. In the subpopulation of patients with complete resection, overall survival was 15.2 months in Arm A versus 12.3 months in Arm B. However, these differences were not significant. Safety was acceptable with prophylactic high doses of corticosteroids., Conclusion: It may be hypothesized that the implantation of 5-fluorouracil-loaded microspheres in the wall of the cavity resection did increase the overall survival, but the present study was not designed and sufficiently powered to demonstrate this.

Context: The "Standards, Options and Recommendations" (SOR) project, which started in 1993, is a collaboration between the Federation of French Cancer Centers (FNCLCC), the 20 French Regional Cancer Centers, and specialists from French public universities, general hospitals and private clinics. The main objective is the development of clinical practice guidelines to improve the quality of health care and the outcome of cancer patients., Objective: To define clinical practice guidelines for the management of adult patients with intracranial glioma in collaboration with the Association of French-speaking Neuro-oncologists (Anocef) and the French society of neurosurgeons (SNCLF). These recommendations cover diagnosis, classification, treatment and follow-up of patients with these tumors., Method: The methodology is based on a literature review and critical appraisal by a multidisciplinary group of experts who define the CPGs according to the definitions of the Standards, Options and Recommendations project. Once the guidelines has been defined, the document is submitted for review by independent reviewers., Results: This article is a summary version of the full document presenting the clinical practice guidelines with algorithms. The main recommendations concern the place of the surgery, radiotherapy, chemiotherapy, imagery and concomitant medical treatments in the specific treatment strategy of grade III and IV glioma, grade II glioma, gliomatosis cerebri, pilocytic astrocytoma, subependymoma, xanthoastrocytoma, intracranial ependymoma and brain stem glioma.

Object: Despite the high frequency of striatal lesions, the rate of movement disorders reported in the literature is lower than expected (< 10%). To maximize the extent of resection in low-grade gliomas invading the right striatum, the authors performed a striatal resection in a series of 14 patients, observed the lack of movement disorders following these procedures, and discuss herein the mechanisms likely to explain these findings., Methods: Fourteen patients harboring a low-grade glioma that was infiltrating the right nondominant striatum, and in whom the results of neurological examination were normal, underwent surgery in which intraoperative electrical mapping was used, allowing the identification of pyramidal pathways. The striatum was resected in all procedures, and corticospinal tracts were systematically detected and preserved. Ten patients presented with a transient postoperative motor deficit, and nine with a loss of interest and affect. These symptoms all resolved within 3 months, except for one case of persistent hemiparesis. No postoperative movement disorder was noted, even transitorily. All resections were categorized as either total or subtotal on control magnetic resonance images., Conclusions: These findings show that the nondominant striatum can be removed in cases of glioma invasion without inducing even transitory movement disorders. This phenomenon could be explained by the combined resection of the two classes of striatal neurons, an associated pallidal and thalamocortical resection, or a compensatory recruitment of parallel networks. Thus, these results may allow the surgeon to maximize the extent of removal of low-grade gliomas involving basal ganglia. Striatal resection may induce transient hemiparesis and "athymhormic syndrome," however, necessitating that the patient be clearly informed before surgery.