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Intraspinal tumors are uncommon. Positive diagnosis is based on medical imaging exams, in particular MRI. Anatomopathological examination allows for definitive diagnosis. Surgery is the treatment of choice for most of them. Prognosis is related to the histological type and patient´s initial clinical condition. We here report the histological profile and progression of intraspinal tumors in our department. We conducted a retrospective study over a period of 10 years. All operated patients with histology-confirmed intraspinal tumor (23 cases) were enrolled. Four unworkable records were excluded. The median time from symptom onset and first consultation was 79 days. Patients presented with spinal cord compression. In 11 cases this was characterized by slow onset. Seven CT scan and 14 MRI were performed, which showed 4 intramedullary tumors, 9 intradural, 1 extradural, and 5 of unknown site. Histological examination showed meningioma in 11 cases, neurinoma in 3 cases. In 7 cases, it confirmed the diagnosis based on Imaging tests (4 CT and 3 MRI). Macroscopically complete resection was performed in 14 cases; it was partial in 5 cases. After a 6-month follow-up period 6 patients had fully recovered, 9 partially. This study highlights diagnosis delays. MRI better defined the lesion, but its histological approach was limited. Meningiomas dominated. Complete resection was most often performed. The postoperative course was uneventful., Competing Interests: Les auteurs ne déclarent aucun conflit d'intérêts., (Copyright: Sylvain Denléwendé Zabsonre et al.)

BAF complexes are chromatin remodelling complexes made up of 15 subunits which overview transcription regulation. A subset of their subunits are notoriously linked to cancer, with the examples of SMARCB1, SMARCA4, ARID1A/1B and PBRM1. The complexes act as tumor suppressor genes, commonly mutated in a wide array of malignancies with an overrepresentation of sarcomas and tumors of the central nervous system. The recurrent inactivation of their genes points towards their driving role in the tumorigenesis of SMARCB1 in malignant rhabdoid tumors and SMARCA4 in small cell carcinoma of the ovary, hypercalcemic type. These tumors are morphologically similar composed of solid sheets of cells displaying vesicular nuclei dotted with clear chromatin and conspicuous nucleoli. Genomically, they share simple diploid profiles with no other alterations than in the culprit gene. Other mesenchymal tumors, distinct from malignant rhabdoid tumors are associated with BAF alterations, namely epithelioid sarcomas, SMARCA4-deficient thoracic sarcomas. BAF subunits are mostly inactivated through mutations or deletions but also occur through translocations in medullary carcinoma of the kidney and synovial sarcomas. Apart from tumors displaying recurrent alterations of the complexes, some variants or tumor variants display BAF alterations, including epithelioid malignant peripheral nerve sheet tumors and poorly differentiated chordomas. Lastly, some malignancies display low frequency of BAF alterations, in keeping with their passenger role in tumorigenesis with the example of dedifferentiated carcinomas, especially in colon, lung and uterus. BAF complexes alterations correlate with morphological features recognizable by microscopy, paving the way for their routine diagnosis and potential therapeutic prospects., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Introduction: PELICAN ("Partager Efficacement en Laboratoire les Informations des Comptes rendus ANatomopathologiques") is a software, which generates standardized reports, and allows to automatically create a database. It has been used in central nervous system tumor pathology at the University Hospital of Nancy since 2014. The purpose of this article was to illustrate the use of this application for meningiomas, with a first statistical evaluation., Materials and Methods: The export of data included all cases of meningiomas recorded in the PELICAN application until July 2018. The PELICAN application is a Microsoft Excel file containing a software, written in Visual Basic for Applications, and used by the pathologist to create the report. The main clinical data were collected from the Hérault Register census form. Follow-up was systematically reported for atypical meningiomas., Results: Two hundred and ninety-five meningiomas were analyzed, including 250 grade I meningiomas, 42 grade II meningiomas, and 3 grade III meningiomas. Grade II meningiomas were characterized by a significantly higher proportion of men (P=0.002) and dural infiltration (P<0.001), a significant increase in the Ki-67 index (P<0.0001), and a significant decrease in progesterone receptor expression (P<0.001). In atypical meningiomas, a Ki-67 index of more than 20 % was significantly correlated with a shorter progression-free survival (P=0.032)., Conclusion: The PELICAN software is an easy-to-use tool that allows to generate standardized reports and feed a database, opening very interesting perspectives from an epidemiological and scientific point of view., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Primary intrabone meningiomas are rare ectopic meningiomas accounting for 1- 2% of all meningiomas. This study aims to illustrate imaging characteristics of intrabone meningiomas contributing to diagnosis and to discuss their differential diagnoses. We report two cases of pterional intrabone meningiomas detected using CT scan and MRI.

Introduction: Meningiomas with orbital extension are the most frequent benign orbital tumors. The few studies available on the postoperative complications of their excision focus on the postoperative evolution of proptosis, visual acuity and visual field deficits. The goal of our study was to highlight the oculoplastic complications secondary to their excision., Material and Method: We identified all cases of meningiomas with orbital extension undergoing excision either neurosurgically or via an orbital approach in the ophthalmology and neurosurgery departments of Pasteur university medical center in Nice between February 2011 and January 2017. The data collection included the postoperative presence of proptosis, oculomotor disturbance, lid disorders, dry eye and trigeminal nerve damage., Results: Twenty-nine patients were included; 89.7 % were women. The mean age was 55 years. Postoperative complications included 25 % residual proptosis; 40.7 % oculomotor disturbance, 75 % of which regressed at least partially; 50 % ptosis, 21 % of which did not regress; 40 % dry eye; and in 21.4 % the trigeminal nerve was involved., Conclusion: The management of meningiomas with orbital extension is difficult due to their anatomical location and requires joint neurosurgical and oculoplastic management. Sufficient follow-up is required before recommending surgery for oculomotor disturbances. The frequency of occurrence of ptosis with potential recommendation for surgery requires caution given the occurrence of trigeminal nerve involvement and dry eye syndrome., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Requests of organs to be transplanted increase. As a matter of urgency, it is not always easy to decide if a patient carrier of a brain tumor can be candidate in the donation. After a review of the literature, the members of the Association of the Neuro-oncologists of French Expression (ANOCEF) and the Club of Neuro-oncology of the French Society of Neurosurgery propose consensual recommendations in case of donor carrier of primitive tumor intra-cranial or intra-medullary. A contact with the neuro-oncologist/neurosurgeon will allow to discuss the indication in case of glioma of grade I/II/III, according to the grade, the current status (absence of progressive disease), the number of surgeries and of lines of treatment. The taking is disadvised in case of glioma of grade IV (glioblastoma), of lymphoma or meningioma of grade III. No contraindication for the meningiomas of grade I, and individual discussion for the meningiomas of grade II. It is advisable to remain careful in case of hemangiopericytoma and of meningeal solitary fibrous tumor. The patients in first complete remission of a medulloblastoma or intra-cranial primitive germinoma seem good candidates for the taking of organ if the follow-up is of at least 10 years (3 years for non germinomas). In every case, a multidisciplinary discussion is desirable when it is materially possible., (Copyright © 2017 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Multiple meningiomas (MMs) or meningiomatosis are defined by the presence of at least 2 lesions that appear simultaneously or not, at different intracranial locations, without the association of neurofibromatosis. They present 1-9 % of meningiomas with a female predominance. The occurrence of multiple meningiomas is not clear. There are 2 main hypotheses for their development, one that supports the independent evolution of these tumors and the other, completely opposite, that suggests the propagation of tumor cells of a unique clone transformation, through cerebrospinal fluid. NF2 gene mutation is an important intrinsic risk factor in the etiology of multiple meningiomas and some exogenous risk factors have been suspected but only ionizing radiation exposure has been proven. These tumors can grow anywhere in the skull but they are more frequently observed in supratentorial locations. Their histologic types are similar to unique meningiomas of psammomatous, fibroblastic, meningothelial or transitional type and in most cases are benign tumors. The prognosis of these tumors is eventually good and does not differ from the unique tumors except for the cases of radiation-induced multiple meningiomas, in the context of NF2 or when diagnosed in children where the outcome is less favorable. Each meningioma lesion should be dealt with individually and their multiple character should not justify their resection at all costs., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

The action of synthetic progestogens, prescribed at a conventional dose in women, for a meningioma, is still poorly understood, and could be related to progesterone receptors. We report two cases illustrating multiple meningiomas with stabilization or tumor reduction after withdrawal of cyproterone acetate originally prescribed for a long term period. We also review the influence of synthetic progestogens on meningiomas, particularly the impact of treatment withdrawal., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Introduction: Optic nerve tumors are uncommon and rarely described. By way of five patients treated at the University Hospital of Clermont-Ferrand, France, over a six-year-period, we discuss their clinical and radiological characteristics, their treatment and their course., Observations: We report two cases of optic nerve glioma in small children, two cases of optic nerve sheath meningioma and a 57-year-old patient treated for lung cancer with an optic nerve metastasis and choroidal and brain metastases. The diagnosis was confirmed by imaging in all patients and histologically for the cases of optic nerve glioma., Results: The children with glioma were exclusively treated with chemotherapy, the two women with meningioma exclusively with radiation therapy, and the man with the optic nerve metastasis by chemotherapy and radiotherapy. The tumors were stabilized in all cases except for a progression in one case of glioma, one year after treatment., Discussion: Gliomas and meningiomas are the most frequent tumors; other tumors are rare. Through a literature review and many illustrations, we discuss epidemiologic, clinical and radiologic characteristics, treatment and course of these uncommon tumors., Conclusion: The diagnosis of optic nerve tumors must be considered in cases of anterior or posterior progressive optic neuropathy. Treatment must be a compromise between effective treatment of the tumor and preservation of visual function. Decisions are made through multidisciplinary consultations, in which the role of the ophthalmologist is crucial for the diagnosis and success of the treatment., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

We report on a 51-year-old woman who presented with a cervical spinal cord tumor clinically suspected to be a metastasis. Histological examination revealed an anaplastic meningioma containing epithelial nests arranged in a gland-like pattern suggestive of adenocarcinoma. This component strongly expressed cytokeratins whereas the meningothelial component was vimentin--epithelial membrane antigen--and progesterone receptor-immunoreactive, suggesting either anaplastic meningioma with adenocarcinoma-like metaplasia, or adenocarcinoma metastasis in a meningioma, but the search for a primitive neoplasia including thoracic-abdominal-pelvic computed tomography and mammography was negative. Anaplastic meningiomas with adenocarcinoma-like metaplasia are uncommon lesions, 4 cases having been reported in the literature so far. Their immunohistochemical and chromosomal characteristics are similar to those observed in secretory meningiomas. When available, fluorescence in situ hybridization detects the same chromosomal alterations in the two components, confirming a common clonal origin. This observation demonstrates the necessity to perform the correct diagnosis of malignant meningioma with adenocarcinomatous metaplasia, whose prognosis and treatment radically differ from those of metastatic adenocarcinoma located in a meningioma., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Radiotherapy is a major modality in the treatment of brain tumours. The target volumes definition has to be precise for the radiation planification. The gross target volume (GTV) is most of the time delineated within the fusion of the planning CT scan with the appropriated MRI sequences. The clinical target volume (CTV) definition is more complex: it varies in time following the evolution of scientific knowledge and also depending of the school of thought. This article offers a review of the literature about the margins applied in brain tumours radiotherapy for gliomas (high grade, anaplastic, low grade and brain stem gliomas), embryologic tumours (medulloblastomas and primitive neuroectodermal tumours [PNET]), ependymomas, atypical teratoid rahbdoid tumours (ATRT), craniopharyngiomas, pineal gland tumours, primary central nervous cell lymphomas, meningiomas and schwannomas. New imaging modalities such as diffusion-weighted imaging, dynamic contrast enhanced, spectroscopic MRI and PET scan will allow us to delineate more precisely the target volumes and to realise dose-painting by adapting the dose to the tumour metabolism., (Copyright © 2013 Société française de radiothérapie oncologique (SFRO). Published by Elsevier SAS. All rights reserved.)

Introduction: Meningiomas are benign primary meningeal tumors. Their diagnosis may be incidental or in response to a work-up for neurological or ophthalmological symptoms., Patients and Methods: The clinical course of five patients with ophthalmological symptoms leading to the diagnosis of meningioma is described., Results: The case reports consist of five women (48 to 54 years old - mean 52 years at the onset of symptoms), all suffering from a progressive unilateral decrease in visual acuity with a normal initial fundus examination and ipsilateral visual field changes. Ancillary testing, in particular MRI and CT-scans, had to be repeated to make the diagnosis of meningioma, which was delayed from 18 months to 4 years., Discussion: The clinical presentation of these five cases was that of a retrobulbar optic neuropathy, which biased the work-up towards an inflammatory disease of the central nervous system such as multiple sclerosis. However, the atypical character of the neuropathy, which did not respond to intravenous steroids, caused the diagnosis to be questioned and radiological examinations repeated. The iso-intense appearance of meningiomas on T1 MR imaging and only slightly hyperintense appearance on T2 may result in a diagnostic delay if the exam is not performed and interpreted by an experienced professional. Gadolinium contrast, fat suppression and centration on the anterior visual pathways are essential to a proper MRI examination., Conclusion: When confronted with a progressive, painless optic neuropathy unresponsive to steroid treatment, the diagnosis of meningioma of the anterior visual pathways must be considered. This diagnosis is enabled by a targeted MRI of the anterior visual pathways., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Papillary meningioma (MP) is a rare and aggressive variant of meningioma, occurring preferentially in young subjects. Histopathological features of papillary meningiomas are characterized by papillary or perivascular pseudorosette patterns. We report the case of a right frontal MP in a 16-year-old boy. Differential diagnosis included other primitive or secondary intracranial papillary neoplasms. MP diagnosis should not be missed due to its aggressive behavior with brain invasion, local recurrence and metastases., (Copyright © 2013. Published by Elsevier Masson SAS.)

Meningiomas are the most common non-malignant tumours of the brain. Gross-total resection remains the preferred treatment, if achievable without morbidity. Radiation therapy is advocated for inoperable, incompletely resected, or recurrent grade 1 tumours, if there is a progressive, symptomatic lesion, or in case of functional impairment. Postoperative radiation therapy is recommended for grade 2 or 3 lesions. Fractionated stereotactic radiotherapy and stereotactic radiosurgery are high precision techniques, allowing good sparing of surrounding tissues. Fractionated stereotactic radiotherapy and stereotactic radiosurgery give comparable results, with excellent 5-year tumour control rates of more than 90% for benign meningiomas. Toxicity is low and seems equivalent, despite a biased use of fractionated stereotactic radiotherapy for larger meningiomas, close to critical structures. Fractionated stereotactic radiotherapy seems to be of special interest in the treatment of cavernous sinus or optic pathways meningiomas. The different therapeutic modalities should be discussed by a multidisciplinary team., (Copyright © 2012 Société française de radiothérapie oncologique (SFRO). Published by Elsevier SAS. All rights reserved.)

Conventional radiotherapy is known to be an effective treatment approach even for "benign" pathologies. However, this kind of treatment yields a high potential for side effects. The Cyberknife, a robotic stereotactic radiotherapy device, enables to offset a large proportion of the disadvantages encountered with conventional radiotherapy essentially through the high precision of dose administration and sparing of healthy tissues. Therefore, it seems to be a treatment of choice in the approach of some benign intracranial diseases. We review published data on indications and outcome of Cyberknife for intracranial "non-malignant" disease.

Magnetic resonance imaging arose as a reference for diagnosis, pre-therapeutic and follow-up of brain tumors. Among parameters obtained from standard MRI (of low specificity), only volumetric growth allows prognostic information. The multiple "advanced" sequences have leaded to increase both sensitivity and specificity of brain MRI. Yet, perfusion-weighted imaging and spectroscopy provide metabolic information, and diffusion tensor imaging and cortical activation provide functional information. Characterization, grading, therapeutic management and follow-up have improved, with prognostic information., (Copyright © 2011. Published by Elsevier Masson SAS.)

Unlabelled: The data from 284 cases of lateral ventricle (VL) tumors treated in 18 departments of Neurosurgery in France between 1995 and 2010 were collected in this series. The mean age was 33 years with a large proportion of children (79/284)., Clinical Presentation: The mean duration of symptoms was short in time (2 months). The revelation was incidental in 10% of the cases; for symptomatic lesions, raised intracranial pressure (ICP, due to hydrocephalus) and neuropsychological signs were the most frequent. Only one third of the patients had no CSF disorder., Histopathology: The two most frequent tumors were meningiomas (40 cases, 15%) and neurocytomas (35 cases, 13%). The sub-ependymal giant cells astrocytomas, the ependymomas and sub-ependymomas were also common (11, 10 and 9% respectively)., Therapeutic Management: A surgical procedure was performed in 258 patients, with the help of neuronavigation in one third of cases. The patients were most frequently operated with a transfrontal approach (40%). The resection was complete in 73%, which was a statistical factor of global survival. The postoperative mortality rate was 4.9%., Outcome: Neuropsychological deficits were the most frequent sequelae (22.8%) due to raised ICP. Campimetric deficits (19%), seizures (19%), hemiparesis (15%) and language disorders (12%) were then found. The mean follow-up was 52 months with good evolution for 58% of patients. Fifteen percent of patients had a handicap and, finally, 12.9% will die. At last, two third of living patients had no tumoral residue or re-evolution., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

This series reports 40 cases of meningiomas of the lateral ventricle treated in France between 1995 and 2010. The mean age was 52 years. The revelation was incidental in 23% of cases. 36 patients underwent surgery with uneventful postoperative course in 75% of them. Visual field deficits were the most frequent sequelae. In six cases, recurrences occurred during the follow-up period. The relevant series of the literature were discussed in order to precise the therapeutic strategies and the outcome., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Lateral ventricular neoplasms are rare, and account for 50% of all intraventricular tumors in adults and 25% in children. Although these neoplasms are easily detected with computed tomography (CT) and magnetic resonance imaging (MRI), both techniques are relatively unspecific in identifying the type of tumor. However, few imaging patterns are specific for a particular pathological process and useful conclusions can be made from the morphological appearance of the lesion, its location and enhancement pattern. The aim of this article was to review and illustrate the CT and MRI findings of a wide spectrum of tumors of the lateral ventricle. We reviewed choroid plexus tumors, meningioma, subependymal giant cell astrocytoma, central neurocytoma, and less frequent lesion such as lymphoma and metastases., (Copyright © 2011. Published by Elsevier Masson SAS.)

Typing a tumor of the lateral ventricle is often an issue, even for an experienced Neuropathologist. In this location are encountered specific entities, such as neurocytoma and subependymal giant cell astrocytoma, as well as more usual tumors, displaying a common misleading morphology, for instance a main clear cell component. The panel of diagnostic tools given to the pathologists has been increasing for a few years, enriched by immunohistochemical and molecular probes., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Aims: Clear cell meningioma (CCM) is a rare variant of meningioma, which is important to distinguish because of its aggressive behaviour. Sixty-eight cases have been previously described in the literature. In this retrospective study, we report seven cases of CCM operated in our institution between 1994 and 2008., Methods: Seven CCM cases were retrieved from the files of our pathology department. Clinical and radiological data were reviewed. A standard histological study was realized and immunohistochemistry was performed with epithelial membrane antigen (EMA), cytokeratin KL1, progesterone receptors, Ki-67 (MIB-1), S100 protein., Results: Patients' age ranged from 2 to 70 years (median age: 36 years), with a female predominance (5/7 patients). Three patients belonged to the same family, probably affected by neurofibromatosis type 2. CCM occurred in various locations: medullary region (two), sphenoid wing (two), ponto-cerebellar angle (two), tentorium (one). The tumour could be fully resected in three cases. Follow-up ranged from 3 months to 15 years: recurrence occurred in four patients, three of whom eventually died from the disease., Discussion: In our series, the frequency of CCM (0,6% of all meningiomas operated on in our institution) and its histological aspects are almost identical to those observed of the literature. We discuss the predictive value of proliferation index (MIB-1) and the role of patient status and quality of surgical resection in the evolution., Conclusion: Our study supports the fact that MCC course is less favourable than meningioma WHO grade I, even in the absence of anaplastic area, high mitotic activity, or necrosis. In this series, MIB-1 index was of no interest identifying patients with or without recurrence., (Copyright 2010 Elsevier Masson SAS. All rights reserved.)

Optic nerve sheath meningiomas account for one-third of optic nerve tumors, 1-2 % of all meningiomas, and 2-3.5 % of all orbital tumors. Loss of vision is the main clinical sign. Diagnosis is easily made after the neuroradiological evaluation. Fractionated stereotactic radiotherapy (RSF) is now the gold standard treatment for controlling tumor volume and preserving visual acuity., (Copyright 2010 Elsevier Masson SAS. All rights reserved.)

Tumor-to-tumor metastasis is a very rare event. We report three cases of tumor metastasizing in another tumor: a clear cell renal cell carcinoma in a vesicular thyroid adenoma, a lung carcinoma in a meningioma and a neuroendocrine lung carcinoma in a clear cell renal cell carcinoma. According to the literature, clear cell renal cell carcinoma is the most common tumor recipient of metastasis while lung carcinoma is the most common donor tumor. Several physiopathological mechanisms can explain this phenomenon, but many of them are still unknown.

Objectives: We report a case of purely extradural spinal meningioma and discuss the potential pitfalls in differential diagnosis., Background: Spinal meningiomas account for 20-30% of all spinal neoplasms. Epidural meningiomas are infrequent intraspinal tumors that can be easily confused with malignant neoplasms or spinal schwannomas., Case: A 62-year-old man with a previous history of malignant disease presented with back pain and weakness of the lower limbs. Magnetic resonance imaging revealed a well-enhanced T4 intraspinal lesion. The intraoperative histological examination showed a meningioma (confirmed by postoperative examination). Opening the dura mater confirmed the purely epidural location of the lesion. The postoperative course was uneventful with no recurrence 12 months after surgery., Conclusion: Purely extradural spinal meningiomas can mimic metastatic tumors or schwannomas. Intraoperative histology is mandatory for optimal surgical decision making.

Introduction: En plaque meningioma are a morphological subgroup of meningioma defined by a carpet or sheet-like lesion that infiltrates the dura and sometimes invades the bone. We present an exceptional case due to its localization and evolution., Case: A 41-year-old female patient consulted for progressive swelling of the left frontoparietal region. CT-scan revealed thickening of the cranial medulla, without cerebral lesion. The tumor was removed. Histological analysis confirmed en plaque meningioma which recurred 7 years after., Discussion: En plaque meningioma are a rare subtype of meningioma which rarely affects the skull vault. Relapse is frequent.

Cutaneous heterotopic meningeal nodules are rare lesions whose pathogenesis remain discussed. They are typically located on the scalp or the rachidian axis. They often are congenital and diagnosed during childhood or young adulthood. There are two admitted theories for pathogenesis of heterotopic meningeal nodules: (1) tumoral proliferation of ectopic arachnoïdal cells (true cutaneous meningioma), (2) meningocele-type developmental defect (rudimentary meningocele). Histological and immunohistochemical aspects are characteristic, showing arachnoidal cells, EMA+ and vimentine+, that infiltrate collagene and whorl around collagene or psammomatous bodies. We report the expression of progesterone receptor (PR) in a cutaneous heterotopic meningeal nodule on the scalp of a 23-year-old man, and propose this PR expression as a new diagnosis tool for such lesions.

Background and Purpose: The invasion of bone responsible for hyperostosis is a typical phenomenon associated with en-plaque meningiomas. Although the sphenoid wing and spheno-orbital region are most frequently affected, petrosal involvement is rare and its surgical treatment difficult. Hyperostosis is caused by bone invasion, is responsible for the clinical signs, and prompts the surgeon to use an à la carte drilling that has to be evaluated preoperatively and carried out depending on tumor extension and the treatment goals., Methods: We report two cases of invasive and evolving en-plaque petrosal meningiomas. Hyperostosis, bony modifications, and intracranial portion of the lesion were responsible for cophosis, facial palsy, trigeminal neuralgia, dysphonia, and laryngeal palsy in one case, and were responsible for hearing loss and facial palsy in the other case., Results: In both cases, the à la carte petrosectomy allowed us to achieve total removal of the lesion. In one case, we used a trans- and infralabyrinthine transjugular approach (to control the extension of the lesion in the jugular foramen, within the sinusojugular axis, and in the internal auditory canal), associated with an anterior petrosectomy (to control the invaded petrous apex, Meckel's cave, and a middle cranial fossa extension). In the other case, we used a retro- and infralabyrinthine transsigmoid transtentorial approach to control the venous axis, the posterior fossa dura, and the tentorium. Total removal of the tumor including bone invasion was achieved in both cases. Neurological deficits improved or remain unchanged. Transient postoperative facial palsy recovered in two months., Conclusions: An à la carte petrosectomy performed by a surgical team with great expertise in the field of petrous bone anatomy and segmentation should lead to total removal including exposure of the dural tail and intracranial portion of the tumor, while preserving all cranial nerve functions.

Unlabelled: The recurrence and progression of treated intracranial meningiomas highlights the problem of the type of follow-up that should be used and whether early complementary treatment is indicated. The aim of this study was to evaluate different biochemical markers involved in cell proliferation and transformation to identify new prognostic factors in intracranial meningiomas. Between 1989 and 2003, 120 intracranial meningiomas were studied biochemically. The levels of estrogen receptors (RE), progesterone receptors (RP), cathepsin B (CB), cathepsin L (CL), stefin A (ATA), stefin B (STB), cystatin C (CYSC), urokinase (u-PA), type 1 plasminogen activator inhibitors (PAI-1), cathepsin D (CD) and thymidine kinase activity (TK) were measured in tumor extracts using biochemical assays., Results: Out of 120 meningiomas, 73 were grade I, 39 grade II and eight grade III according to the WHO classification. Of these patients, 17 showed recurrence. The mean follow-up was 47 months. Monofactorial analysis showed that expression of progesterone receptors (RP) had an inverse correlation with recurrence (p=0.0025 %) and that thymidine kinase activity (TK), cathepsin L (CL), the WHO grade and the degree of tumor resection correlated with recurrence (p<0.05). Principal component analysis and linear discriminant analysis confirmed these results. The results of this study confirm the importance of biological parameters (PR, CL, TK) as prognostic factors for the risk of recurrence in intracranial meningiomas.

The treatment of cavernous sinus meningiomas remains controversial. However, at present, the general consensus is that only tumors with extracavernous extensions should be operated on and that resection is performed only on the extracavernous portions. Since these tumors are deep-seated and in the vicinity of vital and highly functional neurovascular structures, surgery remains difficult and dangerous. To achieve the gross total removal of the extracavernous extensions with minimal sequelae, the operative strategy must take into account the location of the tumor extension, not only in the basal cisterns but also into the skull base structures, the vascularization of the tumor with its feeders, as well as the venous drainage pathways. These anatomical and surgical data are collected from preoperative imaging, which includes CT-scan with bony windows and consecutive fine sections, MRI, angio-MR, and DSA angiography performed with selective arteriography including late venous phases. The operative plan must define the type of craniotomy and complementary osteotomy, the usefulness of an anterior clinoidectomy with skeletonization of the optic nerve, and determine whether extensive exposure of the cerebral middle fossa is necessary to ensure substantial devascularization of the tumor and proximal control of the internal carotid artery at its intrapetrosal portion. In addition, the possible need for an extra-intracranial bypass prior to tumor removal should be evaluated. The high number of neuroimaging investigations is the guarantee of surgical accuracy and safety.

Metastatic spreading is a rare complication of meningioma. We report a subcutaneous metastasis from an atypical meningioma of the tentorium. A 69-year-old man presented with a meningioma of the tentorium. The tumor was totally resected. The histological diagnosis was atypical meningioma. The tumor recurred two years later and was irradiated. Despite irradiation, the tumor progressed and was resected three years later. Five months following the second surgery, an isolated subcutaneous mass developed at the skin incision and was resected. The histological examination of the subcutaneous tumor showed histological features of atypical meningioma. Surgery of atypical meningioma carries the risk of iatrogenic metastasis, despite previous irradiation.

Background and Purpose: Spinal meningioma surgery is usually not difficult and is commonly associated with good outcome. However, advanced age and severe neurological deficit have been considered to be predictors of poor surgical outcome. Therefore, we attempted to assess the surgical outcome of spinal meningiomas in the elderly and to analyze the role of outcome predictors., Methods: From 1990 to 2006, 32 patients 76 years or older with spinal meningiomas were operated on in our Neurosurgery Departments. All patients had MRI. Neurological status was assessed using the Solero score. Neurological evaluations were conducted three months and one year after surgery. The mean follow-up was 36 months. A multiple logistic regression was applied to establish the relationship between the risk factors and outcome., Results: The median age was 79.3 years. The mean duration of symptoms until surgery was 12.7 months. One patient was rated Solero grade I, 11 grade II, 17 grade III and three patients were rated grade IV. Radical tumor removal was performed in 30 patients (94%). All meningiomas were benign. There was no recurrence, morbidity was 9%, and 1-year mortality was 0%. One year after surgery, all patients had improved, 56 % had recovered completely. Among 20 patients with severe paraparesis or paraplegia, 30 % had recovered completely. There was no statistical correlation between outcome and various risk factors: age, gender, ASA, tumor size, quality of tumor removal, and location. Only preoperative Solero score and duration of symptoms before surgery were statistically significant., Conclusions: Surgery is the only treatment of symptomatic spinal meningioma. Advanced age did not seem to contraindicate surgery, even in those with severe preoperative neurological deficits, because quality of life can be improved in the vast majority of cases. There was a correlation between duration and severity of deficit and outcome.

Neoplasms with perivascular épithelioid-cell differentiation (PEComas) are rare tumors with a distinctive immunoreactivity for melanocytic markers. They have been described in various organs. We report an intrapancreatic PEComa discovered in a 46-year-old woman during a workup for diarrhea. CT scan showed a 1.7cm nodule in the body of the pancreas with slight-contrast enhancement at arterial time and isodense at portal time. The aspect was suggestive of an endocrine tumor despite negative somatostatin-receptor scintigraphy. Enucleation was performed. Pathologic evaluation showed a well-circumscribed intrapancreatic tumor consisting of a population of clear to eosinophilic spindle cells and a less abundant population of epithelioid cells arranged around blood vessels. Tumor cells expressed vimentin, HMB45 and actin and only focally S-100 protein, KL1, CD117 and CD34. These features were consistent with a PEComa. Pancreatic PEComas are rare, but should be included in the differential diagnostic of pancreatic clear cells tumors or pancreatic spindle- and epithelioid-cells tumors.

Unlabelled: Tumours of the central nervous system (CNS) have not received much scientific attention in sub-Saharan Africa, especially in the central African zone. The aim of this study was to determine the relative frequency and different histologic types of CNS tumours seen in the neurosurgery units of Cameroon, a multiethnic country of central Africa. This retrospective study covers the decade from January 1996 through December 2006 in the three neurosurgery departments in Cameroon, at the Yaoundé General Hospital, the Yaoundé Central Hospital, and the Douala General Hospital., Inclusion Criteria: All cases undergoing surgery in these units for a histologically-confirmed CNS tumour. There were 231 cases with complete files, accounting for 6.3% of total admissions in the various units. Patients' mean age was 46.3+/-20.6 years (range: 2 weeks to 74 years); the male/female ratio was 0.8. Paediatric patients made up 15.1% (n=35) of the population while adults comprised 84.9% (n=196). In all, 74.9% (n=173) of the tumours were intracranial and 25.1% (n=58) spinal; 167 (72.3%) were the primary site and 64 (27.7%) metastatic. The average age of patients with metastatic tumors was 42+/-18.5 years compared with 36.5+/-17.8 years for cases with primary tumors. Primary tumors were malignant in 34.2% (n=12) of the children and benign in 65.8% (n=23); among adults 22.7% (n=30) were malignant and 77.3% (n=102) benign. Meningiomas were the most frequent tumours in our series, with 56 (24.2%) cases, followed by 42 astrocytomas (18.1%). Most patients were younger than 55 years. In conclusion, CNS tumors occurred mainly before the age of 55 years and had a slight predilection for girls and women. Meningiomas were the most frequent tumors in adults while astrocytomas were more prevalent in children.

Background: Cystic meningioma is a rare variety of meningioma. It represents 1,6 to 10% of intracranial meningiomas, the authors report a case of intracranial cystic meningioma with a review of literature., Case Report: A 46-year-old female presented with left parietooccipital headache followed by right side hemiparesis. CT scan brain showed a left parietal tumor with double solid and cystic components thought to be glioma or metastasis preoperatively. At surgery the extraaxial solid and cystic lesion had a well defined capsule that could be easily separated from the perilesional cortical surface. The tumor was totally removed. The histological study showed a cystic meningioma., Conclusion: Cystic meningioma is an uncommon tumor that should be considered in the differential diagnosis of brain tumors with a cystic component.

Meningioma is an unfrequent optic nerve tumor. Clinical symptoms are not specific, and pathology exam not easily accessible. The diagnosis is usually based on imaging findings. We report our experience, based on twenty patients. Most of them were middle-aged women (mean age=47 years old). The optic nerve meningioma (ONM) was often revealed by visual dysfunction. Ophthalmological evaluation was altered in most patients (visual acuity, visual fields, fundi). The gold-standard exam was MRI of the optic nerve, with and without gadolinium, and with fat-sat sequences. Thirty percent of the lesions remain stable without treatment. Functional prognosis has been improved only by conformal fractionated radiotherapy, but indications and modalities remain to be specified.

Chordoid meningioma is an uncommon histopathological variant of meningioma frequently confused with chordoma. Recurrence is not uncommon. The aim of our study is to present the clinical and pathological characteristics of this entity and a review of the literature. We report two cases of chordoid meningioma occurring in two women aged 47 and 63 years. Both women presented neurological signs without systemic disease. Radiological findings were temporoparietal and temporofrontal lesions with intense enhancement after contrast injection. Complete surgical resection was performed. Histopathologic findings were consistent with chordoid meningioma.

Meningiomas account for approximately one-fourth of all primary central nervous system tumour, arising from arachnoidal cells surrounding brain and spinal cord. They usually affect older adults, particularly women. Ninety percent of meningiomas are slow growing benign tumours. Meningiomas are revealed by various symptoms including neurologic deficits and epileptic seizures. Surgery remains the treatment of choice. Fractionated radiotherapy or stereotactic radiosurgery are used for meningiomas that are recurrent, surgically inacessible, partially excised and either atypical or malignant. Most meningiomas have good long-term prognosis after treatment, some dysplay aggressive clinical behaviour leading to increased patient morbidity and mortality.