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Metastatic meningioma is a rare situation. We conducted a retrospective study from our databases and identified cases of metastatic meningioma. We report three presentations of patients with medical history of surgical removal of meningioma presenting several years later a liver tumor with bone metastasis or multiple lung tumors. These observations highlight the difficulty of the clinical and pathological diagnosis and the absence of consideration of metastatic state for histologically "benign" but clinically aggressive meningiomas in the current WHO 2007 classification of meningiomas. We also reviewed published cases of metastatic meningiomas since they are clearly distinguished from haemangiopericytoma.

PURPOSE: Pilocytic astrocytomas (PA) and hemangioblastomas (HB) can present the same morphological characteristics on conventional MRI sequences, most usually in the form of a cerebellar cystic mass with a mural nodule that strongly enhances on post-contrast T1 images. We discuss here the value of perfusion MRI in the differentiation of these two tumors, the diagnoses of which have already been histopathologically established. METHOD: Eleven patients with PA and eight with HB underwent first-pass perfusion MRI. The maximum relative cerebral blood volume (rCBV(max)), defined as the ratio between the CBV(max) in tumor tissue and the CBV in healthy, contralateral white matter, is considered to be indicative of the type of tumor. RESULTS: The difference between the rCBV(max) of PA (rCBV(max)=1.19+/-0.71, range 0.6-3.27) compared with that of HB (rCBV(max)=9.37+/-2.37, range 5.38-13) was significant (P

Synovial sarcomas are aggressive malignant soft tissue tumors typically observed in adolescents and young adults. They are often characterized by the chromosomal translocation t(X;18)(p11.2;q11.2), which results in the expression of SYT-SSX fusion transcripts. We describe the first case of synovial sarcoma observed in a human fetus. The tumor occurred in the left upper arm and led to intrauterine fetal demise during gestational week 31. Grossly, the tumor measured 10 x 8 x 8 cm, appeared pinkish in color, and developed in the soft tissues of the left arm surrounding the humerus. Histologically, this large tumor showed a dense proliferation of homogeneous spindle cells with some necrotic areas. The positive detection of the SYT-SSX1 fusion transcripts with reverse-transcription polymerase chain reaction in formalin-fixed and paraffin-embedded tissue confirmed the synovial sarcoma diagnosis.

Clear cell meningiomas (CCM) are rare tumors of the nervous system that usually occur in young patients and display high recurrence rates and potentially aggressive behavior. In this report, we describe a primary CCM of the orbit in an 84-year-old man with a previous history of a clear cell carcinoma of the kidney. Histologically, the tumor demonstrated a sheet-like proliferation of clear polygonal cells. Differential diagnosis includes metastasis of clear cell carcinomas. Immunohistochemistry, by showing that tumor cells expressed vimentin, epithelial membrane antigen, and progesterone antigens, and cytogenetic analysis, by identifying a monosomy 22, confirmed the diagnosis of CCM.

Resume Introduction En quelques annees, l’IRM a evolue d’une exploration morphologique vers une exploration metabolique et fonctionnelle. Etat des connaissances L’IRM est la pierre angulaire du bilan des tumeurs cerebrales. Elle a un role lors du bilan initial et permet souvent une bonne caracterisation tissulaire du processus lesionnel. Toutefois, elle ne suffit pas a differencier abces, gliome malin et metastase unique, a apprecier le grade histologique des gliomes, a definir les limites tumorales, a caracteriser les tumeurs meningees. Elle a un role lors du suivi pour controler le geste operatoire, apprecier la reponse au traitement, rechercher des signes de recidive. Neanmoins, l’interpretation ne repose que sur des donnees volumetriques qui peuvent etre prises en defaut. Perspectives La spectroscopie 1 H, la diffusion et la perfusion, techniques disponibles sur la plupart des appareils cliniques apportent des informations complementaires sur le metabolisme et la vascularisation des tumeurs. Elles permettent une meilleure caracterisation tissulaire et une meilleure comprehension des modifications post-therapeutiques. Actuellement, aux donnees morphologiques du bilan IRM d’une tumeur cerebrale doivent s’ajouter des informations metaboliques et fonctionnelles.

To determine the perfusion-sensitive characteristics of cerebral dural metastases and compare them with the data on meningiomas. Twenty-two patients presenting with dural tumor underwent conventional and dynamic susceptibility-contrast MR imaging: breast carcinoma metastases, two patients; colorectal carcinoma metastasis, one patient; lung carcinoma metastasis, one patient; Merkel carcinoma metastasis, one patient; lymphoma, one patient; meningiomas, 16 patients. The imaging characteristics were analyzed using conventional MR imaging. The cerebral blood volume (CBV) maps were obtained for each patient and the relative CBV (rCBV) in different areas was calculated using the ratio between the CBV in the pathological area (CBVp) and in the contralateral white matter (CBVn). The differentiation between a meningioma and a dural metastasis can be difficult using conventional MR imaging. The rCBVs of lung carcinoma metastasis (1 case: 1.26), lymphoma (1 case: 1.29), breast carcinoma metastasis (2 cases: 1.50,1.56) and rectal carcinoma metastasis (1 case: 3.34) were significantly lower than that of meningiomas (16 cases: mean rCBV = 8.97±4.34, range 4–18). Merkel carcinoma metastasis (1 case: 7.56) showed an elevated rCBV, not different from that of meningiomas. Dural metastases are sometimes indistinguishable from meningiomas using conventional MR imaging. rCBV mapping can provide additional information by demonstrating a low rCBV which may suggest the diagnosis of metastasis.

The term chordoid glioma of the third ventricle was first used to describe a rare and slowly growing neoplasm of uncertain histogenesis, with chordoid appearance, occurring preferentially in middle-aged women. Herein we report two additional examples of this novel entity together with a literature review based on the 25 cases previously published. Our review fully confirms the strikingly stereotyped clinical, neuroradiologic, and pathologic features of this unique tumor. The female/male ratio was 1.7:1, and the age range was 24-70 years (mean 44.9 years). In all 27 cases imaging findings were similar showing a well-defined mass (mean 2.8 cm in largest dimension), ovoid in shape, hyperdense on CT scans, with uniform and intense contrast enhancement, arising in the hypothalamic/suprasellar/third ventricular region. Histologically, the main consistent characteristics were cords and clusters of epithelioid cells within an abundant mucinous and often vacuolated background. Mitoses were sparse or absent and anaplastic features, endothelial proliferation, and necrosis were not identified. Lymphoplasmacytic infiltrates with Russell bodies were frequent throughout the tumor and its interface with adjacent brain parenchyma. Most of the tumor cells revealed a strong and diffuse expression of vimentin and glial fibrillary acidic protein. Additionally, the vast majority of tumors showed focal coexpression of cytokeratins, CD34, S-100 protein, and epithelial membrane antigen; the MIB-1 labeling indices were uniformly low. Surprisingly for a glioma assigned WHO grade II, the 19 patients with an available but short follow-up (mean 22.5 months; range 6-68 months) experienced a rather poor outcome (three recurrences and seven deaths), probably reflecting the anatomic site of the neoplasm that precludes a complete surgical excision rather than its histologic composition. Ultrastructural examination of 10 cases demonstrated findings in line with a glial derivation and a putative ependymal origin such as cytoplasmic intermediate filaments, microvilli, intermediate junctions or desmosomes, and focal basal lamina formation. In our case no. 1, and for the first time in this tumor, we observed sparse and abnormal cilia in an aberrant juxtanuclear location, a further argument for considering chordoid glioma as a subtype of ependymoma. However, a better understanding of the biologic behavior and histogenesis of this distinctive clinicopathologic entity needs to be investigated with a larger series. Nevertheless, taking into account its strikingly consistent anatomic localization, its unique histopathologic and immunohistochemical profile, in conjunction with the most recent and convincing ultrastructural arguments, we suggest that chordoid glioma of the third ventricle could be better classified as chordoid ependymoma of the lamina terminalis area.

N-myc amplification is a major prognostic factor in neuroblastomas and is systematically investigated by Southern blot or polymerase chain reaction (PCR). A retrospective study of N -myc amplification has been carried out using fluorescence in situ hybridization (FISH) in 97 fixed neuroblastomas. For each tumour, FISH was performed on the area that contained the most immature neuroblasts. Among these 97 neuroblastomas, 16 were amplified and 12 were not interpretable. FISH was not interpretable in six cases. All neuroblastomas with N-myc amplification detected by Southern blot/PCR were amplified with FISH, except three that were not interpretable. Four tumours that were not interpretable in Southern blot/PCR contained more than five copies of N-myc by FISH: one was aneuploid and three were truly amplified, containing more than ten copies of N-myc. Among these three patients, two died in a short time of their tumours. Ten cases were not amplified by Southern blot/PCR and showed more than five copies by FISH: four were aneuploid and two showed heterogeneous amplification, with a few cells clearly amplified whereas most were not. Four cases were amplified, of which two patients died of their tumours. This study confirms that when applied to the most immature areas of fixed neuroblastomas, FISH displayed a higher sensitivity than molecular techniques (p < 0.001) and could detect heterogeneous amplification. FISH could therefore become an important complementary procedure in assessing prognosis in neuroblastomas.

Resume Nous rapportons l’observation d’un ependymome de double localisation, cerebrale et sacree, revele par un syndrome cerebelleux et une sciatique chez un homme de 68 ans dont l’evolution est marquee par l’apparition de metastases osseuses sept mois apres l’exerese chirurgicale de la tumeur cerebrale.

Desmoplastic small round cell tumors (DSRCTs) present a reciprocal chromosomal translocation, t(11;22)(p13;q12), that results in fusion of Ewing's sarcoma and Wilms' tumor (WT1) genes. The authors evaluated 15 DSRCTs and 71 other tumors often considered in the differential diagnosis for immunoreactivity using a polyclonal antibody directed against the WT1 part of the chimeric protein resulting from this translocation. WT1 immunostaining was performed on paraffin material using the WT(C-19) antibody after heat-antigen retrieval. All the DSRCTs (15 of 15, 100%) demonstrated strong WT1 nuclear immunoreactivity. Ten of 14 nephroblastomas (71%) disclosed WT1-positive nuclei in accordance with the staining reported by others, and rare and focal nuclear positivity was detected in two of 17 rhabdomyosarcomas. WT1 immunoreactivity was not observed in Ewing's sarcoma/primitive neuroectodermal tumors (zero of 21, 0%), neuroblastomas (zero of 17, 0%), or rhabdoid tumors of the kidney (zero of two, 0%). In nephroblastoma, differential diagnosis with DSRCT was not difficult: Clinical and morphologic data are not similar for these two entities. The current study validates WT1 immunoreactivity as a useful marker to separate DSRCT from other small round cell tumors.

In this report we describe three patients with developmental cortical abnormalities (generally referred as cortical dysplasia), revealed by MRI and operated on for intractable epilepsy. Tissue, removed for strictly therapeutic reasons, was defined as the epileptogenic area by electroclinical data and stereo EEG (SEEG) recordings. Tissue samples were processed initially for histology, and selected sections were further processed for immunocytochemical investigation in order to determine whether the region of cortical dysplasia was co-extensive with the epileptogenic area. In two patients with nodular heterotopia, disorganized aggregates of neurons (as revealed by neuronal cytoskeletal markers) were found within the nodules. Both pyramidal and local circuit neurons were present in the nodules, but no reactive gliosis was present. When nodules reached the cortex, the cortical layers were disrupted. In the patient with localized cortical dysplasia, a complete disorganization of the cortical lamination was found, and numerous neurons were also present in the white matter. Disoriented pyramidal neurons weakly labelled with cytoskeletal neuronal markers were also present but no cytomegalic cells were found. One of the patients with nodular heterotopia underwent only partial resection of both the 'epileptogenic area' and of the lesion; this patient still presents with seizures. The other patient with nodular heterotopia is seizure-free after a complete lesionectomy and excision of the epileptogenic area. The third patient, with focal cortical dysplasia, had two surgeries; she became seizure-free only after the excision of the epileptogenic area detected by SEEG recording. The present data suggest that the dysplastic areas identified by MRI should not be considered as the only place of origin of the ictal discharges. From the neuropathological point of view, the focal cortical dysplasia can be considered as a pure form of migrational disorder. However, the presence of large aggregates of neurons interspersed within the white matter, in the subcortical nodular heterotopia, suggests that a defect of neuronal migration could be associated with an exuberant production of neuroblasts and/or a disruption of mechanisms for naturally occurring cell death.

Infiltration of regional lymph nodes by macrophages has been shown after total joint arthroplasty. These pelvic lymph nodes were obtained most often from patients during staging procedures for carcinoma and may be a diagnostic pitfall in the frozen section diagnosis of nodal metastasis. We report an unusual case of association in the same lymph node between histiocytosis and prostatic carcinoma metastasis. Histiocytosis was caused by wear debris from two different prostheses. Inductively coupled plasma mass spectrometry verified this diagnosis.

Sarcoidosis rarely presents as an intracranial mass lesion. We report the case of a patient presenting a disturbance of cognitive functions in whom CT and MRI demonstrated a large right fronto-temporal mass lesion with contrast enhancement and surrounding marked oedema, highly suggestive of a brain tumour. The diagnosis of neurosarcoidosis was made after stereotactic biopsy of the lesion.

Patients having a hypothalamic hamartoma frequently present epileptic attacks of laughter, and they later experience multiple additional seizure types, which invariably lead to a severe drug-resistant epilepsy. If this association is now well-known, relationships between the hypothalamic mass and the different types of seizures remain still mysterious. We report the case of a 16-year-old girl suffering from this peculiar epileptic picture, in whom a stereo-EEG study was performed, allowing us to record both the hamartoma, the neighboring hypothalamic structures, and other bilateral cortical areas. It showed that gelastic fits were strictly linked to ictal discharges which began and remained well localized in the hamartoma. Conversely, atonic seizures, which might result from a secondary epileptogenesis, admitted a widely extended bilateral frontal cortical origin, sparing the lesion, and slightly involving the posterior hypothalamus. Stereotactic radiosurgery of the hamartoma proved to be ineffective on both types of seizures, probably because of the too low dose of X-rays delivered (18 grays), as suggested by the absence of hypothalamic mass changes on MRI. Such data, never reported to our knowledge, seem able to contribute to a better understanding of this very peculiar epileptic syndrome, and perhaps to a better adapted therapeutic management.

Summary Two cases of carcinoid tumour complicating inflammatory bowel disease (IBD) are presented. Both tumours were located in the appendiceal tip. The first case occurred in a man with Crohn's disease (CD), and the second one in a woman suffering from ulcerative colitis (UC). Histochemical and immunohistochemical studies were not allowed on case 1 because the tumour was not still present on serial sections of the appendix. On case 2, tumour cells were not reactive with Grimelius and Masson-Fontana stainings, but were strongly stained with anti-keratin and anti-chromogranin monoclonal antibodies (MAb), and faintly expressed neuron specific enolase (NSE) and Leu-7. Both cases occurred in inflammatory or damaged mucosa which exhibited Paneth cell metaplasia and hyperplasia and areas indefinite for dysplasia. Along with these lesions, hyperplasia of enteroendocrine cells was pointed out in the neighbouring appendiceal and colonic mucosa by means of anti-chromogranin MAb. These data suggest that the association of carcinoid tumour with IBD, albeit rare, is not coincidental and is the result of hyperplastic and dysplastic troubles that may involve enteroendocrine cells as well as such other derivatives of digestive stem cells as columnar cells, goblet cells and Paneth cells.

A case of Lhermitte–Duclos disease is reported. Characterized by architectural disruption of the cerebellar folia with disorganization of the normal three layers, the lesion is easily identified using magnetic resonance imaging.

We investigated four mechanisms of intrinsic chemoresistance in a series of 67 human brain tumours including 31 gliomas (one grade I ganglioglioma, nine grade II and 10 grade III astrocytomas, 11 glioblastomas), 13 cerebral metastases, one medulloblastoma, one malignant teratoma, three ependymomas and 18 meningiomas. We studied four genes by northern blotting: multidrug-resistance (MDR 1), glutathione-s transferase (GST pi), dihydrofolate reductase (DHFR), and topoisomerase II (Topo II). The Topo II gene was absent in the normal adult brain (100%) and in 64% of the tumour samples tested. A second gene, GST pi, was found to be overexpressed in 38% of brain tumours. The two other chemoresistance-related genes were occasionally overexpressed in brain tumours (2% for MDR1, 9% for DHFR). Our results provide evidence that chemoresistance is intrinsic to the brain tissue and seems likely to be a multifactorial process.

International audience; Microcystic and lipomatous meningiomas represent two of the recognised rare subtypes of meningiomas. We describe the CT and MR findings in one adult case of each subtype with emphasis on the diagnostic benefits of perfusion-weighted MR imaging (PWI) and/or proton magnetic resonance spectroscopy (MRS).

A 32-year-old immunocompetent female patient, 8 months pregnant, underwent three complex partial seizures beginning with visual hallucinations and secondarily generalized. She had been in good health until then. The pregnancy was normal and no high blood pressure was noted. Clinical examination revealed a left nystagmus, a right sensory hemibody deficiency and a right pyramidal syndrome. MRI showed an isolated intraparenchymal lesion in the left parieto-occipital region with an enhancement of the left lateral ventricle floor (Fig. 1a,b). The biological assessments, including hemogram, ionogram, CRP, liver enzymes and b2 microglobulin, were all normal. An elevated erythrocyte sedimentation rate was noted. CMV, HBV, HCV, and Lyme serologies were negative. Previous HSV, EBV, and varicella zoster virus infection markers were found. CSF examination revealed hyperproteinorachia (0.95 g/L) without cellular reaction. No tumoral cells were found. Thoracic and abdominal CT was normal. After the brain biopsy, a course of corticosteroids was started. The patient did not present with any other seizures. Five years after the first epileptic manifestations, follow-up MRI demonstrated resolution of the enhancing lesions (Fig. 2) and the patient was in good health with 10 mg of corticosteroids daily.

A 52-year-old male presented with an anterior mediastinal tumor associated with a 2-year history of myasthenia gravis. The patient underwent thymectomy and a 185-g, 10 X 8 X 3.5 cm, well-delineated tumor was resected. On histologic examination the tumor proved to be a thymolipoma composed of mature adipose elements containing cords and nests of thymic tissue. The latter consisted mainly of cortical areas, the thymocytes of which displayed an immunohistochemical profile of cortical cells, i.e., CD 1+, CD 4+, CD 8+, and frequently Ki 67+. Ultrastructural study confirmed the predominant cortical differentiation of the thymic component. No germinal centers, dendritic reticulum cells, or myoid cells were detected by histologic, immunohistochemical, and ultrastructural studies. The association of thymolipoma with myasthenia gravis is rare; this case is the 10th reported. Our findings lead us to believe that (a) the cortical differentiation of the thymic component and the active thymocyte proliferation could represent a factor leading to myasthenia gravis; and (b) thymolipoma could be a peculiar form of thymoma rather than a mixed tumor of mesenchymal and entodermal origin, a lipoma, or a hamartoma of the thymic gland. The reported association of thymolipomas with other immune disturbances or with neoplastic conditions usually associated with true thymomas support these findings.

International audience; PURPOSE: To present the imaging and perfusion data obtained in nine patients with pilocytic astrocytomas (PA) and to discuss the original functional issues of this technique. METHOD: Nine patients with pathologically proven PA underwent conventional and perfusion MR imaging. Various areas of relative cerebral blood volume (rCBV) within the tumors were obtained. The maximum rCBV ratios were identified and considered as representative of the tumor. The results were compared with the pathological findings. RESULTS: In all patients, rCBV was

Ependymomas (EP) represent the third most frequent type of central nervous system (CNS) tumor of childhood, after astrocytomas and medulloblastomas. No prognostic biological markers are available, and differentiation from choroid plexus papilloma (CPP) is difficult. The present objective was, for a sample of 27 children with intracranial EP and 7 with CPP, to describe and compare the methylation status of 19 genes (with current HUGO symbol, if any): p15INK4a (CDKN2B), p16INK4a and p14ARF (both CDKN2A), APC, RB1, RASSF1A (RASSF1), BLU (ZMYND10) FHIT, RARB, MGMT, DAPK (DAPK1), ECAD (CDH1), CASP8, TNFRSF10C, TNFRSF10D, FLIP (CFLAR), INI1 (SMARCB1), TIMP3, and NF2. Three adult corteses were used as a control. We detected a similar percentage of methylated tumors in both groups (71% in CPP and 77% in EP). No gene was methylated in that control group. RASSF1A was the most frequently methylated gene in both benign tumors (66%) and EP (56%). The genes associated with apoptosis were methylated in both groups of tumors. The percentages of TRAIL pathway genes (CASP8, TFRSF10C, and TFRSF10D) methylated were 30, 9.5, and 36.4%, respectively, in ependymomas and 50, 50, and 16.7%, respectively, in choroid plexus papillomas. No other gene was methylated in the benign tumors, whereas FHIT was methylated in 22%, RARB in 14.8%, BLU in 13.6%, p16INK4a in 11.1%, TNFRSF10C in 9.5%, and DAPK in 7.4% of ependymomas. Although we did not observe a statistical relationship between methylation and clinical outcome, the methylation pattern does not appear to be randomly distributed in ependymoma and may represent a mechanism of tumor development and evolution.

Desmoplastic infantile ganglioglioma (World Health Organization grade I) is a rare neoplasm. Despite their common large size and spectacular radiologic and histologic features, the prognosis after surgical resection is good. We present a new case of this tumor in a 14-month-old boy with a recent history of intracranial hypertension. Magnetic resonance imaging revealed a large tumor involving the left collateral trigone with dilatation of the lateral ventricles. Surgery revealed two separate solid tumors: one in the left falco-tentorial region and the other in the left rolandic area. Microscopic examination showed a proliferation of neoplastic astrocytes in reticulin-rich desmoplastic stroma associated with scattered ganglion cells. One year after surgery follow-up magnetic resonance imaging did not show tumor progression.

Central nervous system (CNS) solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms recognized less than a decade ago. Approximately 60 cases of SFT have been reported in the central nervous system. We describe three atypical SFTs of the CNS, two intracranial and one within the spine. One intracranial SFT arose from the sella turcica and expanded into the suprasellar areas. It relapsed twice during the 3 years following partial resection, and the MiB 1 labeling index steadily increased without obvious malignant transformation. The second SFT arose from the confluence of the sinuses, widely invaded the lateral sinus and adjacent bones, had a low MiB 1 index and has not recurred after 5 years. The intraspinal tumor occurred at T5-T7 in a patient with multiple café-au-lait spots, was predominantly myxoid and developed a second similar lesion at S3-S5 14 years later. The MiB 1 index was lower in the second tumor. Immunohistochemistry confirmed that all were SFTs. These atypical presentations gave us an opportunity to provide further information about the natural histological course of CNS SFTs.

N-myc amplification is a major prognostic factor in neuroblastomas and is systematically investigated by Southern blot or polymerase chain reaction (PCR). A retrospective study of N -myc amplification has been carried out using fluorescence in situ hybridization (FISH) in 97 fixed neuroblastomas. For each tumour, FISH was performed on the area that contained the most immature neuroblasts. Among these 97 neuroblastomas, 16 were amplified and 12 were not interpretable. FISH was not interpretable in six cases. All neuroblastomas with N-myc amplification detected by Southern blot/PCR were amplified with FISH, except three that were not interpretable. Four tumours that were not interpretable in Southern blot/PCR contained more than five copies of N-myc by FISH: one was aneuploid and three were truly amplified, containing more than ten copies of N-myc. Among these three patients, two died in a short time of their tumours. Ten cases were not amplified by Southern blot/PCR and showed more than five copies by FISH: four were aneuploid and two showed heterogeneous amplification, with a few cells clearly amplified whereas most were not. Four cases were amplified, of which two patients died of their tumours. This study confirms that when applied to the most immature areas of fixed neuroblastomas, FISH displayed a higher sensitivity than molecular techniques (p0.001) and could detect heterogeneous amplification. FISH could therefore become an important complementary procedure in assessing prognosis in neuroblastomas.

Recent papers suggest that two angiogenic factors (angiopoietin 2 and vascular endothelial growth factor) cooperate in tumoral angiogenesis to support the growing tumor. The purpose of the present work was to demonstrate the existence of such cooperation in a longitudinal study of a brain tumor model during tumor growth by means of immunohistochemistry.The study was performed on 31 rats bearing C6 glioma. At different stages of tumor growth, the histological aspects were described and sections were immunostained for VEGF, Ang-2 and their receptors VEGFR-1, VEGFR-2 and Tie-2. Immunostaining was semi-quantitatively analyzed and the localization of immunostained cells was reported.Ang-2 and Tie-2 were detected in the endothelial cells of vessels surrounded by tumor cells, occuring early in our study, with immunostaining taking place from day 4 to day 24. Immunostaining with VEGF (on tumoral cells) and VEGFR-2 (on endothelial cells) was present after 8 days of tumor growth. A clear increase of vessel density can be observed at the periphery of the tumors after 16 days of tumor growth. At that time, areas of necrosis were present in the tumor with concomitant VEGF and Ang-2 expression.The present study demonstrated cooperation between the early effect of Ang-2 and the secondary effect of VEGF to elaborate new vessels in a longitudinal study of experimental brain tumors. This study is favorable to the new model of tumor angiogenesis, with successive vessel cooption, regression and growth mediated by angiopoietins and VEGF.

Herein we reviewed the histopathological findings in 327 consecutive surgical specimens from patients with pharmaco-resistant epilepsy (PRE). Three major pathological groups (78.3% of all cases) were identified: Ammon's horn sclerosis (85 cases as an isolated lesion plus 18 as one part of a dual pathology), tumors (94 cases), and malformations (77 cases). Tumors, often associated with cortical dysplasias (CDs), were all of low histological grade and included 61 dysembryoplastic neuroepithelial tumors (DNTs), 29 gangliogliomas, and 4 pleomorphic xanthoastrocytomas (PXAs). Among the malformations were observed 52 CDs, 13 cavernomas, 8 cortical tubers, and 4 cysts. The remaining findings consisted of 16 scars (mostly post-traumatic) and 4 Rasmussen's encephalitis. Fifty-one (15.6%) specimens contained non-specific changes, and histological samples from 215 patients with presurgical implantation of electrodes revealed iatrogenic changes. All these figures are in agreement with the most recent and comparable series, and confirm the high incidence of DNTs that appear clearly as the most common tumoral entity in PRE. Our data also support the hypothesis of a close histogenetic relationship between DNT, ganglioglioma, and PXA, with a putative common origin from pluripotential progenitor CD34 positive cells of the subpial granular layer. As for CDs, our study confirms the clinical relevance of two main subtypes: severe CD (or Taylor's type CD) with neuronal cytomegaly and balloon cells, and non-Taylor's type CDs with a better outcome. Eventually, this series demonstrates that most patients have significant histopathological lesions, among which, aspects that are relevant to the diagnostic surgical pathologist are highlighted [published with a complete image database on CD-Rom].

We performed conventional and dynamic susceptibility-contrast MRI imaging in 38 patients with brain tumours: 20 with metastases (breast carcinoma: two; renal carcinoma: five; colorectal carcinoma: one; lung carcinoma: seven; melanoma: five), and 18 with high-grade astrocytomas. We obtained cerebral blood volume (CBV) maps and calculated the relative CBV (rCBV) in different areas using the ratio between the CBV in the pathological area (CBVp) and in the contralateral white matter (CBVn). We calculated the maximum rCBV (rCBVmax) for each tumour and compared the mean rCBVmax in each group of tumours. The mean rCBV of melanoma metastases (5.35+/-2.32, range 3.14-9.23) and of renal carcinoma metastases (8.17+/-2.39, range 5.41-11.64) were significantly greater than those of high-grade astrocytomas (2.61+/-1.17, range 1.3-5.0) ( P=0.002 and

Summary: Purpose: The aim of this study was to correlate the electroclinical and radiologic data with the neuropathologic findings and surgical outcome in epileptic patients with epilepsy and Taylor's focal cortical dysplasia (TFCD) and to characterize further the abnormal intermediate filaments expression in the balloon cell present in the peculiar dysplasia. Methods: We retrospectively selected 13 TFCD patients who underwent surgery for intractable epilepsy with the aim of removing the magnetic resonance (MR)-detectable lesion and/or the epileptogenic zone defined by stereoelectroencephalographic recordings. The surgical specimens were analyzed by means of routine neuropathologic and immunocytochemical studies. Antisera against different intermediate filaments also were used in serial adjacent sections to evaluate their coexpression in balloon cells. Results: Histopathologic abnormalities typical of TFCD were found not only within the MR-visible lesions but also in most of the epileptogenic zones with no MR signal alterations. Furthermore, the MR-visible lesions contained a high proportion of cells with an abnormal expression of intermediate filament proteins. After a long follow-up, 10 of the patients are now seizure free. Conclusions: Our findings indicate that highly epileptogenic zones may correspond to tissue alterations not revealed by neuroimaging. Furthermore, the immunocytochemical data show that the dysplastic tissue detected by MR contained high concentrations of cells filled with abnormal intermediate filaments. The detected colocalization of neuronal and glial markers in balloon cells indicates a failure of cellular commitment during development.

With lymph node enlargement, the possibility of a malignant process such as metastatic carcinoma or lymphoma needs to be excluded. This report describes a 47 year old woman with inguinal lymph node enlargement initially suspicious for lymphoma. Fine needle aspiration findings favoured reactive hyperplasia, but a malignant process could not be excluded. The final histological diagnosis was a foreign body granulomatous inflammatory response as a result of regionally disseminated silicone particles from an over looked metatarsophalangeal joint prosthesis. Because of the large number of joint prostheses world wide, it should be kept in mind that migration of wear particles can create granulomatous inflammation and node enlargement.

A case of a "de novo" ganglioneuroma showing a large area of malignant peripheral nerve sheath tumor (MPNST) is described. The tumor arose in an 11.5-year-old girl with neither stigmata nor family history of von Recklinghausen's neurofibromatosis. In addition, the patient had no previous history of a neuroblastoma or radiation therapy. This report provides new evidence that, although rare, the spontaneous development of an MPNST in a benign ganglioneuroma can occur. Immunohistochemical and electron microscopy studies supported the finding that the spindle cell component was of nerve sheath origin.

A rare case of concurrent plasma cell granulomas (PCG) of the lung and the central nervous system (CNS) is reported. A 30-year-old man was presented with recurrent left headaches lasting for two years. Computerized tomographic (CT) scan and magnetic resonance imaging (MRI) of the head disclosed a process extending from the lateral aspect of the left cavernous sinus to the tentorium cerebelli and the infratemporal fossa through the foramen ovale. At the same time, chest-X ray and CT scan showed three symptomless masses of the pulmonary right lower lobe. Histological examination of cerebral samples and of one of the pulmonary nodules revealed the presence of a fibrous tissue containing numerous lymphocytes and plasma cells as well as remnants of vascular and respiratory structures. Immunohistochemical study proved these cells to be polyclonal. Ultrastructural analysis confirmed the presence of lymphoid cells and failed to disclose any argument for meningioma or histiocytosis X. The differential diagnostic problems of PCG are discussed as well as considerations about clinicopathological features, histogenesis and pathogenesis of inflammatory pseudotumours (IPT).

Magnetic resonance imaging allows the identification of heterotopic gray matter (HGM) in medically intractable partial epilepsies. The relationships between HGM and the epileptogenic zone remain, however, unclear. In a case of a temporo-parietal epilepsy studied by stereo-EEG, interictal and ictal electrical activity of a temporal HGM were recorded, showing: (1) an intralesional electrical activity, (2) the possible presence of asynchronous spikes, and (3) an early but never initial, or isolated, involvement during ictal discharges. This suggests that the presurgical and surgical management of HGM must be guided, as for other lesions, by the coherence existing between ictal clinical and electrical features, and anatomical data.

Neuroepithelial papillary tumor of the pineal region (PTPR) has been described by several groups and recognized by the 2007 World Health Organization Classification of Tumors of the Central Nervous System. The proto-oncogen Blc-2 can function as an apoptosis suppressor and can promote neoplastic transformation. It may also be involved in neuroendocrine differentiation in some tumors. As PTPRs express neuroendocrine markers, we investigated the expression of Bcl-2 in tumoral cells of a new case of PTPR in a 42-year-old woman. Bcl-2 immunostaining was detected in the cytoplasm of the tumoral cells; staining intensity was heterogeneous from cell to cell and more intense in papillary areas. This intense expression of Bcl-2 in one case of PTPR with a high proliferation index (8%) might be related to the malignancy of this neoplasm. It will be interesting to investigate the prognosis impact of Bcl-2 expression in a large series of PTPRs.