Brain Neoplasm

In this review we provide evidence for the existence of genes associated with primary malignant brain tumors. We summarize the current knowledge from studies of familial cancer aggregation, hereditary syndromes, and molecular and cytogenetic studies. The epidemiologic evidence is suggestive but inconclusive for an association between brain tumors and cancers in other family members, including cancers of the breast, lung and colon. Central nervous system (CNS) tumors have been associated with several hereditary syndromes including the Li-Fraumeni cancer family syndrome, neurofibromatosis (types 1 and 2), tuberous sclerosis, nevoid basal cell carcinoma syndrome, familial polyposis, and von Hippel-Lindau disease. Significant studies leading to the recognition of molecular and cytogenetic abnormalities in malignant gliomas are described in detail. The genetic studies conducted thus far suggest a role for inherited susceptibility in some CNS tumors.

The paper reports a quantitative analysis of the perfusion maps of 22 patients, affected by gliomas or by metastasis, with the aim of characterizing the malignant tissue with respect to the normal tissue. The gold standard was obtained by histological exam or nuclear medicine techniques. The perfusion scan provided 11 parametric maps, including Cerebral Blood Volume (CBV), Cerebral Blood Flow (CBF), Average Perfusion (P mean ) and Permeability-surface area product (PS).

Ten dogs with primary (n ¼ 8) and metastatic (n ¼ 2) brain tumours were studied in an attempt to evaluate the diagnostic sensitivity of computed tomography (CT) or magnetic resonance imaging (MRI). Of the clinical signs noticed, seizures (seven of 10), behavioural abnormalities and cognition dysfunction (seven of 10), compulsive walking and circling (six of 10), sensorimotor (five of 10) and neuro-opthalmological (two of 10) dysfunction were the most common. In all 10 animals that finally died of the disease or were killed, the histopathological diagnosis that followed necropsy was taken as a golden standard in the CT or MRI prediction of the histological type of brain neoplasms. In every instance, tumour detection, morphology and histological differentiation were possible with the aid of either CT (seven of 10) or MRI (three of 10) imaging especially after contrast enhancement. Only one CT-evaluated dog, diagnosed as meningioma, was found to be astrocytoma on histopathology. Interestingly, a rare case of cerebellar medulloblastoma was correctly identified in MRI scans.

Congenital tumors of the central nervous system (CNS) are often arbitrarily divided into "definitely congenital" (present or producing symptoms at birth), "probably congenital" (present or producing symptoms within the first week of life), and "possibly congenital" (present or producing symptoms within the first 6 months of life). They represent less than 2% of all childhood brain tumors. The clinical features of newborns include an enlarged head circumference, associated hydrocephalus, and asymmetric skull growth. At birth, a large head or a tense fontanel is the presenting sign in up to 85% of patients. Neurological symptoms as initial symptoms are comparatively rare. The prenatal diagnosis of congenital CNS tumors, while based on ultrasonography, has significantly benefited from the introduction of prenatal magnetic resonance imaging studies. Teratomas constitute about one third to one half of these tumors and are the most common neonatal brain tumor. They are often immature because of primitive neural elements and, rarely, a component of mixed malignant germ cell tumors. Other tumors include astrocytomas, choroid plexus papilloma, primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, and medulloblastomas. Less common histologies include craniopharyngiomas and ependymomas. There is a strong predilection for supratentorial locations, different from tumors of infants and children. Differential diagnoses include spontaneous intracranial hemorrhage that can occur in the presence of coagulation factor deficiency or underlying vascular malformations, and congenital brain malformations, especially giant heterotopia. The prognosis for patients with congenital tumors is generally poor, usually because of the massive size of the tumor. However, tumors can be resected successfully if they are small and favorably located. The most favorable outcomes are achieved with choroid plexus tumors, where aggressive surgical treatment leads to disease-free survival.

Imagerie de perfusion : principes et applications cliniques Abstract Résumé MR and CT perfusion imaging of the brain: principles and clinical applications J Radiol 2007;88:444-71 MR and CT imaging techniques provide both morphological data and functional data. MR and recently CT perfusion have substantially modified the treatment of acute stroke. CT perfusion offers new opportunities to improve the management strategy in vasospasm after subarachnoid hemorrhage. Both are also helpful for the diagnosis of brain tumors and the assessment of treatment effects. Depuis quelques années, l'imagerie médicale donne accès, en plus des données morphologiques, à des données fonctionnelles. L'étude de la perfusion cérébrale, en IRM puis en TDM, a profondément modifié l'approche thérapeutique de l'accident vasculaire cérébral. La TDM de perfusion apparaît particulièrement prometteuse dans le diagnostic précoce et la stratégie thérapeutique du vasospasme compliquant l'hémorragie sous-arachnoïdienne. L'IRM et la TDM aident également au diagnostic et au suivi thérapeutique des tumeurs cérébrales.

Tumeurs de la fosse postérieure Les séquences après injection seront acquises au mieux et dans au moins deux plans de coupes orthogonaux. La séquence de diffusion doit faire partie au même titre que les séquences précédentes, de l'exploration en IRM d'un processus d'allure évolutive de la fosse postérieure. Elle participe, peu fréquemment, mais de façon décisive, à la caractérisation tissulaire, comme dans le cas des kystes épidermoïdes ou des médulloblastomes. Elle a surtout, et le plus souvent, un rôle dans le diagnostic différentiel redressant le diagnostic de lésion d'allure évolutive, vers celui d'abcès ou d'accident vasculaire cérébral. Dans le cas d'une lésion extra-axiale siégeant dans les citernes, il est nécessaire d'acquérir une séquence en T2 haute résolution Abstract Résumé Infra tentorial tumors.

Introduction Conventional magnetic resonance (MR) imaging has limited capacity to differentiate between glioblastoma multiforme (GBM) and metastasis. The purposes of this study were: (1) to compare microvascular leakage (MVL), cerebral blood volume (CBV), and blood flow (CBF) in the distinction of metastasis from GBM using dynamic susceptibility-weighted contrast-enhanced perfusion MR imaging (DSC-MRI), and (2) to estimate the diagnostic accuracy of perfusion and permeability MR imaging.

To delineate the causes of death (COD) in adults with supratentorial glioblastoma multiforme (GM) we reviewed 117 consecutive cases examined at autopsy over a nineteen year period at the University of Washington. Twenty cases (17%) had expired unexpectedly without ante mortem diagnosis, 5 patients (4%) had been diagnosed as having lower grade astrocytomas prior to death. Other than the 20 patients without ante mortem diagnosis, all patients had a surgical procedure for treatment and/or diagnosis (biopsy 10%, craniotomy 90%). Postsurgical therapy varied, but there was no significant difference in median length of survival among the different treatment groups. Factors considered as potential COD were: herniation (axial, transtentorial, subfalcine, tonsillar), surgical complications (death within thirty days of surgery secondary to cerebral hemorrhage and/or edema), severe systemic illness, brainstem invasion by tumor, and neutroninduced cerebral injury (cerebral and brainstem gliosis were evident in these cases). A potential COD could be identified in 93% of patients. Patients with no ante mortem diagnosis were likely to have herniated (p = 0.01), whereas patients who underwent neutron irradiation were unlikely to have herniated (p = 0.001). No other variables were statistically significant predictors of herniation, including multifocal tumors (20 patients), and brainstem invasion by tumor (18 patients). No patients died as a result of treatment except those who underwent neutron radiotherapy and those who died postoperatively. Although significant mass effect, as evidenced by herniation, was apparent in 61% of patients, most of these patients had an additional identifiable COD. We conclude that the COD in patients with GM varies and is often multifactorial.

Glioblastoma Multiforme (GBM) is an aggressively invasive brain neoplasm with poor patient prognosis. We have previously shown that the bioactive lipid sphingosine-1-phosphate (S1P) stimulates in vitro invasiveness of GBM cells and that high expression levels of the enzyme that forms S1P, sphingosine kinase-1 (SphK1), correlate with shorter survival time of GBM patients. We also recently showed that S1P induces expression of CCN1 (also known as Cyr61), a matricellular protein known to correlate with poor patient prognosis, in GBM cells. In this study we further explored the role of CCN1 as well as the urokinase plasminogen activator (uPA), a protein known to stimulate GBM cell invasiveness, in S1P-induced invasion using a spheroid invasion assay. We also investigated the roles of various S1P receptors in stimulating invasiveness through these pathways. S1P induced expression of uPA, and its receptor uPAR in GBM cells. While S1P 1-3 receptors all contribute at least partially, S1P 1 overexpression led to the most dramatic induction of the uPA system and of spheroid invasion, even in the absence of added S1P. Furthermore, neutralizing antibodies directed against uPA or CCN1 significantly decreased both basal and S1P-stimulated GBM cell invasiveness. Inhibition of SphK blocked basal expression of uPA and uPAR, as well as glioma cell invasion, however overexpression of SphK did not augment S1P receptor-mediated enhancement of uPA activity or invasion. Thus, SphK is necessary for basal activity of the uPA system and glioma cell invasion, while S1P receptor signaling enhances invasion, partially through uPA and CCN1.

Radiosurgery techniques permit high doses of single fraction irradiation to be administered to small volumes of tumor with relative sparing of surrounding brain tissue. The tolerance of surrounding normal brain tissue to dose distributions from linear accelerator radiosurgery with different collimator sizes is an important factor that must be estimated by anyone using these treatment techniques. The exponential and linear quadratic versions of the integrated logistic formula were used to estimate the probability of brain necrosis at different doses for radiosnrgical dose distributions administered by a 6 MV linear accelerator with a 5 arc technique for collimator sizes from 12.5 to 30 mm in diameter. Dose-volume isoeffect curves for a 3% risk of brain necrosis from linear accelerator radiosnrgery were then calculated. These curves approximate those calculated for gamma knife radiosurgery and a published 1% dose-volume isoeffect line predicted for proton beam irradiation. Similar dose-volume isoeffect curves were calculated for single fraction radiosurgery boosts administered after 30 Gy of whole brain irradiation in 12 fractions. The integrated logistic formula appears to be a useful tool for estimating tolerance and providing guidelines for prescribing radiation doses for linear accelerator radiosurgery.

Objective: The objective of this study was to analyze a series of patients harboring a tuberculum sellae meningioma with regard to clinical presentation and long-term functional outcome. Methods: Data in a consecutive series of 62 patients harboring a tuberculum sellae meningioma treated microsurgically between 1990 and 2003 were retrospectively reviewed. Results: The mean age of the 46 women and 16 men enrolled in the study was 53 years (range = 29-81 years). The presenting symptom was visual compromise in 87.1% of the patients, and examination revealed decreased visual acuity in 79% and impaired visual fields in 64.5% of the patients. In addition, 14.5% of the patients had preoperative hormonal abnormalities. Simpson grades I and II resections, usually via a pterional approach, were achieved in 90.3% of the patients. Postoperatively, vision improved in 53.2%, remained unchanged in 29.8%, and deteriorated in 17.0% of the patients. The intraoperative finding predicting an unfavorable visual outcome was a thin atrophic optic nerve, encasement of the nerve, or tumor adhesion to its undersurface. Of the patients, 12.9% required permanent postoperative hormonal replacement. After a mean follow-up period of 6.0 years (range = 18 months-14 years), 88.7% of the patients resumed normal life activity and 2 recurrent tumors were observed (3.2%) and reoperated. Conclusions: Preoperative magnetic resonance imaging provides reliable information with regard to dislocation of critical vascular structures. However, the relationship between optic nerves and tumors (eg, adhesion and encasement) affected postoperative results and can only be fully appreciated during microsurgery. Visual outcome may be improved by preserving the microvasculature supplying the optic apparatus. D

To measure the accuracy of single-voxel, image-guided proton MR spectroscopy in distinguishing normal from abnormal brain tissue and neoplastic from nonneoplastic brain disease. METHODS: MR spectroscopy was performed at 0.5 T with the point-resolved spectroscopic pulse sequence and conventional postprocessing techniques. Subjects consisted of a consecutive series of patients with suspected brain neoplasms or recurrent neoplasia and 10 healthy adult volunteers. Fifty-five lesions in 53 patients with subsequently verified final diagnoses were included. Spectra were interpreted qualitatively by visual inspection by nonblinded readers (prospectively) with the benefit of prior clinical data and imaging studies, and by blinded readers (retrospectively). The nonblinded readers interpreted the spectra as diagnostic or not, and, if diagnostic, as neoplastic or nonneoplastic. The blinded readers classified the spectra as diagnostic or not, and, if diagnostic, as normal or abnormal and as neoplastic or nonneoplastic (when abnormal). The sensitivity, specificity, positive and negative predictive values, and accuracy were calculated from blinded and nonblinded MR spectroscopy interpretations. A receiver operator characteristic (ROC) curve analysis was performed on blinded MR spectroscopy interpretations. RESULTS: The diagnostic accuracy averaged across four blinded readers in differentiating patients from control subjects was .96, while the area under the aggregate (pooled interpretations) ROC curve approached unity. Accuracy in the nonblinded and blinded discrimination of neoplastic from nonneoplastic disease was .96 and .83, respectively. The area under the aggregate ROC curve in the blinded discrimination of neoplasm from nonneoplasm was .89. CONCLUSIONS: Image-guided proton spectra obtained at 0.5 T from patients with suspected neoplasia can be distinguished from spectra in healthy control subjects, and neoplastic spectra can be distinguished from nonneoplastic spectra with a high degree of diagnostic accuracy.

Phase II Central nervous system neoplasms Brain neoplasms Platelet-derived growth factor alpha receptor C-kit Drug toxicity Pharmacokinetics A B S T R A C T Purpose: To evaluate the safety, efficacy and pharmacokinetics of imatinib in children with recurrent or refractory central nervous system (CNS) tumours expressing KIT and/or PDG-FRA. Methods: Nineteen patients aged 2-18 years, with recurrent or refractory CNS tumours expressing either of the target receptors KIT and/or PDGFRA (by immunohistochemistry) were eligible. Participants received imatinib orally at a dose of 440 mg/m 2 /day and toxicities and tumour responses were monitored. Serial blood and cerebrospinal fluid samples for pharmacokinetics were obtained in a subset of consenting patients. Frozen tumour samples were analysed retrospectively for KIT and PDGFRA gene amplification in a subset of patients for whom samples were available. Results: Common toxicities were lymphopaenia, neutropaenia, leucopaenia, elevated serum transaminases and vomiting. No intratumoural haemorrhages were observed.

RESUMO -Realizou-se análise de 22 pacientes portadores de tumores e lesões vasculares (malformações arteriovenosas e aneurismas) das regiões posteriores do giro parahipocampal e do pulvinar do tálamo e operados pelo acesso supratentorial-infraoccipital. Em quinze pacientes com tumor, a ressecção foi completa em seis e parcial em nove. Os cinco pacientes com malformação arteriovenosa foram submetidos a exérese total da lesão. Realizou-se também o estudo anatômico deste acesso em cinco segmentos cefálicos (dez lados). Os resultados cirúrgicos e do estudo anatômico sugerem que o acesso supratentorial-infraoccipital representa opção para abordar lesões do pulvinar do tálamo e do lobo temporal posteromedial que afloram na fissura transversa do cérebro.

While MRI has been instrumental in significantly improving care in children harbouring brain tumours, conventional sequences lack information regarding functional parameters including cellularity, haemodynamics and metabolism. Advanced MR imaging modalities, such as diffusion (including diffusion tensor imaging and fibre tractography), perfusion and spectroscopy have significantly improved our understanding of the physiopathology of brain tumours and have provided invaluable additional information for treatment planning and monitoring of treatment results. The contribution of these methods to the characterization of brain neoplasms in children is the focus of the present manuscript.

Purpose: To delineate the magnetic resonance (MR) appearance of leptomeningeal melanosis in children. Method: Retrospective review of the medical, surgical, pathologic and MR findings in four children with a confirmed histologic diagnosis of leptomeningeal melanosis. The brain MR was performed with T1-and T2-weighted images in all four children and three had Tl-weighted post-gadolinium images. Two of the children also had Tl-weighted post gadolinium spine images. Results: The MR brain findings consisted of cortical plaque and nodular hyperintense lesions on the noncontrast Tl-weighted images in one child, marked, diffuse leptomeningeal enhancement in two children, and an enlarged, hypointense left temporal lobe with adjacent mild leptomeningeal enhancement in one child. The MR spine findings consisted of diffuse leptomeningeal enhancement in one child and hyperintensity of the cerebrospinai fluid in the other. Conclusion: The Tl-weighted pre-and post-contrast images were the best to demonstrate the MR findings in leptomeningeal melanosis which consisted of either diffuse or localized enhancement of the leptomeninges; or cortical plaque and nodular hyperintense lesions pre-contrast.

Introduction The purpose of this study was to compare the non-invasive 3D pseudo-continuous arterial spin labelling (PC ASL) technique with the clinically established dynamic susceptibility contrast perfusion magnetic resonance imaging (DSC-MRI) for evaluation of brain tumours. Methods A prospective study of 28 patients with contrastenhancing brain tumours was performed at 3 T using DSC-MRI and PC ASL with whole-brain coverage. The visual qualitative evaluation of signal enhancement in tumour was scored from 0 to 3 (0 = no signal enhancement compared with white matter, 3 = pronounced signal enhancement with equal or higher signal intensity than in grey matter/basal ganglia). The extent of susceptibility artefacts in the tumour was scored from 0 to 2 (0 = no susceptibility artefacts and 2 = extensive susceptibility artefacts (maximum diameter>2 cm)). A quantitative analysis was performed with normalised tumour blood flow values (ASL nTBF, DSC nTBF): mean value for region of interest (ROI) in an area with maximum signal enhancement/ the mean value for ROIs in cerebellum. Results There was no difference in total visual score for signal enhancement between PC ASL and DSC relative cerebral blood flow (p=0.12). ASL had a lower susceptibility-artefact score than DSC-MRI (p=0.03). There was good correlation between DSC nTBF and ASL nTBF values with a correlation coefficient of 0.82. Conclusion PC ASL is an alternative to DSC-MRI for the evaluation of perfusion in brain tumours. The method has fewer susceptibility artefacts than DSC-MRI and can be used in patients with renal failure because no contrast injection is needed.

The treatment outcome of 24 patients with pathologically-proven non-germinomatous germ cell tumor was retrospectively investigated to determine the effectiveness of radiotherapy. The patients were divided into three groups as follows: group 1, five patients with mature teratoma with or without germinoma; group 2, six patients with immature teratoma with or without germinoma; group 3, 13 patients with other highly malignant tumors. The overall actuarial survival and relapse-free rates at 5 years were 82% and 59%, respectively, with a median follow-up period of 62 months. The actuarial relapse-free rate at 5 years was 100% for group 1, 63% for group 2 and 44% for group 3. There was no difference in the relapse-free rates between total resection and partial resection. Usage of chemotherapy was adversely related to survival probably due to selection bias. No local failure was observed with 10 Gy or more for group 1, 40 Gy or more for group 2 and 54 Gy or more for group 3. In groups 1 and 2, there was no spinal relapses without craniospinal irradiation. In group 3, three of eight patients who did not receive craniospinal irradiation and none of five patients who received craniospinal irradiation experienced spinal relapse. In conclusion, highly malignant GCTs show a high incidence of spinal metastasis and craniospinal irradiation may reduce the risk of spinal metastasis. Radiation dose and volume are to be determined according to the histopathological aggressiveness.

Purpose: To quantitatively evaluate dose conformity achieved using Gamma Knife radiosurgery, compare results with those reported in the literature, and evaluate risk factors for complications. Methods and Materials: All lesions treated at our institution with Gamma Knife radiosurgery from May 1993 (when volume criteria were routinely recorded) through December 1998 were reviewed. Lesions were excluded from analysis for reasons listed below. Conformity index (the ratio of prescription volume to target volume) was calculated for all evaluable lesions and for lesions comparable to those reported in the literature on conformity of linac radiosurgery. Univariate Cox regression models were used to test for associations between treatment parameters and toxicity. Results: Of 1612 targets treated in 874 patients, 274 were excluded, most commonly for unavailability of individual prescription volume data because two or more lesions were included within the same dose matrix (176 lesions), intentional partial coverage for staged treatment of large arteriovenous malformations (AVMs) (33 lesions), and missing target volume data (26 lesions). The median conformity indices were 1.67 for all 1338 evaluable lesions and 1.40 -1.43 for lesions comparable to two linac radiosurgery series that reported conformity indices of 1.8 and 2.7, respectively. Among all 651 patients evaluable for complications, there were one Grade 5, eight Grade 4, and 27 Grade 3 complications. Increased risk of toxicity was associated with larger target volume, maximum lesion diameter, prescription volume, or volume of nontarget tissue within the prescription volume. Conclusions: Gamma Knife radiosurgery achieves much more conformal dose distributions than those reported for conventional linac radiosurgery and somewhat more conformal dose distributions than sophisticated linac radiosurgery techniques. Larger target, nontarget, or prescription volumes are associated with increased risk of toxicity.

Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma (NHL) confined to the brain, leptomeninges, eyes, or spinal cord. The majority of PCNSL cases occur in the immunocompetent host, the focus of this review. The prognosis of PCNSL is inferior to that of other NHL subtypes including other organ-specific subtypes of extranodal NHL. The 5-and 10-year survival proportions for PCNSL are 29.3% and 21.6%, respectively. The diagnosis and management of PCNSL differs from that of other primary brain cancers and NHL in other parts of the body. Learning Objectives • The reader will become familiar with the consensus criteria for the diagnosis of primary PCNSL • The reader will become familiar with the consensus criteria for assessment of treatment response in PCNSL • The reader will become familiar with the treatment options for newly diagnosed PCNSL as defined in randomized clinical trials • The reader will understand the options for the treatment of relapsed PCNSL Epidemiology Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma (NHL) confined to the brain, leptomeninges, eyes, or spinal cord. 1 PCNSL accounts for~2% of all primary central nervous system tumors, with patients' median age of 65 years at diagnosis. 2 Since 2000, there has been an increase in the overall incidence of PCNSL, especially in the elderly. Pathology Approximately 90% of PCNSL cases are diffuse large B-cell lymphomas (DLBCL), with the remainder consisting of T-cell lymphomas, poorly characterized low-grade lymphomas, or Burkitt lymphomas. 3 Although the incidence of Epstein-Barr Virus (EBV) is high in immunocompromised hosts, virtually all tumor specimens from immunocompetent hosts are EBV-negative. More than 90% of primary CNS DLBCL closely resemble the activated B cell-like (ABC) subtype. Like systemic DLBCL, PCNSL harbors chromosomal translocations of the BCL6 gene, deletions in 6q, and aberrant somatic hypermutation in proto-oncogenes including MYC and PAX5. Inactivation of CDKN2A is also commonly observed in both entities. Gene expression profiles demonstrate that PCNSL is characterized by differential expression of genes related to adhesion and extracellular matrix pathways, including MUM1, CXCL13, and CHI3L1. The ongoing somatic hypermutation with biased use of V H gene segments that has been observed in PCNSL is suggestive of an antigen-dependent proliferation. These observations are consistent 25 Methotrexate, procarbazine, vincristine, rituximab 5 .

The objective of this retrospective study was to evaluate the relative effect of surgery and radiotherapy (RT) on the survival of 25 consecutive children and young adults treated for ependymoma [18 in the posterior fossa (PF), 2 in supratentorial locations (ST) and 5 in intraspinal locations (IS)]. Five-year survival rates for patients with PF, ST and IS tumors were 28%, 0% and 100%, respectively. Total tumor resection was a positive prognostic factor in the case of PF tumors. No patients with subtotal removal of a PF tumor survived for longer than 5 years. The effect of RT on survival of patients with PF ependymomas in this series was uncertain. No patients with PF ependymoma had disseminated disease at diagnosis, and all tumor recurrences were local. Based on these observations, we see no indications for craniospinal RT of PF Keywords Brain neoplasm · Ependymoma · Survival · Surgery · Radiotherapy Child's Nerv Syst (2001) 17:24-30

Summary The majority of intracranial chondromas arise from cartilage rests in the synchondrosis at the base of the skull. Chondromas are most commonly found in the sellar and parasellar regions, usually located extradurally. In rare instances, these tumours originate from the dura mater of the convexity. We report a rare case of a chondroma arising from the convexity dura mater. The origin of this tumour is analysed and the literature reviewed. ª Summary Pelvic schwannoma is a rare cause of sciatic pain. We report a case of retroperitoneal pelvic schwannoma presenting with chronic sciatica which was diagnosed and monitored radiologically for several years before successful surgical resection. ª

A 12-year-old girl was admitted (in November 1994) with a 2-year history of headache (worsening over the last 2 months) and nausea and vomiting of 2 weeks' duration. Neurological examination revealed a slight left-sided hemiparesis and bilateral papilloedema Child's Nerv Syst (1998) 14: 350-353 Abstract Meningiomas of the lateral ventricles of the brain are rare tumours, accounting for approximately 0.5-5% of all intracranial meningiomas. Their natural history and symptomatology and the possibilities of early diagnosis are presented. The intraventricular location of the slow-growing benign mass provides a compensatory mechanism in the form of reserve space, which contributes to the delay in clinical demonstration of symptoms and signs. This makes the choice of diagnostic procedure an essential prob-lem. CT and MRI are useful in detecting these masses, and magnetic resonance angiography (MRA) has also proved to be of great value in demonstrating the vascular supply of the tumour. This paper deals with two cases. In case 1 CT, MRI and MRA and in case 2 CT examination proved to be very useful. The tumours were removed by a transcortical approach in the posterior area.

In 216 patients who underwent stereotactic irradiation (STI), MRI was performed 4-12 times for 17-29 months after STI. Frequency of new radiation-induced lesions more than 2 cm in diameter on sequential MRI following STI, and characteristics on sequential MRI findings were investigated. Ten patients displayed new lesions following STI. These patients comprised eight of 208 patients with intracranial neoplasm and two of seven patients with AVM. Lesions appeared 1-12 months after STI. The lesions appeared near primary lesions in nine patients. Contrast-enhancement was seen along the cerebral cortex in three patients. Sequential MRI is effective in detecting radiationinduced lesions following STI. q

To determine the accuracy of relative cerebral blood volume (rCBV) fraction for distinguishing high-grade recurrent neoplasm from treatment-related necrosis (TRN) in enhancing masses identified on surveillance magnetic resonance (MR) images following treatment for primary or secondary brain neoplasm.

Ex-vivo and in-vitro nuclear magnetic resonance (NMR) spectroscopy techniques have been used for studying chemical metabolites in surgically resected specimens of human neoplasms, and may provide complementary information to in-vivo whole-body magnetic-resonance spectroscopy (MRS). We describe an ex-vivo NMR in water method for measurement of water-soluble metabolites in unprocessed normal rat brain tissue and human intracranial neoplasms. The NMR spectra obtained using the method described here were comparable to those obtained using high-resolution magic-angle spinning (HRMAS) NMR methods, with good correlation in metabolite concentrations relative to creatine (r 2 = 0.7635). Improved spectral resolution and baseline were noted compared to HRMAS, but macromolecule resonances were not detected. Ex-vivo NMR of unprocessed tissue in water is rapid and technically simple to perform, and has the potential to be used for direct assessment of intracranial neoplasms.

Objective: The aim of this study is to select the risk factors for local failure requiring salvage neurosurgery in patients with brain metastases treated with stereotactic radiosurgery in a single institution. Methods: The follow-up of 123 patients, with 255 brain ...

Congenital tumors of the central nervous system (CNS) are often arbitrarily divided into "definitely congenital" (present or producing symptoms at birth), "probably congenital" (present or producing symptoms within the first week of life), and "possibly congenital" (present or producing symptoms within the first 6 months of life). They represent less than 2% of all childhood brain tumors. The clinical features of newborns include an enlarged head circumference, associated hydrocephalus, and asymmetric skull growth. At birth, a large head or a tense fontanel is the presenting sign in up to 85% of patients. Neurological symptoms as initial symptoms are comparatively rare. The prenatal diagnosis of congenital CNS tumors, while based on ultrasonography, has significantly benefited from the introduction of prenatal magnetic resonance imaging studies. Teratomas constitute about one third to one half of these tumors and are the most common neonatal brain tumor. They are often immature because of primitive neural elements and, rarely, a component of mixed malignant germ cell tumors. Other tumors include astrocytomas, choroid plexus papilloma, primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, and medulloblastomas. Less common histologies include craniopharyngiomas and ependymomas. There is a strong predilection for supratentorial locations, different from tumors of infants and children. Differential diagnoses include spontaneous intracranial hemorrhage that can occur in the presence of coagulation factor deficiency or underlying vascular malformations, and congenital brain malformations, especially giant heterotopia. The prognosis for patients with congenital tumors is generally poor, usually because of the massive size of the tumor. However, tumors can be resected successfully if they are small and favorably located. The most favorable outcomes are achieved with choroid plexus tumors, where aggressive surgical treatment leads to disease-free survival.

Background A number of tools, including intraoperative ultrasound, are reported to facilitate surgical resection of high-grade gliomas. However, results from selected surgical series do not necessarily reflect the effectiveness in common neurosurgical practice. Delineation of seemingly similar brain tumours vary in different ultrasound-guided operations, perhaps limiting usefulness in certain patients. Methods We explore and describe the results associated with use of the SonoWand system with intraoperative ultrasound in a population-based, unselected, high-grade glioma series. Surgeons filled out questionnaires about presumed extent of resection, use of ultrasound and ultrasound image quality just after surgery. We evaluate the impact of ultrasound image quality. We also explore the importance of patient selection for surgical results. Results Of 156 consecutive malignant glioma operations, 142 (91%) were resections whilst 14 (9%) were only biopsies. We achieved gross total resection (GTR) in 37% of all high-grade glioma resections, whilst worsening of functional status was seen in 13%. The risk of getting worse was significantly higher in reoperations, resections in eloquent locations, resections in cases with poor ultrasound image quality, resection when surgeons' resection grade estimates were inaccurate and in cases with surgery-related complications. Aiming for GTR, unifocality of lesion, non-eloquent location and medium or good ultrasound image quality were identified as independent factors associated with achieving GTR. Conclusion We report good overall results, both in terms of resection grades and functional outcome in consecutive malignant glioma resections, in which intraoperative ultrasound was used in 95%. We observed a seeming dose-response relationship between ultrasound image quality and clinical and radiological results. This may suggest that better ultrasound facilitates better surgery. The study also clearly demonstrates that, in terms of surgical results, the selection of patients seems to be much more important than the selection of surgical tools.

This paper reviews the scientific literature published from 1986 to 1999 assessing the relationship between dietary factors and brain tumors in adults. The work aimed to describe the estimated associations and to discuss methodological aspects that might influence the results. The studies generally appear to show a moderate association between dietary factors and brain tumors. There is evidence that N-nitroso compounds enhance the risk of developing such tumors and that consumption of fruits and vegetables can inhibit them. Use of proxies in most of the studies may have introduced bias, thereby contributing to some inconsistent observations. Epidemiological research on diet and brain tumors should consider other components of food besides N-nitroso compounds. It is important to carefully assess exposure periods and to prevent bias related to control selection and recall.

A reliable prognostic score for cancer patients with brain metastases submitting to stereotactic radiosurgery (SR) was established. Between July 1993 and December 1995, 34 cancer patients (36 treatments and 69 lesions) with brain metastases were treated with SR at the Hospital Israelita Albert Einstein of São Paulo, Brazil. The exclusion criteria were: more than 5 lesions, any lesion larger than 30 cm3, Karnofsky performance status (KS) less than 50, clinical evidence urging neurosurgical intervention or very poor overall prognosis due to fast progressive systemic disease (SD). The score index for stereotactic radiosurgery of brain metastases (SIR) was obtained by summing up the points of the five variables: age, KS, SD, size (LS), and number of lesions (NL). The Kaplan–Meier method together with log rank was used to estimate and test correlation between survival and all prognostic factors. Applying the SIR definition to our patients' survival data through the Kaplan–Meier method we found out that the expected median survival for patients with 6 points or less was 3.67 months and for those with more than 6 points it was 16.16 months (p = 0.0005). Age, SD, KS, and NL were also significantly correlated with the prognosis (p-values at log-rank test of 0.0020, 0.0071, 0.0181, and 0.0454, respectively). Analyzing our data we concluded that the SIR is a reliable prognostic score for cancer patients with brain metastases submitting to SR. This index should be tested for larger series.

The objective of this study was to present survival data and outcome status in the long-term survivors of a consecutive series of 111 children and young adults treated for posterior fossa medulloblastoma in our departments from 1960 to 1997. The total surgical mortality was 13%. The surgical mortality rate declined significantly during the time period overall, from 23% before 1970 to 0% after 1990. The 5-year survival rate for patients treated between 1960 and 1973 was 0%, while 5-year survival for patients treated after the introduction of systematic craniospinal radiation in 1974 was 53% . Thirty-four patients were alive at the close of this study, with a mean observation time of 13.5 years.