Clinical, radiological, pathological and prognostic aspects of intraventricular oligodendroglioma: comparison with central neurocytoma.

Studies comparing intraventricular oligodendroglioma (IVO) and central neurocytoma (CN) in terms of their clinical, radiological and pathological features are scarce. We, therefore, investigated the similarities and differences between these types of tumors to get a better understanding of how they may be more properly diagnosed and treated. The clinical manifestations, CT/MRI findings, pathological characteristics and clinical outcomes of 8 cases of IVOs and 12 cases of CNs were analyzed retrospectively. Both IVO and CN occurred most commonly in young adults and manifested with symptoms of increased intracranial pressure secondary to obstructive hydrocephalus. However, they were radiologically different in location (p = 0.007), diffusion-weighted imaging (p = 0.001), "scalloping" appearance (p = 0.006), flow void sign (p = 0.006) and ventricular wall invasion (p = 0.000). Histologically, significant differences in mitotic count (p = 0.008) and parenchymal infiltration (p = 0.01) were noted. Immunohistochemically, significant differences in the expression of Olig2 (p = 0.000), Syn (p = 0.01) and NeuN (p = 0.000) were observed. In addition, MIB-1 labeling index (p = 0.035) and case fatality rate (p = 0.021) of IVO were much higher than those of CN, while survival rate of IVO was much lower than that of CN (p = 0.028). IVO and CN are similar in onset age and clinical manifestations, but have different imaging and pathological features. Patients with IVOs may have a relatively poorer prognosis compared to those with CNs.

'Goose bumps' as presenting feature of intraventricular glioblastoma multiforme.

'Goose-bumps' seizures are rare manifestations of epilepsy. They are rarely reported by patients and can be easily dismissed by clinicians. Clinically, it carries some diagnostic localising value especially with unilateral onset. In this report, we present a case of intraventricular glioblastoma multiforme with ipsilateral goose bumps and review the literature.

Secondary normal pressure hydrocephalus in a patient with isolated frontal dilatation--an insight into pathophysiology?

Current theories of the pathophysiology of normal pressure hydrocephalus suggest the classical symptoms are a consequence of disruption of normal frontal function. We present the case of a 70-year-old patient with an isolated, frontal dilatation of his lateral ventricles in the presence of a complete triad as supportive of these theories.

[Identification of highly specific electroencephalographic patterns in patients with neuroepithelial tumors of the III ventricle by algorithmic methods].

An algorithm was suggested for identifying highly specific electroencephalographic (EEG) patterns in neurooncologic patients. The algorithm provides selection of patients with their further classification into main and control groups based on the already existing database of EEG indicators; requests to it; generation of mono-indicator candidates for EEG-patterns on the basis of a 4-dipole table for selecting and verifying sensitive and specific EEG patterns and outlining the best ones. Our material included 368 patients with basal-diencephalic tumors. Algorithmic methods revealed new EEG patterns in patients with different anatomical and topographical variants of neuroepithelial tumors in the III ventricle. We think it reasonable to use the revealed syndromes to improve diagnosis and identify pathophysiological basis of clinical syndromes.

An unusual presentation of colloid cyst--implications for lifestyle advice.

Colloid cysts are rare intracranial neoplasms which typically present with headaches. There is risk of neurological deterioration or death due to acute hydrocephalus. We report a case of colloid cyst presenting after a sudden acceleration/deceleration force from a theme park ride, highlighting the importance of lifestyle advice in these patients.

Diagnosis and Therapeutic Management of Ventricular Gangliogliomas: An Illustrated Review.

BACKGROUND: Gangliogliomas (GGs) are extremely rare benign neoplasms frequently located within the temporal lobe that usually present with seizures. GGs growing predominantly within the ventricular system (VGGs) are even more infrequent, so definite conclusions concerning their diagnosis and therapeutic management are lacking. METHODS: A retrospective review of case reports of VGGs was performed from the introduction of modern imaging techniques, including 4 new illustrative cases treated in our department. RESULTS: Thirty-four cases were collected. Ages ranged from 10 to 71 years (mean, 26.62 years), and 55.9% were male. Most patients developed symptoms related to high intracranial pressure. The lateral ventricles were predominantly involved (58.8%). Obstructive hydrocephalus was observed in 54.5% of patients. Cystic degeneration and calcification were frequently observed. Surgical treatment was carried out in all cases. Morbidity and mortality were 17.6% and 2.9%, respectively. Gross total tumor resection was achieved in 64.5% of patients. Four patients experienced tumor dissemination along the neural axis. More than 90% of patients maintained a good functional status at last follow-up. CONCLUSIONS: Despite their low incidence, a diagnosis of VGGs should be considered in young male adults who progressively develop intracranial hypertension, caused by a ventricular mass showing signs of cystic degeneration and calcification. Maximal and safe surgical resection represents the gold standard for the treatment of symptomatic VGGs, although total removal is frequently precluded by difficulties in defining appropriate tumor boundaries. Adjuvant radiotherapy should be considered if an incomplete resection was carried out, especially in World Health Organization grade III neoplasms.

Stem cell associated gene expression in glioblastoma multiforme: relationship to survival and the subventricular zone.

Current therapies for glioblastoma (GBM) target bulk tumor through measures such as resection and radiotherapy. However, recent evidence suggests that targeting a subset of tumor cells, so-called cancer stem cells, may be critical for inhibiting tumor growth and relapse. The subventricular zone (SVZ), which lines the ventricles of the brain, is thought to be the origin for the majority of neural stem cells and potentially cancer stem cells. Therefore, we assessed the relationship between tumor contact with the SVZ as determined by MRI, cancer stem cell gene expression and survival in 47 patients with GBM. Using DNA microarrays, we found that genes associated with cancer stem cells were not over-expressed in tumors contacting the SVZ. Contact with the SVZ trended with shorter survival (median 358 versus 644, P = 0.066). Over-expression of CD133 (prominin-1) and maternal embryonic leucine zipper kinase (MELK) was associated with shorter survival, whereas mitogen activated protein kinase 8 (MAPK8) was associated with longer survival (P values 0.008, 0.005 and 0.002 respectively). Thus we found no evidence of a stem-cell derived genetic signature specific for GBM in contact with the SVZ, but there was a relationship between stem cell gene expression and survival. More research is required to clarify the relationship between the SVZ, cancer stem cells and survival.

Relationships between brain tumor and optic tract or calcarine fissure are involved in visual field deficits after surgery for brain tumor.

PURPOSE: Diffusion tensor tractography provides useful information regarding the surgical strategy for brain tumors. The goal of the present study was to analyze relationships between visual field deficits and the locations of brain tumors compared with optic tracts as visualized by tractography, and compared with the calcarine fissure. METHODS: Subjects comprised 11 patients with brain tumor in the occipital lobe or atrium of the lateral ventricle who underwent surgery between October 2006 and February 2009. Tumors were categorized as Type A, with almost all the optic tract in the occipital lobe or atrium of the lateral ventricle running close to and stretched by the brain tumor; and Type B, with the optic tract running at least partially distant to the brain tumor and remaining unstretched. RESULTS: Those type A optic tracts that were laterally compressed by brain tumors (Cases 1-3) displayed hemianopsia after surgery. When the brain tumor was located rostro-medial to the calcarine fissure and optic tracts were compressed caudally by the tumor, lower quadrant hemianopsia remained after surgery (Cases 4, 5). In other cases, the visual field remained or improved to normal after surgery. CONCLUSION: The relationship between optic tracts or the calcarine fissure, and brain tumors in the occipital lobe or atrium of the lateral ventricle is related to visual field deficits after surgery. In particular, those Type A optic tracts that are compressed laterally show hemianopsia of the visual field after surgery.

[Three pediatric cases with choroid plexus papilloma in the fourth ventricle].

Choroid plexus papillomas are rare; they comprise less than 1% of all intracranial tumors. In children, most of these neoplasms arise in the lateral ventricle while in adults they are primarily located in the fourth ventricle. We report 3 children with choroid plexus papilloma in the fourth ventricle; they were one 5-month-old girl and 2 boys aged 8-and 15-years. The baby girl presented with macrocephaly and signs of raised intracranial pressure, the 2 boys exhibited cerebellar signs. On magnetic resonance imaging (MRI) the tumors were well-enhanced with a cauliflower-like contour and hydrocephalus. In one case, diffusion weighted images (DWIs) showed an isointense tumor signal clearly different from the well-known hyperintensity of medulloblastomas, Via suboccipital craniotomy we succeeded in the total or subtotal surgical resection of these tumors and there were no sequela. None of the tumors have recurred in the intervening 2-9 years. In conclusion, choroid plexus papilloma should be included as a differential diagnosis in children presenting with tumors in the fourth ventricle. The contour of the tumor on MRI and its intensity on DWI may be clues for a correct preoperative diagnosis.

Transcallosal approach for intraventricular gliomas: neuropsychological outcome and functionality of the corpus callosum.

The transcallosal approach is commonly used for surgery of lateral and third ventricle lesions. Cognitive deficits due to the transcallosal approach still remain controversial. Even if enormous efforts have been made in order to understand specific functions of the corpus callosum, still little is known. The present study was aimed to evaluate the neuropsychological results and the functionality of the corpus callosum in transferring visual, auditory and tactile information between the two hemispheres in a group of patients who were treated using the transcallosal approach. The study evaluated the neuropsychological status of five selected patients presenting low-grade lesions of lateral ventricles that had not previously undergone surgical treatments and that did not receive radiotherapy and chemotherapy. All patients were administered an extensive neuropsychological testing postoperatively and the interhemispheric transfer of visual, auditory and tactile information was also evaluated. Two patients were tested preoperatively. Incisions of 2.4 cm maximum of the corpus callosum length were operated. The postoperative cognitive profile was normal. In some patients, a postoperative subnormal performance in memory functions was found but it cannot be attributable to the surgical approach given that it was altered even pre-surgically. Small incisions of the corpus callosum preserved the integrity of this anatomical structure in transferring lateralized information between the two hemispheres. The transcallosal approach is a safe surgical route to lateral ventricles lesions and the neuropsychological evaluation of these cases could give new insights in the comprehension of corpus callosum functions.

Trigonal cavernous angioma: a short illustrated review.

PURPOSE: Intraventricular cavernomas are rare. Even more rare are those presenting in the trigone of the lateral ventricles. METHODS: We performed a search of the literature of the last 30 years and identified all cases of intraventricular cavernous angiomas. Trigonal cavernomas were separately identified and analysed. Our search yielded a total of 13 trigonal cavernomas. RESULTS: Of a total of 61 intraventricular cases, 13 were located in the trigone of the lateral ventricles. The most prominent presenting symptom was intracranial hypertension (68.9%), followed by seizures (18.2%) and hemorrhage (13.1%).The literature review revealed a trend of intraventricular cavernomas to present with intracranial hypertension rather than seizures or focal neurologic deficit, unlike their intraparenchymal counterparts. We feel that this difference has received little attention in the international literature. We discuss a possible pathogenetic mechanism for the presence of intracranial hypertension and address different aspects of diagnosis and treatment of this benign lesion. CONCLUSIONS: Trigonal cavernomas are benign lesions that have an excellent outcome after radical excision. Symptoms and signs of intracranial hypertension and hydrocephalus may be the prominent initial presentation of this rare ailment.

Comparison of hypothalamopituitary axis dysfunction of intrasellar and third ventricular craniopharyngiomas in children.

In this study, we attempted to determine if different locations of a tumor influence the hypothalamopituitary axis function and outcomes with childhood craniopharyngiomas. The preoperative, postoperative, and long-term follow-up endocrinological disturbances of 66 children with a craniopharyngioma were retrospectively studied. The patients were divided into two subgroups according to the location of the tumor (intrasellar and third ventricle floor). The mean age at onset was 8.02 (range, 1.42-17.58)years. These patients were followed-up for a median duration of 7.2 (range, 2-22)years. Vision problems as the first symptom were more common in Group One (with intrasellar tumors) compared to Group Two (55.6% vs 15.4%; p=0.001; Fisher's exact test). Increased intracranial pressure was the most common initial symptom in patients in Group Two (51.3%) and the second most common symptom in Group One (37%). The majority of patients in both Group One and Group Two required some forms of pituitary hormone supplements (96% vs 84%). At the last follow-up, more patients with intrasellar craniopharyngiomas needed cortisone supplements (79.2% in Group One vs 45.9% in Group Two; p=0.016; Fisher's exact test); however, children with third ventricle floor tumors had more prevalent weight gain (4.2% in Group One vs 27.0% in Group Two; p=0.038; Fisher's exact test). There were different initial presentations and endocrinological outcomes between children with intrasellar and third ventricle floor craniopharyngiomas. The intrasellar tumors had greater pituitary hormone disturbance. However, at the long-term follow-up, children with third ventricle floor tumors had a greater prevalence of being overweight and obese, which was associated with hypothalamic dysfunction.

A patient with subacute onset of ophthalmoplegia, papilledema, and proptosis.

A young man presented with headache, proptosis, and asymmetric ophthalmoplegia. He was found to have a mass in an unusual area of the brain. The differential diagnosis, diagnostic testing, pathology, treatment, and prognosis of this rare tumor are discussed in detail.

Pilomyxoid astrocytoma of the fourth ventricle in an adult.

Pilomyxoid astrocytomas have been identified as a variant of pilocytic astrocytoma. They are listed as a novel clinico-pathological entity in the 2007 World Health Organisation (WHO) classification of tumours of the central nervous system. This tumour corresponds to a WHO grade II neoplasm whereas pilocytic astrocytoma corresponds to WHO grade I. We have encountered an infratentorial tumour with pilomyxoid features in an adult. A 25 year old man presented with tinnitus and hyperacusis. Brain MRI revealed a mass occupying the fourth ventricle. We performed partial resection, but no adjuvant therapy was given. The staining index for the anti-Ki-67 monoclonal antibody MIB-1 was less than 1% in this patient. Pilomyxoid astrocytomas are not limited to the hypothalamic/chiasmatic region in children. Additional knowledge and recognition of this entity is necessary to improve treatment of pilomyxoid astrocytoma.

Meningiomas of the lateral ventricles. A review of 10 cases.

BACKGROUND: Intraventricular meningiomas are rare tumours that represent about 2% of all intracranial meningiomas, and represent one of the most challenging problems in neurosurgery. They are located deep within the brain and often are sizable and highly vascular. We report on a series of 10 meningiomas of the lateral ventricles treated at our institution during the last 28 years. PATIENTS: Ten patients (6 women, 4 men; mean age 41.6 yrs) were admitted to our medical center between 1978-2005 with meningioma of the lateral ventricles. Headache was the first symptom in 8 cases and ocular signs were present in 5 patients. RESULTS: Seven tumours were located in the right ventricle (70%) ranging in size from 2-8 cm, with 7 tumours larger than 3 cm in diameter. Nine patients underwent surgery with total excision in 8 cases and subtotal in the other; the remaining patient only received radiosurgery. CONCLUSIONS: Total resection is the gold standard for treatment which was possible in all but one of the cases undergoing surgery.

[Pediatric intraventricular tumors]. / Gyermekkori intraventricularis agydaganatok.

Pediatric intraventricular tumors present a well circumscribed group from surgical point of view. These tumors growing in the ventricular system cause hydrocephalus in most of the cases, the presenting symptoms are the signs of raised intracranial pressure. The mass lesion may remain silent for a long period, especially in infancy due to compensatory mechanisms, and the tumor might reach extreme size making the surgery a real challenge. This group has very specific postoperative problems resulting from the disturbance of CSF circulation. In this study we present the retrospective analysis of 55 patient operated for intraventricular tumor in the National Institute of Neurosurgery between 1991 and 2006. Data were analysed regarding histological type, presenting symptoms, type of surgical approach, radicalitiy of the resection and postoperative complications. In addition to our own results brief presentation of the specific histological groups is given based on the available literature.

Hypothalamic relapse of a cardiac large B-cell lymphoma presenting with memory loss, confabulation, alexia-agraphia, apathy, hypersomnia, appetite disturbances and diabetes insipidus.

A 37-year-old Hispanic man with a right atrial intracardiac mass diagnosed as diffuse large B-cell lymphoma (DLBCL) was successfully treated with surgery and chemotherapy. During 4 years, several total-body positron emission tomography and MRI scans showed no extracardiac lymphoma. On year 5 after the cardiac surgery, patient presented with sleepiness, hyperphagia, memory loss, confabulation, dementia and diabetes insipidus. Brain MRI showed a single hypothalamic recurrence of the original lymphoma that responded to high-dose methotrexate treatment. Correction of diabetes insipidus improved alertness but amnesia and cognitive deficits persisted, including incapacity to read and write. This case illustrates two unusual locations of DLBCL: primary cardiac lymphoma and hypothalamus. We emphasise the importance of third ventricle tumours as causing amnesia, confabulation, behavioural changes, alexia-agraphia, endocrine disorders and alterations of the circadian rhythm of wakefulness-sleep secondary to lesions of specific hypothalamic nuclei and disruption of hypothalamic-thalamic circuits.

Unusual exophytic subependymoma in the bulbo-cerebellar angle. Case report.

Subependymoma was first described by Scheinker in 1945; it frequently occurs in the ventricles and rarely in the spinal canal representing 0.7% of all central nervous system tumours. Most of these intraventricular tumours are subclinical entities, remaining of small size and discovered at autopsy with 0.4%incidence. We report a case of subependymoma with a completely exophytic growth from the foramen of Luscka: only a similar one has been described in the literature but with a lesser cysternal involvement. Neuroradiological and anatomopathological features of subependymoma are discussed.

Transcavum septum pellucidum interforniceal approach for the colloid cyst of the third ventricle Operative nuance.

BACKGROUND: Despite their unfavorable locations, lesions of the third ventricle can be successfully removed via an interhemispheric, transcallosal approach. In cases with normal ventricular anatomy, this approach requires unilateral or bilateral identification of the foramen of Monro. TECHNIQUE: However, in the presence of abnormal ventricular configuration such as cavum septum pellucidum (CSP), this basic knowledge needs to be modified. After routine callosotomy, there may be a confusion while entering the CSP due to the invisualization of ventricular landmarks such as the foramen of Monro, thalamostriate vein, and choroid plexus. The floor of the CSP is formed by the fornices, and a direct approach to the interforniceal area is easier via the CSP. But the interforniceal approach is not a routine way to reach the third ventricle, which has higher risks than other modalities. CONCLUSION: This approach should be planned and used in selected cases of the CSP. Opening of the walls of CSP is recommended both to expose both the foramen of Monro and to gain safe access to the third ventricle before manipulating the interforniceal area.