Intracranial Location Research Articles

Abstract 5594: Cyclin D1 expression correlates with supratentorial location of ependymomas

Abstract Ependymomas are rare central nervous system tumors. Clinical variables are intercorrelated and usually are not able to predict the disease course. Cyclin D1 is an important regulator of cell cycle and its overexpression has been related with several cancers. The prognostic value of increased cyclin D1 expression in ependymomas has not yet been fully established. We aimed to analyze cyclin D1 gene (CCND1) and protein expression in a large series of ependymomas. We initially performed CCND1 expression in 32 cases of ependymomas. We further analyzed cyclin D1 protein expression by immunohistochemistry of a tissue microarray of 149 samples of ependymomas, corresponding to 121 patients (including relapses). Forty patients were under 18 years and 81 patients were adults (mean 27.2 years). There were 61 intracranial and 60 spinal cases, of which 10 tumors were grade I (myxopapillary), 92 grade II and 19 grade III (anaplastic). Gross total resection was achieved in 62% of cases. Relapse was confirmed in 40.4% of cases (mean follow up of 53 months) and tissue samples of the first surgery and the relapse resection were available for 23 patients. Immunohistochemical reactions were analyzed by two observers independently and final labeling indices (LI) were calculated as the percentage of the positively stained nuclei (negative=0, 0-25%=1, 26-50%=2, 51-75%=3, >75%=4, and focal=F) over the intensity (weak=1, moderate=2, strong=3). Higher CCND1 expression levels were observed in anaplastic cases (p<0.0001) with supratentorial localization (p=0.029). Cyclin D1 expression was considered as negative in 12.9% (LI=0), focal in 17%, weak in 23.1% (LI=1, 2 or 3), moderate in 19.7% (LI=4 or 6) and strong in 27.2% (LI=9 or 12). There was a correlation between mRNA and protein expression levels (p=0.017). Additionally, statistically significant correlations between cyclin D1 LI score and clinical variables as age (p=0.001), tumor location (p<0.0001), histological grade (p<0.0001) and relapse (p=0.046) were observed. Stratified analysis demonstrated that cyclin D1 expression was strong in supratentorial tumor location in grade II (8/12, p<0.0001) and grade III (23/26, p=0.002), in children (22/26 p<0.001) and adults (9/12, p<0.0001). However, cyclin D1 expression did not correlated with progression free survival (p=0.107). Multivariate analysis revealed that risk of recurrence was associated with intracranial tumor location (hazard ratio=4.7; p=0.03) and incomplete resection of tumors (hazard ratio = 6.35; p=0.012) in this cohort. Although there were more relapses in patients with gross total resection and strong cyclin D1 expression (p=0.013), no correlation with incomplete resection was observed (p=0.430). Determination of cyclin D1 expression LI could be used to guide treatment in supratentorial cases with gross total resection. Citation Format: Fernanda G. Andrade, Suely K. N. Marie, Hamilton Matushita, Sergio Rosemberg, Sueli M. Oba-Shinjo. Cyclin D1 expression correlates with supratentorial location of ependymomas. [abstract]. In: Proceedings of the 105th Annual Meeting of the American Association for Cancer Research; 2014 Apr 5-9; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2014;74(19 Suppl):Abstract nr 5594. doi:10.1158/1538-7445.AM2014-5594

Internalized elevation perception of simple stimuli in cochlear-implant and normal-hearing listeners.

In normal-hearing (NH) listeners, elevation perception is produced by the spectral cues imposed by the pinna, head, and torso. Elevation perception in cochlear-implant (CI) listeners appears to be non-existent; this may be a result of poorly encoded spectral cues. In this study, an analog of elevation perception was investigated by having 15 CI and 8 NH listeners report the intracranial location of spectrally simple signals (single-electrode or bandlimited acoustic stimuli, respectively) in both horizontal and vertical dimensions. Thirteen CI listeners and all of the NH listeners showed an association between place of stimulation (i.e., stimulus frequency) and perceived elevation, generally responding with higher elevations for more basal stimulation. This association persisted in the presence of a randomized temporal pitch, suggesting that listeners were not associating pitch with elevation. These data provide evidence that CI listeners might perceive changes in elevation if they were presented stimuli with sufficiently salient elevation cues.

E-021 De Novo, Progressive, Multiple, and Recurrent Intracranial Dural Arteriovenous Fistulas: Characteristics and Outcomes

PurposeAlthough many intracranial dural arteriovenous fistulas (DAVFs) are straightforward to treat, de novo and rapidly progressive (“runaway” or “malignant”) DAVFs are more complex, often requiring multiple treatment sessions with suboptimal...

P-029 Use of the Sceptre C Dual-Lumen Balloon Microcatheter in Onyx Embolization of Cerebral Arteriovenous Malformations: A Multi-Center Experience

IntroductionConventional onyx embolization of cerebral arteriovenous malformations (AVMs) requires lengthy procedure and fluoroscopy times to form an adequate ‘proximal plug’ for distal delivery of the material while minimising the likelihood...

Congenital teratoma in a rare cervical location

Objective – Teratomas with sacrococcygeal, mediastinal and gonadal locations are the most frequently occurring pediatric germ cell tumors. Cervicofacial and intracranial locations are rare. Approximately 20% of giant cervical teratoma causes airway compression. We report a congenital cervical teratoma with partial airway compression diagnosed post-natally in a preterm infant. Case report – A 27 year old mother delivered a male infant at 35 weeks gestation after her first uneventful pregnancy. The newborn had a notable anterior neck mass measuring 4x5 cm. The alpha fetoprotein level was elevated at 317.5 ng/ml and β-HCG was less than 1.2 mI U/ml. An MRI scan showed a 24x53x27 mm prelaryngeal and paratracheal solid-cystic mass compressing and shifting the larynx and trachea to the right. The tumor was approached via a wide collar incision. It did not communicate with the oesophagus, trachea or thyroid gland and did not infiltrate the surrounding tissues. It was completely excised. Pathological examination revealed an immature teratoma. The recovery was uneventful. Conclusion – Teratoma in infancy may present in an unusual cervical location. Not only giant tumors may compress the airway

Solitary clival plasmocytomas: Misleading clinical and radiological features of a rare pathology with a specific biological behaviour

Tumours of the clivus are exceptionally rare, representing a diagnostic and a therapeutic challenge. Clival solitary plasmocytomas have been described only as single case reports or included in small clinical series with other intracranial location. Authors report clinical, radiological, and survival data of four patients, who underwent surgery for clival plasmocytomas between 1989 and 2012 in a single centre. Current knowledge about solitary plasmocytomas of the clivus are reviewed. Follow-up time was 54 months (range: 9-165). Mean age of patient was 57 years, no gender predilection was observed. Main symptoms were headache (75 %) and double vision (75 %), due to third or sixth cranial nerve palsy. Mean time to diagnosis was 8.2 months. All patients underwent surgery as primary treatment, through either a transsphenoidal (75 %) or a transmaxillary approach (25 %). In all cases adjuvant conventional radiotherapy was performed with a median delivered dose of 45 Gy. Only one case of progression into multiple myeloma was observed 13 months after surgery, and the patient died 9 months later. No other recurrences or progression were observed. Mean overall survival and progression free survival time were, respectively, 54 and 51.7 months. Although extremely rare, clival plasmocytomas have to be considered in the differential diagnosis of a solitary clival lesion. Biological and clinical features of these tumours strongly differ from those of similar lesions in other part of the body. Early diagnosis, extensive tumour removal, opportune indication of adjuvant treatment with radiotherapy and chemotherapy are the keys to manage these cases.

Staged bilateral far-lateral approach for bilateral cervicomedullary junction neurenteric cysts in a 10-year-old girl

Neurenteric cysts are rare and benign lesions that consist of ectopic alimentary tissue residing in the central nervous system. They tend to occur most frequently in an intraspinal rather than intracranial location. Intracranial neurenteric cysts are a rare occurrence in the pediatric population. These lesions typically present as unilateral cystic structures in the lower cerebellopontine angle and craniocervical junction. To the authors' knowledge, there have been no reported cases of bilateral localization of intracranial neurenteric cysts. In this report, they present an unusual case of a 10-year-old girl who was found to have bilateral intracranial neurenteric cysts at the pontomedullary junction. The patient was successfully treated with staged, bilateral far-lateral transcondylar resection of the cysts. The authors also provide a brief overview of the literature describing intracranial neurenteric cysts in children.

Pattern of cerebrovascular atherosclerotic stenosis in older Chinese patients with stroke

The clinical pattern and angiographic distribution of cerebral atherosclerosis varies among different ethnic groups. This study was designed to identify the clinical background for intracranial and extracranial atherosclerotic stenosis in older eastern Chinese patients using digital subtraction angiography. We retrospectively reviewed the data collected from the Nanjing Stroke Registry Program, from January 2004 to March 2011. The analysis focused on the intracranial or extracranial location of stenosis in the anterior and posterior circulations. In total, records of 1041 patients were included in the study. Of these patients, 19.88% had intracranial carotid stenosis, 18.73% had stenosis in the extracranial vessels, and 33.33% had concurrent stenoses. A total of 2002 stenotic sites were detected in 749 patients. Among those patients with stenosis, a single stenosis was found in 170 (16.33%) and multiple stenoses were found in 579 (55.62%). The prevalence of intracranial stenosis in the single-stenosis group was 54.12%, while in the multiple-stenosis group it was 47.87% (p=0.127). A higher incidence of severe stenosis (70–99% blockage) and occlusion was found in the intracranial vessels than in the extracranial vessels (p=0.018). Older Chinese patients with atherosclerotic stenosis tend to have more intracranial stenoses.

Effect of mismatched place-of-stimulation on the salience of binaural cues in conditions that simulate bilateral cochlear-implant listening

Although bilateral cochlear implantation has the potential to improve sound localization and speech understanding in noise, obstacles exist in presenting maximally useful binaural information to bilateral cochlear-implant (CI) users. One obstacle is that electrode arrays may differ in cochlear position by several millimeters, thereby stimulating different neural populations. Effects of interaural frequency mismatch on binaural processing were studied in normal-hearing (NH) listeners using band-limited pulse trains, thereby avoiding confounding factors that may occur in CI users. In experiment 1, binaural image fusion was measured to capture perceptual number, location, and compactness. Subjects heard a single, compact image on 73% of the trials. In experiment 2, intracranial image location was measured for different interaural time differences (ITDs) and interaural level differences (ILDs). For larger mismatch, locations perceptually shifted towards the ear with the higher carrier frequency. In experiment 3, ITD and ILD just-noticeable differences (JNDs) were measured. JNDs increased with decreasing bandwidth and increasing mismatch, but were always measurable up to 3 mm of mismatch. If binaural-hearing mechanisms are similar between NH and CI subjects, these results may explain reduced sensitivity of ITDs and ILDs in CI users. Large mismatches may lead to distorted spatial maps and reduced binaural image fusion.

Durable response of intracranial cellular hemangioma to bevacizumab and temozolomide

Cellular hemangioma is a subtype of hemangioma that is associated with cellular immaturity and the potential for recurrence. Intracranial location of these lesions is extremely rare, and definitive treatment often requires radical neurosurgical resection. The authors report a case of a 12-year-old boy with a subtemporal cellular hemangioma. He underwent gross-total resection of the tumor, but within 1.5 months the tumor recurred, necessitating a second resection. Because of its proximity to vascular structures, only subtotal resection was possible. Repeat MRI 1 month after the second surgery showed significant tumor recurrence. Given the tumor's demonstrated capacity for recurrence and its proximity to the vein of Labbé and sigmoid sinus, further resection was not indicated. In an effort to limit radiation therapy for this young patient, treatment with bevacizumab and temozolomide was chosen and achieved a complete response that has proven durable for 36 months after cessation of therapy. This is the first report of the successful use of chemotherapy to treat an intracranial hemangioma, a rare condition with limited therapeutic options.

Focal Peak Activities in Spread of Interictal-Ictal Discharges in Epilepsy with Beamformer MEG: Evidence for an Epileptic Network?

Non-invasive studies to predict regions of seizure onset are important for planning intracranial grid locations for invasive cortical recordings prior to resective surgery for patients with medically intractable epilepsy. The neurosurgeon needs to know both the seizure onset zone (SOZ) and the region of immediate cortical spread to determine the epileptogenic zone to be resected. The immediate zone of spread may be immediately adjacent, on a nearby gyrus, in a different lobe, and sometimes even in the contralateral cerebral hemisphere. We reviewed consecutive simultaneous EEG/MEG recordings on 162 children with medically intractable epilepsy. We analyzed the MEG signals in the bandwidth 20–70 Hz with a beamformer algorithm, synthetic aperture magnetometry, at a 2.5 mm voxel spacing throughout the brain (virtual sensor locations, VSLs) with the kurtosis statistic (g2) to determine presence of excess kurtosis (γ2) consistent with intermittent increased high frequency spikiness of the background. The MEG time series was reconstructed (virtual sensor signals) at each of these VSLs. The VS signals were further examined with a relative peak amplitude spike detection algorithm. The time of VS spike detection was compared to the simultaneous EEG and MEG sensor signals for presence of conventional epileptiform spike morphology in the latter signals. The time of VS spike detection was compared across VSLs to determine earliest and last VSL to show a VS spike. Seven subjects showed delay in activation across VS locations detectable on visual examination. We compared the VS locations that showed earliest and later VS spikes with the locations on intracranial grid locations by electrocorticography (ECoG) that showed spikes and both onset and spread of seizures. We compared completeness of resection of VS locations to postoperative outcome. The VS locations for spike onset and spread were similar to locations for ictal onset and spread by ECoG.

Multiple Intracranial Meningiomas: A Review of the Literature and a Case Report

Multiple intracranial meningiomas are a condition where there is more than one meningioma in several intracranial locations in the same patient without signs of neurofibromatosis. Incidence varies from 1 to 10%. The prognosis of multiple intracranial meningioma does not differ from benign solitary meningiomas despite the multiplicity. However, the simultaneous occurrence of different grades of malignancy is observed in one-third of multiple meningiomas. Surgery remains the best option for treatment of symptomatic lesions. Our case review aims to present and discuss a 75-year-old female patient diagnosed with multiple intracranial meningiomas, describing their clinical, radiological, histological characteristics. It also highlights the fact that the patient had two tumours, underwent surgery, and so far has a good quality of life.

Invasive thymoma metastatic to the cavernous sinus

Background:Thymomas are typically benign tumors of thymic epithelium. Metastases to distal sites, particularly intracranial locations, are extremely rare. Herein, we present the third case of thymoma and the second invasive thymoma to metastasize to the cavernous sinus, adjacent to the pituitary.Case Description:A 41-year-old female patient presented with headaches, stuffy nose, and drooping of the right face. A magnetic resonance imaging scan revealed a complex, multilobulated mass centered upon the right cavernous sinus. The mass was removed via transsphenoidal surgery, and histopathological investigation confirmed the diagnosis of metastatic thymoma. A positron emission tomography-computed tomography scan demonstrated a large anterior mediastinal mass. A biopsy confirmed the diagnosis of invasive thymoma morphologically identical to the World Health Organization type B2 sellar region metastasis.Conclusion:Although rare, thymomas can metastasize to the central nervous system. Our case is the second invasive thymoma to metastasize to the cavernous sinus, adjacent to the pituitary.

Bilateral intraventricular mass in a child

An 8-year-old boy presented with a history of holocranial headache for 4 weeks which was mild to moderate in intensity. There was history of intermittent vomiting for the same duration. The patient was drowsy for the last 10 days. Non-contrast CT scan of head showed a space-occupying lesion in lateral ventricles with hydrocephalus. MRI showed a large lobulated well-defined heterogeneous mass lesion filling up both lateral ventricles at the posterior and temporal horns, bilaterally reaching up to the level of the foramen magnum. The mass lesion was hypointense on T2-weighted images (T2W) and isointense to grey mater on T1W images. On contrast images there was intense homogeneous enhancement seen at the lesion (Fig. 1). Right fronto-temporo-parietal craniotomy with decompression of the tumor was performed. Perioperatively, the tumor was yellowish in color, firm, not able to be aspirated and minimally vascular. Clinical diagnosis of choroid plexus tumor was considered.

E-018 Initial experience with distal guide catheter placement in the treatment of cerebrovascular disease: clinical safety and efficacy

BackgroundThe treatment of cerebrovascular diseases continues to grow with improvements in technology. The use of stable guide catheters in the large cervical cerebral vessels has historically been paramount to the...

Large epidermoids of the quadrigeminal cistern: an experience of 15 consecutive cases and review of literature

Large epidermoids of the quadrigeminal cistern (LEQCs) of more than 4 cm in size are benign intracranial tumours and present with diverse clinical manifestations. Fifteen cases of LEQC have been treated in our institute over last 10years. This is a retrospective study of the 15 LEQCs treated in our institute from January 2001 to May 2011. We classified these epidermoids into three types, based on the location of the major bulk of the epidermoid, to tailor surgical approaches to these tumours. Fifteen patients (age range 14-45years, mean age 27.2years) were operated upon after clinicoradiological evaluation. Eight were females and seven were males. The mean duration of symptoms was 9.2months. Symptoms of raised intracranial pressure were present in ten patients. Surgical approaches included infratentorial-supracerebellar approach in nine cases, the occipital transtentorial approach in five cases and subtemporal approach one case. Complete excision was possible in ten (66.6%) cases, near total in four (26.6%) and subtotal excision in one case. Six patients underwent cerebrospinal fluid (CSF) diversion by ventriculoperitoneal (VP) shunt. Twelve patients improved with surgery and are functionally active. One patient developed hemiparesis. There were two deaths in our series. LEQC may present with hydrocephalus, unlike epidermoids at other intracranial locations. Complete surgical excision is possible in most of the patients and it helps in re-establishing the CSF pathway. Early surgery in these patients prevents postoperative shunt dependence. Most of the patients improve after surgery.

Biomarkers and location of atherosclerosis: Matrix metalloproteinase-2 may be related to intracranial atherosclerosis

BackgroundVarious biomarkers are linked with the pathophysiology of atherosclerosis. We hypothesized that these factors may be associated with the location and burden of cerebral atherosclerosis. MethodsWe evaluated 177 consecutive patients with chronic (>6 months) ischemic stroke: 68 with small vessel occlusion (SVO) and 109 with large-artery atherosclerosis (LAA), with the latter further sub-classified into 80 patients with intracranial atherosclerosis (ICAS) and 29 with extracranial atherosclerosis (ECAS). The number of ≥50% steno-occlusions on magnetic resonance angiography was used to assess the burden of atherosclerosis. Serum concentrations of the biomarkers (matrix metalloproteinases (MMP)-2 and -9, homocysteine, interleukin (IL)-6, tumor necrosis factor-α, C-reactive protein, adiponectin, leptin, resistin, free fatty acid, and lipoprotein(a)) and the metabolic syndrome were measured in each study subject. ResultsDecreased plasma concentrations of MMP-2 (p = 0.020) and homocysteine (p = 0.038) were more closely associated with ICAS than with ECAS, whereas increased IL-6 concentrations were related to severe (≥4 steno-occlusions) atherosclerosis (p = 0.031). Multiple logistic regression analysis showed that the lowest tertile of MMP-2 was independently associated with ICAS (OR 4.84, 95% CI 1.29–18.19, p = 0.022). ConclusionLow MMP-2 plasma levels are associated with intracranial location of cerebral atherosclerosis, suggesting that MMP-2 may play a role in the development of ICAS.

The three-channel model of sound localization mechanisms: Interaural level differences

The current understanding of mammalian sound localization is that azimuthal (horizontal) position assignments are dependent upon the relative activation of two populations of broadly-tuned hemifield neurons with overlapping medial borders. Recent psychophysical work has provided evidence for a third channel of low-frequency interaural time difference (ITD)-sensitive neurons tuned to the azimuthal midline. However, the neurophysiological data on free-field azimuth receptive fields, especially of cortical neurons, has primarily studied high-frequency cells whose receptive fields are more likely to have been shaped by interaural level differences (ILDs) than ITDs. In four experiments, a selective adaptation paradigm was used to probe for the existence of a midline channel in the domain of ILDs. If no midline channel exists, symmetrical adaptation of the lateral channels should not result in a shift in the perceived intracranial location of subsequent test tones away from the adaptors because the relative activation of the two channels will remain unchanged. Instead, results indicate a shift in perceived test tone location away from the adaptors, which supports the existence of a midline channel in the domain of ILDs. Interestingly, this shift occurs not only at high frequencies, traditionally associated with ILDs in natural settings, but at low frequencies as well.

Intradural chordoma mimicking a lateral sphenoid wing meningioma: a

Chordomas are rare tumours arising from notochordal remnants. Classical chordomas are generally extradural and, despite benign histopathology, they typically destroy the clivus and surrounding bone structures. Intradural lesions are extremely rare and less than thirty cases of intracranial, exclusively intradural chordomas have been reported so far. The intracranial, intradural but extranotochordal location of chordoma is extremely unique. The authors present a case of chordoma in intracranial location that clinically mimics lateral sphenoid wing meningioma. A previously healthy 39-year-old man was admitted to our Department because of optic disc oedema without neurological deficits. Neuroimaging studies showed a large, contrast-enhanced tumour in the right frontotemporal region that was thought to be a pterional meningioma. The patient underwent successful removal of the tumour. Histopathological study revealed a typical pattern of chordoma, confirmed by immunohistochemical findings. Because of the tumour location the differentiation between chordoma and chordoid meningioma ought to be considered. Such cases, including the present one, may lead to the conclusion that embryonic notochordal remnants may be lost in different places, even away from the neuroaxis.

Intracranial germ cell tumors at unusual locations

Germ cell tumor (GCT) is relatively uncommon in intracranial locations. They constitute ~ 0.3-0.6% of intracranial neoplasms and encompass a wide pathologic range. The majority occurs in young adults and occupies the midline locations like pineal gland followed by suprasellar compartment. These tumors are rare in the cerebral hemisphere, basal ganglia, thalamus and ventricles. Neuroimaging studies cannot differentiate GCTs from other tumors, and therefore, the diagnosis usually requires histological confirmation. Germ cell tumors can be divided into major groups including germinomas and nongerminomatous GCTs (NGGCTs). Their proper identification as well as histopathological typing is important as treatment and prognosis vary greatly between different groups. Germinomas have a superior prognosis and are more radiosensitive as compared to non-germinomatous germ cell tumors. Standard management is still controversial. In this case series we are presenting three cases of intracranial germ cell tumors arising in two unusual locations, that is intraventricular and thalamic region. Apart from the clinical, radiological, histopathological and surgical details we also discuss the various aspects of intracranial germ cell tumors.