A Rare Case of Supratentorial Cavernous Angioma Associated with Arterialized Developmental Venous Anomaly.

The association of cavernous malformations and developmental venous anomalies (DVA) is well known, but the presence of arterial fistulous connection with the main venous collector has been reported in the literature only once. We report the unusual case of a hemorrhagic cavernous angioma associated with DVA characterized by a fine arterial supply to the main venous collector. During surgery, after the excision of the cavernous angioma, few small arterial feeders were found entering the main channel of the venous developmental anomaly. The presence of an arterial fistulous connection with the main venous collector of a DVA may be a possible mechanism involved in a higher bleeding potential of cavernous angioma.

Primary Cardiac High-grade Myxofibrosarcoma Presenting with Multiple Brain Metastases: A Case Report.

Herein we describe the case of a young patient who presented with a recent history of epilepsy due to multiple brain lesions; he did not complain about any cardiopulmonary impairments. The patient died as a consequence of hemorrhagic progression of brain metastatic disease. Regardless of a thorough investigation, the heart tumor remained occult. Primary cardiac tumors are very rare entities. Most of these are benign, but approximately 25% are malignant, and the majority of these are sarcomas. Myxofibrosarcoma and osteosarcoma are exceptionally rare. To date, we find only small series of cardiac myxofibrosarcoma, and to our knowledge, this one exceptionally presented with multiple brain metastatic lesions without cardiopulmonary symptoms.

The effect of re-operation on survival in patients with recurrent glioblastoma.

BACKGROUND: Treatment options for glioblastoma (GBM) at recurrence have limited efficacy. Re-surgery has been used for confirmation of recurrent disease and to provide relief of symptoms but the real impact on survival is unknown. PATIENTS AND METHODS: A retrospective analysis was performed for GBM patients followed between 01/2005 and 06/2010 at our Institution. RESULTS: Two hundred and thirty-two patients with recurrent GBM were evaluated. One hundred and two patients (44%) were treated with re-surgery followed by chemotherapy and 130 patients (56%) with chemotherapy alone. In multivariate analysis, no significant effect of re-surgery was found, with age (p=0.001), MGMT methylation (p=0.002) and PFS at 6 months (p=0.0001) being significant prognostic factors. CONCLUSION: Second surgery might have a limited impact in the clinical course of recurrent GBM patients.

Post progression survival in glioblastoma: where are we?

The optimal end point for phase II studies for recurrent glioblastoma (GBM) is unclear and a matter of debate. Moreover, data about post-progression survival (PPS) after the first disease progression in GBM patients treated according to EORTC 26981/22981/NCIC CE.3 trial are limited. The aim of this study was to evaluate the PPS in GBM patients. The analysis was made with a database on 1,006 GBM patients followed prospectively between 06/2005 and 06/2010. Eligibility criteria for the study were: age ≥ 18 years; PS: 0-2; chemotherapy given at disease progression after RT/TMZ. 232 patients (mean age 52 years, range 18-77 years) were enrolled. The median PFS following second line chemotherapy (PFS2) was 2.5 months (95 % CI 2.1-2.9) and the rate of patients free of progression at 6 months (PFS2-6 mo), was 21.6 % (95 % CI 16.3-26.9 %). The median PPS was 8.6 months (95 % CI 7.4-9.8), PPS rates were: PPS-6: 66 % (95 % CI 60.3-72.9 %), PPS-9: 48.2 % (95 % CI 41.5-54.9 %) and PPS-12: 31.7 % (95 % CI 25.2-38.2 %). PPS in unselected patients treated with alkylating agents is about 8 months. PPS rates could be of interest as an end point in future studies in recurrent GBM.

Erratum. The Neuroradiology Journal 27: 452-455, 2014 - doi: 10.15274/NRJ-2014-10060 - www.centauro.it. Olfactory Neuroblastoma and Olfactory Ventricle.

Unfortunately, the affiliation of the author name, Antonella Bacci, was incorrectly listed as Antonella Bacci(1) instead of Antonella Bacci(3), and the affiliations were incorrectly listed as (1) Department of Neurosurgery, IRCCS Neurological Research Hospital; Bologna, Italy - (2) Section of Pathology "M. Malpighi", Bellaria Hospital, Azienda USL of Bologna, Bologna, Italy instead of (1) Department of Neurosurgery, (3) Department of Neuroradiology, IRCCS Neurological Research Hospital; Bologna, Italy. (2) Section of Pathology "M. Malpighi", Bellaria Hospital, Azienda USL of Bologna, Bologna, Italy in the original publication of this paper.

Olfactory neuroblastoma and olfactory ventricle. A case report.

A case of dumbbell nasal and intracerebral olfactory neuroblastoma (ONB) related to possible seeding through an "olfactory ventricle" in the olfactory bulb and nerve is reported. This anatomic variant was recognized at operation for the first time: it consists of a hollow cavity within the olfactory bulb and nerve which may be connected to the subventricular zone of the frontal horn of the lateral ventricle. Better scrutiny of this structure is necessary in view of its possible interference with the course and diffusion of ONBs.

Endoscopic treatment of a fourth ventricle arachnoid cyst via the third ventricle: a case report.

BACKGROUND AND IMPORTANCE: Arachnoid cysts within the fourth ventricle have rarely been reported in the literature. Different procedures have been performed to restore a normal cerebrospinal fluid dynamic or pressure, including shunting and partial or complete excision of the cyst by open microsurgery. Cerebrospinal fluid shunts give only partial improvement of symptoms and are prone to malfunctions. The microsurgical excision of the cyst seems to offer the best chance of success. CLINICAL PRESENTATION: We report the case of a fourth ventricle arachnoid cyst successfully treated with a complete endoscopic cerebral procedure via the third ventricle. CONCLUSION: Endoscopic fenestration of fourth ventricle arachnoid cysts may be considered an effective neurosurgical treatment.

Symptomatic cerebral cavernomas in pregnancy: a series of 6 cases and review of the literature.

OBJECTIVE: To present our experience of symptomatic cerebral cavernous malformations (CCMs) in pregnancy and to review the literature on the topic. METHODS: We retrospectively collected a case series of symptomatic CCMs during pregnancy or the puerperium. A literature search was performed to identify all similar reports. RESULTS: We collected 16 cases of symptomatic CCMs. Haemorrhage occurred in 10 patients. Two patients opted for termination of pregnancy. Delivery occurred preterm in four cases, in only one case due to neurological symptoms at 30 weeks' gestation. Caesarean section was performed in 9 cases; concern over CCM was the indication for delivery in eight of these cases. Four out of 16 patients underwent neurosurgery, three during pregnancy. CONCLUSION: Symptomatic CCMs seldom require neurosurgery either during or after pregnancy and are not associated with preterm delivery.

Neurogenesis in temporal lobe epilepsy: relationship between histological findings and changes in dentate gyrus proliferative properties.

OBJECTIVE: The relationship between hippocampal histopathological abnormalities, epileptogenesis and neurogenesis remains rather unclear. METHODS: Tissue samples including the subgranular zone of dentate gyrus (DG) were freshly collected for tissue culture for neurospheres generation in 16 patients who underwent surgery for drug-resistant temporal lobe epilepsy. Remaining tissues were histologically examined to assess the presence of mesial temporal sclerosis (MTS) and focal cortical dysplasia. RESULTS: MTS was detected in 8 cases. Neurospheres were formed in 10/16 cases. Only three out of these 10 cases exhibited MTS; on the contrary 5/6 cases lacking neurosphere proliferation presented MTS. There was a significant correlation between presence of MTS and absence of proliferation (p = 0.0389). We also observed a correlation between history of febrile seizures (FS) and presence of MTS (p = 0.0004) and among the 6 cases lacking neurosphere proliferation, 4 cases (66.6%) had experienced prolonged FS. Among "proliferating" cases the percentage of granular cells pathology (GCP) was lower (20% vs 50%) compared to "non proliferating" cases. CONCLUSION: A decreased potential to generate neurosphere from the SGZ is related to MTS and to alterations of dentate gyrus granule cells, especially in MTS type 1b and GCP type 1. These histological findings may have different prognostic implications, regarding seizure and neuropsychological outcome, compared to patients with other epileptogenic lesions (such as FCD, glioneuronal tumours, vascular lesions).

Successful removal and reimplant of vagal nerve stimulator device after 10 years.

The number of implanted vagal nerve stimulators is growing and the need for removal or revision of the devices will become even more frequent. A significant concern about Vagus Nerve Stimulation (VNS) therapy is the presence of the spiral stimulating electrodes, wrapped around the nerve, once treatment is considered ineffective or is no longer desired. Our purpose is to demonstrate the feasibility of complete removal and replacement of the vagal nerve stimulator electrodes using microsurgical technique even after a long period, without damaging the nerve. We attempted removal and replacement of spiral stimulating electrodes from a patient who received a 10-year long VNS therapy for drug-resistant epilepsy. Our results indicate that the spiral electrodes may be safely removed from the vagus nerve, even after several years. The reversibility of lead implantation may enhance the attractiveness of VNS therapy. Furthermore, with a correct microsurgical technique, it is possible to respect the normal anatomy and functionality of vagal nerve and to reimplant a new VNS system with all its components, maintaining the same therapeutic efficacy after many years.

Temporo-mesial extraventricular neurocytoma and cortical dysplasia in focal temporal lobe epilepsy.

We describe a 17-year-old boy with a left extraventricular temporo-mesial neurocytoma associated with cortical dysplasia causing focal pharmacoresistant temporal lobe epilepsy. He presented with a long history of medically refractory, temporal complex partial seizures. MRI showed a left temporo-mesial lesion suspect to be a low-grade tumor. Based on the pre-operative non-invasive neurophysiological studies, the patient underwent a left tailored temporal antero-mesial resection. Histopathological examination showed an extraventricular neurocytoma associated with architectural dysplasia (Type 1a) of the temporal pole. The patient was seizure-free at 2 years follow-up. Extraventricular neurocytomas must be considered in the differential diagnosis of the plethora of low-grade tumors associated with focal epilepsy that typically involve the temporal lobe, and are frequently associated with focal cortical dysplasia.

The putative role of the venous system in the genesis of vascular malformations.

Recent clinical and experimental evidence has challenged the traditional concept of the venous system as a "passive" element in the genesis and evolution of intracranial vascular malformations. The authors review the clinical and experimental evidence linking the venous system and its anomalies to the genesis of various intracranial vascular malformations, including dural arteriovenous fistulas, cavernous malformations, parenchymal arteriovenous malformations, and capillary telangiectasia. They also describe the potential significance of different associations of these vascular anomalies.

Cavernous malformations of the central nervous system in the pediatric age group.

OBJECTIVE: The main clinico-diagnostic features, risk factors and associated diseases of cavernous malformations (CMs), also called cavernous angiomas or cavernomas, of the central nervous system (CNS) in children are described, and the most relevant differences compared to the affected adult population are pointed out, focusing on the management of pediatric patients harboring cranial and spinal CMs. MATERIALS: This was a retrospective study of a series of 42 children symptomatic for CMs of the cranial and spinal compartments (35 supratentorial brain lesions, 5 infratentorial and 2 in the spinal region) operated on between 1975 and 2005, with a clinical follow-up ranging from 12 to 192 months. The results were compared with those found in the most recent literature dealing with pediatric CMs of the CNS. RESULTS: Surgical treatment produced excellent or good results in 69% of our 42 children. Unchanged neurological deficits were observed in 23.8% of cases, while morbidity from surgical procedures was 7.14%. Mortality was absent in this series. These surgical results are comparable with those found in the literature, where morbidity and mortality rates from surgery are 8.8 and 1.13%, respectively, and are mostly associated with procedures for the excision of deep, critically located cavernomas. CONCLUSION: CMs represent the most common CNS vascular lesion in children, although their incidence is 4 times lower than that of the adult population. The natural history of pediatric CMs throughout the neuraxis seems to be more aggressive than in adult patients; these lesions have higher rates of growth and hemorrhage, larger dimensions and often atypical radiological pictures at diagnosis. Beside the familial form of the disease, which is more often associated with multiple lesions and an earlier age of clinical presentation, the major risk factor for CMs in children seems to be radiotherapy for CNS tumors. Furthermore, a greater number of CMs coexistent with mixed angiomatous lesions have been reported in children than in adults. Surgical results are related to the preoperative neurological status of the children; symptomatic patients who are operated on early, before they develop severe neurological deficits or long-standing seizures, may achieve the best clinical outcome. Radiosurgery does not seem to be advisable in children as an alternative treatment for deep CMs or those causing epilepsy.

Association between response to primary treatments and MGMT status in glioblastoma.

The median overall survival and, more importantly, the 2-year survival rate of patients with newly diagnosed glioblastoma are increased by the administration of combined temozolomide and radiotherapy, which has recently become the new standard of treatment in patients with a histological confirmation of diagnosis. Moreover, the assessment of O(6)-methylguanine-DNA methyltransferase gene promoter methylation has clarified the impact of this approach, and improved upon the interpretation of doubtful cases after concurrent radiotherapy/temozolomide treatment. Therefore, future strategies in the treatment of glioblastoma patients will include stratification for MGMT methylation status, and various approaches based on epigenetic features are currently under investigation.

The neurovascular triad: mixed cavernous, capillary, and venous malformations of the brainstem.

OBJECT: The four types of cerebrovascular malformations may sometimes be combined and more often occur in pairs; triads are exceptional. The authors present six patients with the clinicoradiographic profile of mixed vascular malformations of the brainstem, including cavernous malformation (CM), capillary telangiectasia, and developmental venous anomaly (DVA). METHODS: Five patients (one of whom was a child) suffered from hemorrhage, suggesting that this complex association has a high bleeding potential. Progressive growth, rebleeding, and de novo occurrence of the associated CM were documented in three cases. Magnetic resonance imaging of the brain was obtained in all patients by using one or more of the following modalities: T1-weighted sequences before and after gadolinium administration; T2-weighted sequences; T2-weighted fluid attenuated Inversion recovery; T1-weighted fast spin echo; and diffusion weighted, diffusion tensor, and perfusion imaging in three cases. RESULTS: Three patients were surgically treated with the intention of excising the hemorrhagic lesion, but only two patients had their malformations successfully removed. In the third case, diffuse pontine telangiectasia precluded the safe excision of the CM. Histological examination demonstrated a blended pathological milieu characterized by coalescent telangiectasia and venules associated with loculated endothelial chambers resembling an immature or de novo CM. Three patients were treated conservatively; recurrent minor hemorrhage occurred in one case. The authors found these malformations to be arranged in two basic relationships: CM inside the telangiectasia and CM in the radicles of the DVA. Stenosis of the main venous collector and dilation of the medullary veins were important findings. CONCLUSIONS: The pathogenesis of this malformation may be referred to a developmental deviance of the brainstem capillary-venous network associated with transitional vessels and loculated endothelial vascular spaces related to genetic and acquired origins, probably in a restrictive venous outflow milieu.

Rapid growth and regression of intracranial meningiomas in lymphangioleiomyomatosis: case report.

BACKGROUND: Lymphangioleiomyomatosis is a progressive interstitial lung disease that affects young women. It has been suggested that estrogens play a role in its evolution, and progesterone therapy is often provided in these cases. CASE DESCRIPTION: We present a case of a postmenopausal woman with LAM treated with progesterone; subsequently, rapid growth of multiple intracranial meningiomas was observed. One prominent lesion was excised, and 3 other lesions regressed spontaneously over 2 years. CONCLUSIONS: This is a rare case of a non-pregnancy-related regression of meningiomas in a woman affected by LAM. The significance of this association and the hormonal treatment of the disease are discussed.

Acute presentation of spinal epidural cavernous angiomas: case report.

OBJECTIVE: Spinal extramedullary hematomas stemming from cavernous angiomas in the epidural compartment are rare. It is more common for spinal epidural cavernous angiomas to present with slow and progressive myelopathy or radiculopathy. They seldom present with clinical evidence of acute spinal cord or nerve root compression. CLINICAL PRESENTATION: Three consecutive cases of acute spinal cavernous angiomas with overt neurological deficits were presented. These presentations included acute onset of neck pain and tetraparesis, bilateral lower extremity pain and paraparesis, and acute sciatic pain with plantar flexor weakness. The lesions were located in the cervical, thoracic, and sacral spine, respectively. The cases included either abrupt lesion enlargement secondary to a pure intralesional hemorrhage or a cavernous hemorrhage that invaded the epidural space. INTERVENTION: All patients were treated with either laminotomy or complete resection of the hematoma and cavernoma within 12 hours after admission. CONCLUSION: Spontaneous spinal epidural hematomas presented with significant pain and acute spinal cord and nerve root compression may represent the manifestation of a cavernous angioma. Appropriate interpretation of preoperative imaging studies may prevent delay in proper management, especially for patients in whom nerve root deficit is the only clinical expression.

Bleeding of a familial cerebral cavernous malformation after prophylactic anticoagulation therapy. Case report.

The risk of hemorrhage in cavernous malformations (CMs) depends on lesion, host, and environmental factors. Anticoagulation therapy is a well-known risk factor for intracerebral bleeding, but the occurrence of hemorrhages in patients with CMs has not been reported. Low molecular weight heparin therapy is generally considered to be safe, although significant hemorrhagic complications have recently been reported. The authors report a case of intralesional bleeding in a CM after prophylactic anticoagulation therapy was administered in a patient with the familial form of the disease. The leaky endothelial structure of CMs may constitute an unexpected target of the vascular effects of heparin.

Traumatic intracerebellar hemorrhage: clinicoradiological analysis of 81 patients.

OBJECTIVE: We report 81 patients with a traumatic intracerebellar hemorrhagic contusion or hematoma managed between 1996 and 1998 at 13 Italian neurosurgical centers. METHODS: Each center provided data about patients' clinicoradiological findings, management, and outcomes, which were retrospectively reviewed. RESULTS: A poor result occurred in 36 patients (44.4%). Forty-five patients (55.6%) had favorable results. For the purpose of data analysis, patients were divided into two groups according to their admission Glasgow Coma Scale (GCS) scores. In Group 1 (39/81 cases; GCS score, > or =8), the outcome was favorable in 95% of cases. In Group 2 (42/81 cases; GCS score, <8), the outcome was poor in 81% of cases. Twenty-seven patients underwent posterior fossa surgery. Factors correlating with outcome were GCS score, status of the basal cisterns and the fourth ventricle, associated supratentorial traumatic lesions, mechanism of injury, and intracerebellar clot size. Multivariate analysis showed significant independent prognostic effect only for GCS score (P = 0.000) and the concomitant presence of supratentorial lesions (P = 0.0035). CONCLUSION: This study describes clinicoradiological findings and prognostic factors regarding traumatic cerebellar injury. A general consensus emerged from this analysis that a conservative approach can be considered a viable, safe treatment option for noncomatose patients with intracerebellar clots measuring less than or equal to 3 cm, except when associated with other extradural or subdural posterior fossa focal lesions. Also, a general consensus was reached that surgery should be recommended for all patients with clots larger than 3 cm. The pathogenesis, biomechanics, and optimal management criteria of these rare lesions are still unclear, and larger observational studies are necessary.