Pattern of care and effectiveness of treatment for glioblastoma patients in the real world: Results from a prospective population-based registry. Could survival differ in a high-volume center?

BACKGROUND: As yet, no population-based prospective studies have been conducted to investigate the incidence and clinical outcome of glioblastoma (GBM) or the diffusion and impact of the current standard therapeutic approach in newly diagnosed patients younger than aged 70 years. METHODS: Data on all new cases of primary brain tumors observed from January 1, 2009, to December 31, 2010, in adults residing within the Emilia-Romagna region were recorded in a prospective registry in the Project of Emilia Romagna on Neuro-Oncology (PERNO). Based on the data from this registry, a prospective evaluation was made of the treatment efficacy and outcome in GBM patients. RESULTS: Two hundred sixty-seven GBM patients (median age, 64 y; range, 29-84 y) were enrolled. The median overall survival (OS) was 10.7 months (95% CI, 9.2-12.4). The 139 patients ≤aged 70 years who were given standard temozolomide treatment concomitant with and adjuvant to radiotherapy had a median OS of 16.4 months (95% CI, 14.0-18.5). With multivariate analysis, OS correlated significantly with KPS (HR = 0.458; 95% CI, 0.248-0.847; P = .0127), MGMT methylation status (HR = 0.612; 95% CI, 0.388-0.966; P = .0350), and treatment received in a high versus low-volume center (HR = 0.56; 95% CI, 0.328-0.986; P = .0446). CONCLUSIONS: The median OS following standard temozolomide treatment concurrent with and adjuvant to radiotherapy given to (72.8% of) patients aged ≤70 years is consistent with findings reported from randomized phase III trials. The volume and expertise of the treatment center should be further investigated as a prognostic factor.

Revascularized giant aneurysm of the anterior communicating artery after surgery and embolization, occluded by placement of a Leo+Baby intracranial stent. A case report.

Balt (Montmorency, France) recently manufactured the Leo+Baby dedicated intracranial stent for arteries with a calibre between 1.5 and 3.10 mm. We describe a patient with a partially thrombosed giant sacciform aneurysm of the anterior communicating artery treated without success by surgery and coil embolization subsequently occluded by placement of a Leo+Baby stent (Balt, Montmorency, France). A 56-year-old man presented with a giant aneurysm in the anterior communicating artery region. Following successive surgical intervention and embolization procedures the patient was referred to us with a revascularized aneurysm measuring 15×9×8 cm. To stabilize the endovascular occlusion a combined treatment was scheduled with coil embolization and stent deployment after dual antiplatelet therapy started five days before the interventional procedure. Treatment was undertaken two weeks later under general anaesthesia and total heparinization. A microcatheter was inserted into the aneurysmal sac and four metal coils were released for a total of 61cm obtaining almost complete occlusion of the aneurysm from the circulation. A Leo+Baby stent (2.5×18 mm) was subsequently deployed across the aneurysm neck. At follow-up angiography two months later the aneurysm appeared substantially excluded from the arterial circulation except for a small portion in the neck. Six months later, four months after suspending antiplatelet therapy, follow-up angiography disclosed the complete exclusion of the aneurysm from the circulation. Deployment of the new ministent through the same microcatheter used to release the coils made the interventional procedure simpler and faster.

Focal epilepsies associated with glioneuronal tumors: review article.

Glioneuronal tumors (GNTs) are an increasingly recognized cause of focal epilepsies, particularly in children and young adults. GNTs consist of a mixture of glial and neuronal elements and most commonly arise in the temporal lobe, particularly in the temporo-anterior-basal mesial site. They are often associated with cortical dysplasia or other neuronal migration abnormalities. Epilepsy associated with GNT is poorly controlled by antiepileptic drugs in many cases; but, it is extremely responsive to surgical treatment. However, the best management strategy of tumor-related focal epilepsies remains controversial and still remain one of the contemporary issues in epilepsy surgery. Temporo-mesial GNT are associated with a widespread epileptic network, defining, therefore, a distinct anatomo-clinico-pathological group with complex epileptogenic mechanisms. By using an epilepsy surgery oriented strategy GNT associated with focal epilepsies may have an excellent seizure outcome and, therefore, surgical treatment can be offered early to avoid both the consequences of uncontrolled seizures as well as the side effects of prolonged pharmacological therapy and the rare risk of tumor growth or malignant transformation.

Endoscope-controlled microneurosurgery for the treatment of intracranial fluid cysts.

OBJECTIVE: This study assessed the role of endoscope-controlled microneurosurgery (ECM) in the treatment of intracranial cerebrospinal fluid (CSF) cysts. METHODS: Twelve patients affected by symptomatic arachnoid cysts (11 in the sylvian fissure and 1 in the cerebellopontine angle) underwent ECM, in which the endoscope is used to view the operating field while microsurgical instruments can be passed alongside the endoscope for surgical maneuvers. All the cysts were superficially located and could be reached directly through a burr hole on their surface, so that the endoscope was inserted into the cyst without passing through the brain parenchyma. We used a rigid endoscope, performing a wide fenestration of the cyst in the basal cisterns (cystocisternostomy). The excellent endoscopic vision facilitated the surgical procedure with safe maneuvers through a minimally invasive approach. In 11 patients, we observed the resolution of symptoms. A radiological reduction in the cyst was observed in 7 cases. No mortality or major complications were observed. CONCLUSIONS: Endoscope-controlled microsurgery is a valid minimally invasive procedure for treating superficially located intracranial arachnoid cysts.

Bobble-head doll syndrome due to a suprasellar arachnoid cyst: endoscopic treatment in two cases.

OBJECT: We report two cases of bobble-head doll syndrome associated with a large suprasellar arachnoid cyst successfully treated with a minimally invasive endoscopic approach. METHODS: The clinical history, surgical treatment and results of two children, a 9-year-old boy and a 1-year-old girl, both presenting the clinical features of the bobble-head doll syndrome, are described. As a first procedure, a ventriculo-cystostomy was endoscopically performed in both patients, obtaining either resolution of the symptoms or notable cyst reduction. In the girl, a re-closure of the stoma, with cyst re-expansion, was observed after 18 months. She then underwent a second procedure, a ventriculo-cysto-cisternostomy, with a good result. After 3 years' follow-up, the neurological condition of both patients remains good with complete resolution of abnormal head movement. CONCLUSION: In our opinion, endoscopic treatment is the procedure of choice for this condition, as it involves few complications and gives good results.

[A case of cerebral coenurosis]. / Su di un caso di cenurosi cerebrale.

In this article the authors describe a case of cerebral coenurosis, a rare infection caused by the larval stage of the tapeworm Taenia multiceps, encountered during their professional practice. The specific epidemiological elements, linked to the parasitic lifecycle in dogs, will also be covered. The authors consider the diagnostic, pathologic and clinical elements that allow differential diagnosis with respect to neurocysticercosis, a parasitic infection caused by the larval form of other Cestoda, discussing possible medical therapeutic approaches (albendazole or praziquantel) and neurosurgical intervention. They emphasize the need to obtain a correct hystopathological diagnosis in order to achieve a differential diagnosis versus the other larval parasitosis. According to current public health regulations this diagnosis must be reported to the Health Official to allow the necessary epidemiological interventions to be planned.

Early ictal speech and motor inhibition in fronto-mesial epileptic seizures: a polygraphic study in one patient.

OBJECTIVE: To investigate ictal motor inhibition occurring during seizures in a patient with a tumor located in the left fronto-mesial pre-central cortex. METHODS: Awake and sleep video-polygraphic monitoring, recording scalp EEG and EMG activities from several cranial, trunk and limbs muscles, was performed in a patient with drug-resistant recurrent focal motor seizures before surgical treatment. Speech/motor tasks were repeatedly administered to the patient during the recording sessions in order to evaluate the occurrence of early ictal motor inhibition. RESULTS: Thirty-four seizures were recorded during wakefulness showing a stereotyped pattern of inhibition of speech and voluntary movements followed by sequential activation of upper limb-trunk-lower limb muscles contralateral to the tumor. Polygraphic recordings showed that: (1) initial speech and motor arrest were associated with the EMG evidence of progressive muscle tone suppression in cranial and right distal upper limb muscles; (2) tonic contraction of right deltoid, biceps brachii, intercostalis and paraspinalis muscles appeared after motor inhibition; (3) tonic-clonic activity in the right tibialis anterior muscle occurred at the end of seizures. Eleven subclinical seizures were recorded during sleep showing mild focal tonic EMG activity in right side trunk muscles. CONCLUSIONS: Our findings evidenced early and somatotopically organized inhibition of voluntary movement at the beginning of epileptic seizures with fronto-mesial onset. The demonstration that speech and motor arrest were associated with progressive EMG suppression in cranial and limb muscles supports the hypothesis of motor inhibitory seizures originating in the mesial aspect of pre-motor frontal cortex.

Hypertension, hyperekplexia, and pyramidal paresis due to vascular compression of the medulla.

MRI showed impingement of the vertebral artery on the left lateral medulla in two patients with arterial hypertension, exaggerated startle reflexes (hyperekplexia), and progressive spastic paresis. One patient underwent microvascular decompression with normalization of arterial hypertension, disappearance of hyperekplexia, and improvement of spastic paresis. The combination of arterial hypertension, hyperekplexia, and progressive spastic paresis should arouse suspicion of neurovascular compression of the lateral medulla.

[Metastasis of pleural malignant mesothelioma to the brain and upper maxilla: description of 2 cases]. / Metastasi di mesotelioma maligno pleurico all'encefalo e al mascellare superiore: descrizione di due casi.

Two cases of malignant mesothelioma metastatic to the brain and maxillary bone respectively are reported. This latter localization has never been described in literature. The diagnosis resided primarily on immunohistochemistry.

Therapeutic considerations in cerebellopontine angle lipomas inducing hemifacial spasm.

Lipoma is a very rare tumour at the cerebellopontine angle. We report a case of incomplete hemifacial spasm, associated with a lipoma involving and compressing both facial and acoustic nerves at their origin in the brainstem. The patient was treated with medical therapy (botulinum toxin A) and surgery. We present a review of the last ten years of the literature, with particular regard to management.

Small acoustic neuromas: monitoring the growth rate by MRI.

In a proportion of small acoustic neuroma patients, monitoring with magnetic resonance imaging shows no volumetric increase of tumour size over the years. The object of the study was to identify some indications for the clinical choice between immediate surgery (with the related risks) and watchful waiting. We performed a retrospective study of 47 non-surgically-treated patients affected by acoustic neuroma and monitored by gadolinium-enhanced MRI between January 1990 and February 1999. Six clinical variables (tumour size, sex, age, initial symptoms, ABR pattern and duration of the symptoms) were examined by univariate analysis. Chi-square test and variance analysis were performed to evaluate the statistical significance. In 30/47 (63.8%) cases, no growth was observed during the entire period of follow-up. In the remaining 17/47 (36.2%) patients, a volumetric increase was detected, most often within the first year of observation. The clinical factors examined did not significantly correlate with growth. Despite the relatively short period of observation, we believe that immediate surgery does not need to be considered mandatory for small acoustic neuromas, even in young patients. However the irregular behaviour of the tumour underlines the importance of monitoring with MRI at least once a year.

Expanding lacunae causing triventricular hydrocephalus. Report of two cases.

Two patients are reported in whom the presence of triventricular hydrocephalus and aqueductal obstruction or stenosis due to multiple expanding lacunae in the mesencephalothalamic region possibly corresponds to abnormally dilated perivascular spaces. Placement of a ventriculoperitoneal cerebrospinal fluid (CSF) shunt in one patient and the performance of a third ventricle cisternotomy in the other reversed the hydrocephalic syndrome, but did not modify the complex neuroophthalmological disturbance and rubral tremor presumably related to the compressive effects of the lacunae on adjacent parenchyma. In one patient the number and size of the lacunae were increased 4 years after CSF shunt placement. A review of the literature revealed two cases in which magnetic resonance imaging demonstrated a similar, poorly understood pathological condition.

High-performance liquid chromatographic analysis of physiological amino acids in human brain tumors by pre-column derivatization with phenylisothiocyanate.

A reversed-phase high-performance liquid chromatographic technique for the determination of free amino acids in five biopsies of human brain tumors (two meningiomas, one glioblastoma and two oligodendrogliomas) is described. The frozen tissues were homogenized, deproteinized with perchloric acid and neutralized with potassium hydroxide. Aliquots of the supernatant containing the physiological amino acids are used for pre-column derivatization with phenylisothiocyanate. The derivatized PTC-amino acids (phenylthiocarbamyl derivatives) are stable for a five day period if stored as a powder at -20 degrees C in an inert atmosphere and they can be analyzed on a reversed-phase column (PicoTag) using a gradient of two eluents with absorption detection at a wavelength of 254 nm. Good resolution of several amino acids (>30) is achieved within ca. 60 min. For most amino acids this method is suitable for an accurate measurement over a wide range of physiological concentrations (50-400 pmol) starting from a very small amount of sample.

Osseous metaplasia in a growth hormone-secreting pituitary adenoma.

A case of growth hormone-secreting adenoma of the pituitary gland showing osseous metaplasia is described in a 56-year-old acromegalic female. The tumor was composed of nests of densely granulated cells separated by and intermixed with calcifications, trabeculae of mature bone and fat. Calcifications are seldom found in pituitary adenomas. In rare instances, calcium deposits can be prominent enough to lead to the formation of pituitary stones and bone which replace the entire tumor mass. Analogously with metaplastic meningiomas, we propose using the term metaplastic adenoma to define cases with osseous metaplasia in order to distinguish between lesions containing bone from the more frequently seen calcified adenomas.

Cytogenetic investigation of chordomas of the skull.

We report the first cytogenetic investigation of cranial chordoma. Three cranial chordomas were examined, two of which could be further histopathologically classified as chondroid chordomas. In addition, we have included a case of chordoma of a cervical vertebra to compare the cytogenetic abnormalities. Diagnosis was made at histological and immunohistochemical levels. The three cases of cranial chordoma showed a normal karyotype, while one vertebra showed 46,XY,t(6;11)(q12;q23). Chordomas, particularly those containing cartilage, have to be distinguished from chondrosarcomas of the skull base. Such a distinction is normally based on expression of epithelial markers which usually are lacking in chondrosarcoma. Cytogenetic investigation may eventually prove to be useful in the distinction of the two lesions, if chromosome anomalies are consistently absent in chordoma, although some chondrosarcomas may also present a normal karyotype. Such a distinction has clinical implications because chondroid chordomas show better survival, whereas chondrosarcomas show a propensity to infiltrate the surrounding tissues.

[Cytogenetics of tumors of the central nervous system: a case study and review of the literature]. / Citogenetica di tumori del sistema nervoso centrale: studio casistico e revisione della letteratura.

The introduction of cytogenetic techniques, especially chromosome banding techniques, has facilitated a more detailed study of the chromosomal basis of hematological malignancies and solid tumours. With the advent of molecular cell biology many new insights have been gained into the pathogenetic mechanisms of cancer. With G banding technique we have studied chromosomal aberrations of 174 tumours of the Central Nervous System in adults and the results obtained have been compared with the literature data.

[Intracranial location of a case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) mimicking meningioma]. / Localizzazione intracranica di un caso di istiocitosi dei seni con linfoadenopatia massiva (malattia di Rosai-Dorfman) simulante un meningioma.

Sinus histiocytosis with massive lymphadenopathy was first described in 1969 in lymphnodes by Rosai and Dorfman ad subsequently in other organs including skin, bone ad soft tissue. Extranodal involvement has also been recognised, but central nervous system manifestations are extremely rare and clinical and radiological findings are suggestive of meningioma. We report a meningeal nodule in a young woman, clinically diagnosed as possible meningioma. A review of literature of previously reported intracranial lesions is presented.