Epilepsy in primary cerebral tumors: the characteristics of epilepsy at the onset (results from the PERNO study--Project of Emilia Romagna Region on Neuro-Oncology).

PURPOSE: To present new information on the semiology and short-term evolution of seizures associated with primary brain tumors (PBTs) in a prospective study. METHODS: This study is a section of the PERNO study--Project of Emilia Romagna Region on Neuro-Oncology, the main aim of which is to collect prospectively all cases of PBTs occurring in the Emilia-Romagna region, northeast Italy (3,983,346 population) from January 2009 to December 2011, to allow epidemiologic, clinical, and biomolecular studies. The epilepsy section of the PERNO study included all the patients who experienced seizures, either as first symptom of the tumor or appearing during the course of the disease. Each patient was interviewed by the referring neurologist with a specific interest in epilepsy. The patients who entered the study were followed up with visits on a quarterly basis. KEY FINDINGS: We collected 100 cases with full clinical, neuroradiologic, and pathologic data. The majority (79%) had high grade PBTs (glioblastoma in 50 cases), whereas the remaining patients had low-grade gliomas, mostly localized in the frontal (60%), temporal (38%), and parietal (28%) lobes. Seizures were the first symptom of the tumor in 72 cases. Overall, the initial seizures were tonic-clonic (48%) (without clear initial focal signs in more than half of the patients), focal motor (26%), complex partial (10%), and somatosensitive (8%). The majority of cases (60%) had isolated seizures or a low seizure frequency at the onset of the disease, whereas a high seizure frequency or status epilepticus was observed in 18% and 12% of cases, respectively. Ninety-two patients underwent surgical removal of the tumor, which was either radical (38%) or partial (53%). Seven patients underwent only cerebral biopsy. In the 72 patients in whom seizures were the first symptom, the mean time to the surgical treatment was 174 days, with a significant difference between high grade (95 days) and low grade (481 days) gliomas. At the time of our first observation, the majority of patients (69%) had already undergone surgical removal, with a mean follow-up of 3 months after the procedure. Overall, 39 patients (56%) were seizure free after tumor removal. The good outcome did not depend on presurgical seizure frequency or tumor type, although there was a trend for better results with low-grade PBTs. SIGNIFICANCE: These data provide evidence that seizures are strictly linked to the tumoral lesion: They are the initial symptom of the tumor, reflect the tumor location and type, are usually resistant to antiepileptic treatment, and may disappear after the treatment of the lesion.

Chromosome 1p and 19q evaluation in low-grade oligodendrogliomas: a descriptive study.

Oligodendrogliomas are rare primary brain tumors with variable patient outcomes which are not always adequately accounted for by clinical or pathological variables. The present study evaluated the prognostic implications of chromosome 1p and 19q status in a set of 23 low grade oligodendrogliomas (OGD II), and correlated the results with patient outcome. Loss of heterozygosity (LOH) and fluorescent in situ hybridization (FISH) analyses, the most widely used standard procedures, were used. 1p and 19q deletions were found in 65 and 61% of cases, respectively, using FISH and in 78 and 72% of cases using LOH. Both deletions were found in 56 and 64% of patients using FISH and LOH, respectively. Concordance between the results from the two techniques, determined by the Kappa statistics, ranged from fair to substantial depending on whether single or combined deletions were considered. Our results showed that the molecular alterations are associated with age and tumor localization. With regard to the impact of chromosomal alterations on clinical outcome, chromosome 19q deletions detected by LOH would seem to indicate a subgroup of patients at a higher risk of relapse, although the small number of patients recruited does not permit any definitive conclusions to be drawn. Further studies are now ongoing to determine whether this methodological approach could be potentially useful in low grade oligodendrogliomas to better characterize chromosomal alterations of 1p/19q and identify subgroups of patients with a higher risk of disease recurrence.

Follicular lymphoma of the dura associated with meningioma: a case report and review of the literature.

A 70-year-old immunocompetent woman who had been radiologically diagnosed with a left parasagittal meningioma 5 years previously developed a palpable subgaleal mass and underwent neurosurgery. Histologically the tumor was composed of thickened fibrotic dural tissue, infiltrated by a follicular lymphoma, of grade IIIB. A coexistent transitional meningioma had been infiltrated by the neoplastic lymphoid tissue. Two months after surgery, the patient developed evidence of extracranial dissemination of the lymphoma to the cervical lymph nodes and was treated with polychemotherapy plus Rituximab and intrathecal methotrextate. The patient is alive and recurrence-free at 1-year follow up. A review of the literature indicates that lymphomas developing primarily in the meninges, have an indolent course and tend to be localized in areas rich in meningothelial cells.

Gliosarcoma with features of osteoblastic osteosarcoma: a review.

CONTEXT: Gliosarcoma is a rare tumor of the central nervous system characterized by a biphasic histologic pattern, consisting of a gliomatous and a sarcomatous component, respectively. In most instances the sarcomatous component is represented by a fibrosarcoma, but other stromal malignancies have also been described. Osteosarcomatous differentiation in gliosarcoma has been rarely reported. OBJECTIVE: To review characteristic radiologic and histopathologic features of this rare neoplasm, to debate about possible differential diagnoses that should be taken into consideration, and to provide an overview of the potential histopathogenesis of gliosarcomas. DATA SOURCES: Relevant articles indexed in PubMed (National Library of Medicine) and reference medical texts. CONCLUSIONS: Recent molecular studies suggest that sarcomatous and gliomatous components of gliosarcoma might be derived from a single precursor cell clone, progressing in 2 subclones with distinct morphologic features during tumor evolution. Nonetheless, events determining splitting of the original clone into 2 histologic populations remain to be investigated.

Traumatic encephalocele related to orbital roof fractures: report of six cases and literature review.

BACKGROUND: Orbital roof fractures after blunt injury are rare. Traumatic encephaloceles in the orbital cavity are even rarer, with only 15 cases published to date. METHODS: The clinical, radiological, and surgical findings of 6 cases of traumatic encephalocele treated at our institution from June 1998 to January 2000 are presented. They are also compared with previously published series. RESULTS: In contrast to other published cases, 5 out of 6 patients in our series were adults. The most common cause of trauma was road traffic accident. Ecchymosis and preoperative exophthalmos/proptosis were frequent. In all of our patients a coronal CT scan (3 mm increments with bone windows) was obtained. It demonstrated the extension of the orbital roof fractures and a possible encephalocele in 4 cases. Associated frontal brain contusions were seen in 5 cases. An MRI was performed in 3 patients (and only in 2 previously published cases); it showed the extension of the brain herniation into the orbital cavity. Surgical treatment via a fronto-basal approach with evacuation of the contused herniated brain tissue and orbital roof reconstruction was performed. The outcome at 6 months was good recovery in five patients with one patient still in a persistent vegetative state. Postoperatively the ocular disturbances improved in 5 cases. A review of the other published cases confirmed recovery of normal ocular function in the vast majority of the cases. CONCLUSIONS: Whenever orbital roof fractures associated with frontal contusions are identified in an acute brain injured patient, an orbital encephalocele should be suspected. In our opinion MRI is the investigation of choice in such patients. If the encephalocele is confirmed, a surgical approach via the subfrontal route is indicated with resection of herniated contused brain tissue, dural closure, and orbital roof reconstruction. Good results in regard to the orbital symptoms (mainly exophthalmos) can be expected.