Simple and complex forms of dysembryoplastic neuroepithelial tumors (DNTs) are readily recognizable but forms with diffuse growth pattern, and hybrid tumors, that is, mixed DNT and ganglioglioma (DNT/GG), are more contentious entities. Rare DNTs have shown aggressive behavior. We reviewed cortical growth patterns, immunophenotype (including CD34, nestin and calbindin), genetic profile, and outcome in 101 DNT in adults. Simple (n = 18), complex (n = 31), diffuse (n = 35) DNT, and mixed DNT/GG (n = 17) showed no difference in age of onset, associated seizure type, or outcome (67.5% free from seizure; mean follow-up, 6 years). CD34 was seen in 61%, calbindin in 57%, and nestin in 86% of all DNT types; these markers were less common in simple DNT. Peritumoral cortical changes (Layer I hypercellularity [61%], satellite nodules [51.6%]) were frequent, but dyslamination (cortical dysplasia) was not identified. Molecular genetic abnormalities identified in 17 cases were IDH1 mutation (n = 3), 1p/19q loss (n = 10), isolated loss 9q (n = 2), and PTEN loss (n = 3), which were not associated with tumor type or location, higher cell proliferation, or distinguishing clinical features (mean age of epilepsy onset, 9 years; age at surgery = 31 years; 69% free from seizure); none had progression on magnetic resonance imaging (mean follow-up, 6 years). No single feature was predictive of seizure-free outcome, but there was a trend for better outcome in CD34-positive tumors (p = 0.07). One case has shown transformation to a higher grade. This study supports the existence of a range of subtypes of DNT some with overlapping features with ganglioglioma; molecular genetic abnormalities were not predictive of atypical behavior.
The evidence is neutral, neither for nor against seizure prophylaxis, in people with brain tumors. These conclusions apply only for the antiepileptic drugs phenytoin, phenobarbital, and divalproex sodium. The decision to start an antiepileptic drug for seizure prophylaxis is ultimately guided by assessment of individual risk factors and careful discussion with patients.
Background: Traumatic clival epidural hematoma is an extremely rare reported entity. Case Description: We describe the case of a 26-year-old woman involved in a car accident who presented with a Glasgow Coma Scale score of 13, bilateral abducens palsy, bilateral numbness on the mandibular territory of the trigeminal nerve, and left hypoglossal palsy. Radiological examinations revealed a clival epidural hematoma. The patient was managed conservatively, with clinical improvement of her neurological condition. This is the first traumatic clival epidural hematoma reported in an adult. From a review of the literature, we found only 8 cases. Conclusion:The pathophysiology of these hematomas is still a subject of debate; occipitoatlantoaxial ligamentous instability may play a role in it. In one third of the cases, bilateral cranial nerve palsies were associated. Apparently, they have a benign outcome. D
In patients with basilar skull fracture, there is inadequate evidence to recommend for or against antibiotic prophylaxis in the prevention of meningitis, irrespective of cerebrospinal fluid leakage. METHODS DATA SOURCES The authors electronically searched the Cochrane Central Register of Controlled Trials (February 2011), MEDLINE, EMBASE, and Literatura Latino-Americana e do Caribe em Ciências da Saúde (LILACS) and hand searched abstracts of neurosurgical conference proceedings and reference lists. Experts in the field were contacted to request additional sources. No language or publication restrictions were applied. STUDY SELECTIONStudies were included if they were randomized controlled trials comparing antibiotics versus placebo or no intervention after basilar skull fracture. Primary outcome was the development of meningitis. Similar observational trials were also identified for inclusion in a separate meta-analysis. Three authors independently identified potentially suitable articles from the search strategy, and disagreements were resolved by discussion with the fourth author. DATA EXTRACTION AND SYNTHESISTwo authors independently extracted data with standardized data collection forms and globally
Epithelioid hemangioendothelioma is a rare tumor of intermediate malignancy. Twelve intracranial cases have been well documented. We report an intra- and extra-cranial single mass epithelioid hemangioendothelioma. We describe the case of a 27-year-old male who presented a left temporal and retroauricular pain and an intra and extra-cranial mass extending to the left infratemporal fossa. The neoplasm was isointense with gray matter in both T1 and T2-weighted images, showing marked nodular gadolinium enhancement. About 90% of the tumor was surgically removed. The neoplasm was fibrous, well defined and arose from the left temporal artery branches. Recovery was uneventful. The histological diagnosis was epithelioid hemangioendothelioma. Enhanced magnetic resonance scan was repeated at 3 and 6 months after surgery, showing an increasing mass volume extending to the surrounding tissues, including intracranially, infratemporal fossa and left orbit. The patient died 8 months after the initial diagnosis. Epithelioid hemangioendotheliomas can be very aggressive tumors, presenting initially at an intra- and extra-cranial location, due to its fast growth. From our review of the literature, they seem to have an equal gender distribution (M : F = 7 : 6) and tend to affect people under the age of 30 (10/13). We report a unique and extremely aggressive tumor. The rarity of reports and the continuous spectrum of differentiation, ranging from borderline to highly malignant are obstacles to identifying initial therapeutic protocols and the adjunctive therapy after surgery.
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