Giant cell ependymoma of the thoracic spine: pathology case report.

UNLABELLED: INTRODUCTION AND IMPORTANCE: Spinal ependymomas are slow-growing lesions that comprise the majority of primary spinal cord neoplasms. When surgery is indicated, the extent of tumor removal is most prognostic for long-term survival. Unusual histological subtypes can make intraoperative diagnosis spurious, possibly altering the surgical approach from gross total resection for ependymomas to debulking for high-grade astrocytomas. CLINICAL PRESENTATION: We describe a 67-year-old woman with a thoracic spine intramedullary giant cell ependymoma. She presented with decreased lower extremity sensation leading to unsteadiness and an eventual fall. A physical examination revealed lower extremity hyperreflexia and ankle clonus, but no clear sensory level. Magnetic resonance imaging demonstrated an intramedullary T1 and T2 hypointense, homogenously enhancing lesion at T8 with extensive cephalad and caudal edema. INTERVENTION AND TECHNIQUE: A laminectomy at T8 to T9 afforded gross total resection of the lesion that had a clear cleavage plane with normal spinal cord. Intraoperative pathology suggested a high-grade glioblastoma, but final section showed sporadic giant cells with marked pleomorphism, uniform immunofluorescence staining with both glial fibrillary acidic protein and cluster of differentiation 99, and high MIB-1 index. Electron microscopy showed "zipper-like" junctions. There were no detected genomic abnormalities consistent with glioblastoma. CONCLUSION: We present this first reported case of thoracic spine giant cell ependymoma alongside scant literature yielding 1 case in the cervical spine and 2 cases at the filum terminale. Those cases had benign courses, whereas ours demonstrates a high degree of proliferation, making the malignant potential difficult to assess.

Brain tumors and epilepsy: pathophysiology of peritumoral changes.

Epilepsy commonly develops among patients with brain tumors, frequently even as the presenting symptom, and such patients consequently experience substantial morbidity from both the seizures and the underlying disease. At clinical presentation, these seizures are most commonly focal with secondary generalization and conventional medical management is often met with less efficacy. The molecular pathophysiology of these seizures is being elucidated with findings that both the tumoral and peritumoral microenvironments may exhibit epileptogenic phenotypes owing to disordered neuronal connectivity and regulation, impaired glial cell function, and the presence of altered vascular supply and permeability. Neoplastic tissue can itself be the initiation site of seizure activity, particularly for tumors arising from neuronal cell lines, such as gangliogliomas or dysembryoblastic neuroepithelial tumors. Conversely, a growing intracranial lesion can both structurally and functionally alter the surrounding brain tissue with edema, vascular insufficiency, inflammation, and release of metabolically active molecules, hence also promoting seizure activity. The involved mechanisms are certain to be multifactorial and depend on specific tumor histology, integrity of the blood brain barrier, and characteristics of the peritumoral environment. Understanding these changes that underlie tumor-related epilepsy may have roles in both optimal medical management for the seizure symptom and optimal surgical objective and management of the underlying disease.

Modifiable risk factors for intracerebral hemorrhage: study of anticoagulated patients.

OBJECTIVE: To determine whether there are modifiable risk factors for spontaneous intracerebral hemorrhage in patients receiving oral anticoagulation (OAC) therapy. DESIGN: Retrospective chart review between January 2002 and December 2004. PARTICIPANTS: A total of 315 consecutive patients presenting with spontaneous intracerebral hemorrhage. MAIN OUTCOME MEASURES: Overall mortality rates and surgical mortality rates, and discharge home compared with discharge to a long-term care facility. RESULTS: Of the 315 patients reviewed, 65 (20.6%) were receiving OAC therapy. Age, Glasgow Coma Scale score, and size of hematoma at presentation were similar in the 65 patients taking OAC and the 250 patients not taking it. Mean arterial pressure at presentation was significantly higher in the OAC group than in the control group (132 mm Hg vs 107 mm Hg, P = .01) as was the number of hematomas that progressed (52% vs 14%, P = .01). Overall mortality rates were higher in the OAC group than in the control group (52% vs 41%, P = .03) as were surgical mortality rates (62% vs 41%, P = .04). There were no significant differences in morbidity between the 2 groups. CONCLUSION: Mortality rates were higher among patients taking OAC therapy despite their having similarly sized hematomas at presentation. The higher initial mean arterial pressure among such patients has not been described previously in this setting. This higher mean arterial pressure correlates with the propensity of these patients' hematomas to expand after initial imaging and might partially mediate the mortality effect. In patients taking OAC, hypertension appears to be a modifiable risk factor for morbidity and mortality from intracerebral hemorrhage.

Clinical predictors of facial nerve outcome after translabyrinthine resection of acoustic neuromas.

PURPOSE: The translabyrinthine approach to acoustic neuroma resection offers excellent exposure for facial nerve dissection with 95% preservation of anatomic continuity. Acceptable outcome in facial asymptomatic patients is reported at 64-90%, but transient postoperative deterioration often occurs. The objective of this study was to identify preoperative clinical presentation and intraoperative surgical findings that predispose patients to facial nerve dysfunction after acoustic neuroma surgery. METHODS: The charts of 128 consecutive translabyrinthine patients were examined retrospectively to identify new clinical and intraoperative predictors of facial nerve outcome. Postoperative evaluation of patients to normal function or mild asymmetry upon close inspection (House-Brackmann grades of I or II) was defined as an acceptable outcome, with obvious asymmetry to no movement (grades III to VI) defined as unacceptable. Intraoperative nerve stimulation was performed in all cases, and clinical grading was performed by a single neurosurgeon in all cases. RESULTS: Among patients with no preoperative facial nerve deficit, 87% had an acceptable result. Small size (P < 0.01) and low intraoperative nerve stimulation of < 0.10 mA (P< 0.01) were reaffirmed as predictive of functional nerve preservation. Additionally, preoperative tinnitus (P = 0.03), short duration of hearing loss (P< 0. 01), and lack of subjective tumour adherence to the facial nerve (P = 0.02) were independently correlated with positive outcome. CONCLUSIONS: Our experience with the translabyrinthine approach reveals the previously unestablished associations of facial nerve outcome to include presence of tinnitus and duration of hypoacusis. Independent predictors of tumour size and nerve stimulation thresholds were reaffirmed, and the subjective description of tumour adherence to the facial nerve making dissection more difficult appears to be important.

Syndromic and sporadic pediatric optic pathway gliomas: review of clinical and histopathological differences and treatment implications.

Optic pathway gliomas (OPGs) are the most common primary neoplasm of the optic pathway. These lesions usually present in childhood and can arise anywhere along the optic pathway; they occur more frequently in women; and they rarely undergo late progression. Management strategies after the initial diagnosis are controversial, compounded by the different behaviors exhibited by sporadic and syndromic tumors. Neurofibromatosis Type 1 (NF1), with aberrant oncogenic signaling and consequent predisposition to intracranial tumors, is the most common associated syndrome, with nearly 20% of NF1 patients developing OPGs. A comorbid NF1 diagnosis has implications for tumor location with greater predilection for optic nerve involvement, whereas chiasmal and postchiasmal lesions are more frequently seen in sporadic cases. Syndromic OPGs often exhibit more indolent behavior and lower rates of clinical progression, and the majority of these are diagnosed by routine neuroophthalmological screening. When treatment is indicated, however, the molecular abnormalities that constitute this syndrome can limit the available chemotherapy and radiotherapy options because clinicians fear secondary malignancy and cerebrovascular complications. Furthermore, radiotherapy early in life can impair an individual's intellectual development, endocrine function, and physical growth, thereby limiting the role of this modality in the treatment of this childhood lesion. Differential gene expression and histogenesis among sporadic and syndromic OPGs may account for the different tumor behaviors, but studies correlating specific genetic and proteomic changes with patient outcome are pending. Loss of heterozygosity at 10 and 17q are more common among patients with NF1, and Ki67 labeling intensity of 2-3% and low p53 labeling intensity seem prognostic of aggressive tumor behavior. Recent advances in the development of a preclinical mouse model of NF1-associated OPG will permit investigation into improved detection strategies and chemotherapeutic and radiotherapy treatment protocols.

Myofibroblastic sarcoma in meningioma: a new variant of "metaplastic" meningioma.

The authors describe a mixed malignant dural tumor composed of meningioma and myofibroblastic sarcoma (MFS). The meningioma component displayed epithelial membrane immunoreactivity and interdigitating cellular processes with desmosomal junctions on electron microscopy. MFS cells were immunoreactive for smooth muscle actin and vimentin, and focally for factor XIIIa, CD31, CD34, and Ulex europeus lectin receptors. Electron microscopy showed collections of intermediate filaments, stress fibers, subsarcolemmal densities of microfilaments, occasional fibronexus fibrils, few pinocytic vesicles, and discontinuous external lamina. After gross total removal, the tumor recurred 1 year later as aggressive MFS only. Development of MFS in continuity with meningioma suggests induction of MFS by meningioma or a divergent differentiation of precursor of the neoplastic arachnoid cell.