Surgical management of a rare myxopapillary ependymoma of the gluteal region: A case report.

BACKGROUND: Ependymomas are rare tumors originating from neuroepithelial cells lining the wall of the ventricles or central canal of the spinal cord. While these tumors mainly occur within the central nervous system (CNS), there are occasional reports in children and young adult patients with a primary tumor occurrence outside of the CNS. Ependymomas of the sacrococcygeal region have been infrequently described in the literature with no standard of care established. We present a case report and review of the literature regarding this rare entity. CASE DESCRIPTION: A 24-year-old woman presented with right gluteal pain worsened by sitting and a palpable soft tissue mass of the sacrococcygeal region. Magnetic resonance imaging revealed a 3.7 cm cystic mass centered in the right gluteal region. She underwent a biopsy at an outside institution, with histology revealing myxopapillary ependymoma. The patient was referred to our hospital and underwent an interdisciplinary neurosurgical and orthopedic oncology en bloc resection of the ependymoma, which intraoperatively appeared to originate from the coccygeal nerve. CONCLUSION: In the present report, the authors demonstrate that a myxopapillary ependymoma may present as an isolated gluteal mass attached to the coccygeal nerve, without frank CNS involvement. Furthermore, an interdisciplinary approach to surgical resection of this lesion appears to represent an effective treatment modality.

Multimodal management of giant cerebral aneurysms: review of literature and case presentation.

The pathophysiology of giant cerebral aneurysms renders them difficult to treat. Advances in technology have attempted to address any shortcomings associated with open surgery or endovascular therapies. Since the introduction of the flow diversion technique, the endovascular approach with flow diversion has become the first-line modality chosen to treat giant aneurysms. A subset of these giant aneurysms may persistent despite any treatment modality. Perhaps the best option for these recurrent and/or persistent giant aneurysms is to employ a multimodal approach-both surgical and endovascular-rather than any single technique to provide a curative result with favourable patient outcomes. This paper provides a review of the histopathology and treatment options for giant cerebral aneurysms. Additionally, an illustrative case is presented to highlight the unique challenges of a curative solution for giant cerebral aneurysms that persist despite initial treatment.

Vasospasm in the setting of traumatic bilateral carotid-cavernous fistulas and its effect on treatment.

BACKGROUND: Direct, Type A, cavernous-carotid fistulas (CCFs) are predominantly caused by head trauma, especially when basilar skull fractures are present. Transarterial endovascular treatment of direct CCFs is the preferred method of treatment. Bilateral CCFs are estimated to be present in 1-2% of the cases. The treatment of bilateral CCFs is difficult often requiring a combination of endovascular and open surgical approaches. CASE DESCRIPTION: We present a case of traumatic bilateral CCFs presenting with vasospasm of the anterior circulation seen on the initial angiogram on day 1 and our treatment paradigm. CONCLUSION: This case illustrates the challenges in managing bilateral CCFs as well as the changes in collateral circulation because of cerebral vasospasm which affected our treatment paradigm.

Screening individuals with intracranial aneurysms for abdominal aortic aneurysms is cost-effective based on estimated coprevalence.

OBJECTIVE: Aneurysm rupture is a major cause of morbidity and mortality, and evidence suggests shared risk for both abdominal aortic aneurysms (AAAs) and intracranial aneurysms (IAs). We hypothesized that screening for AAA in patients with known IA is cost-effective. METHODS: We used a decision tree model to compare costs and outcomes of AAA screening vs no screening in a hypothetical cohort of patients with IA. We measured expected outcomes using quality-adjusted life-years (QALYs) and the incremental cost-effectiveness ratio (ICER). We performed a Monte Carlo simulation and additional sensitivity analyses to assess the effects of ranging base case variables on model outcomes and identified thresholds where a decision alternative dominated the model (both less expensive and more effective than the alternative). RESULTS: In our base case analysis, screening for AAA provided an additional 0.17 QALY (2.5-97.5 percentile: 0.11-0.27 QALY) at a saving of $201 (2.5-97.5 percentile: $-127 to $896). This yielded an ICER of $-1150/QALY (2.5-97.5 percentile: $-4299 to $6374/QALY), that is, screening saves $1150 per QALY gained. CONCLUSIONS: Based on this model, screening for AAA in individuals with IA is cost-effective at an ICER of $1150/QALY, well below accepted societal thresholds estimated at $60,000/QALY. Cost-effectiveness of cross-screening in these populations is sensitive to aneurysm coprevalence and risk of rupture. Further prospective study is warranted to validate this finding.

The predictive value of magnetic resonance imaging in evaluating intracranial arteriovenous malformation obliteration after stereotactic radiosurgery.

OBJECT: The current gold standard for diagnosing arteriovenous malformation (AVM) and assessing its obliteration after stereotactic radiosurgery (SRS) is digital subtraction angiography (DSA). Recently, MRI and MR angiography (MRA) have become increasingly popular imaging modalities for the follow-up of patients with an AVM because of their convenient setup and noninvasiveness. In this study, the authors assessed the sensitivity and specificity of MRI/MRA in evaluating AVM nidus obliteration as assessed by DSA. METHODS: The authors study a consecutive series of 136 patients who underwent SRS between January 2000 and December 2012 and who underwent regular clinical examinations, several MRI studies, and at least 1 post-SRS DSA follow- up evaluation at the University of Virginia. The average follow-up time was 47.3 months (range 10.1-165.2 months). Two blinded observers were enrolled to interpret the results of MRI/MRA compared with those of DSA. The sensitivity, specificity, positive predictive value, and negative predictive value for the obliteration of AVM were reported. RESULTS: On the basis of DSA, 73 patients (53.7%) achieved final angiographic obliteration in a median of 28.8 months. The sensitivity (the probability of finding obliteration on MRI/MRA among those for whom complete obliteration was shown on DSA) was 84.9% for one observer (Observer 1) and 76.7% for the other (Observer 2). The specificity was 88.9% and 95.2%, respectively. The false-negative interpretations were significantly related to the presence of draining veins, perinidal edema on T2-weighted images, and the interval between the MRI/MRA and DSA studies. CONCLUSIONS: MRI/MRA predicted AVM obliteration after SRS in most patients and can be used in their follow-up. However, because the specificity of MRI/MRA is not perfect, DSA should still be performed to confirm AVM nidus obliteration after SRS.

Seizure and anticonvulsant outcomes following stereotactic radiosurgery for intracranial arteriovenous malformations.

OBJECT: Epilepsy associated with arteriovenous malformations (AVMs) has an unclear course after stereotactic radiosurgery (SRS). Neither the risks of persistent seizures nor the requirement for postoperative antiepileptic drugs (AEDs) are well defined. METHODS: The authors performed a retrospective review of all patients with AVMs who underwent SRS at the University of Virginia Health System from 1989 to 2012. Seizure status was categorized according to a modified Engel classification. The effects of demographic, AVM-related, and SRS treatment factors on seizure outcomes were evaluated with logistic regression analysis. Changes in AED status were evaluated using McNemar's test. RESULTS: Of the AVM patients with pre- or post-SRS seizures, 73 with pre-SRS epilepsy had evaluable data for subsequent analysis. The median patient age was 37 years (range 5-69 years), and the median follow-up period was 65.6 months (range 12-221 months). Sixty-five patients (89%) achieved seizure remission (Engel Class IA or IB outcome). Patients presenting with simple partial or secondarily generalized seizures were more likely to achieve Engel Class I outcome (p = 0.045). Twenty-one (33%) of 63 patients tapered off of pre-SRS AEDs. The incidence of freedom from AED therapy increased significantly after SRS (p < 0.001, McNemar's test). Of the Engel Class IA patients who continued AED therapy, 54% had patent AVM nidi, whereas only 19% continued AED therapy with complete AVM obliteration (p = 0.05). CONCLUSIONS: Stereotactic radiosurgery is an effective treatment for long-term AVM-related epilepsy. Seizure-free patients on continued AED therapy were more likely to have residual AVM nidi. Simple partial or secondarily generalized seizure type were associated with better seizure outcomes following SRS.

Stereotactic radiosurgery for facial nerve schwannomas: A preliminary assessment and review of the literature.

OBJECTIVE: Facial nerve schwannomas (FNS) are rare tumors, and their appropriate management remains the subject of considerable debate. This report details the results of a series of patients with FNS treated with stereotactic radiosurgery (SRS) at the University of Virginia. METHODS: We performed a retrospective review of the clinical and imaging outcomes of 5 patients who underwent Gamma Knife RS (GKRS) for small-to-medium-sized (<5 mL) FNS at our institution. The study population consisted of 3 males and 2 females with a median age of 35 years. All patients presented with varying degrees of facial palsy and/or hearing dysfunction. Tumor volumes at GKRS ranged from 0.1 to 5 (median=0.8) mL. The median maximum radiosurgical dose and tumor margin dose were 24 Gy and 12 Gy, respectively. RESULTS: After a median follow-up period of 12 (range, 6-36) months, three tumors were radiographically smaller and two remained stable. Facial function improved in three patients, remained stable in one patient, and slightly declined from House-Brackmann grade I to II in one patient. Hearing function was preserved in three patients and deteriorated in two patients, one from Gardner-Robertson grade I to II and the other from serviceable hearing grade II to III. CONCLUSION: SRS appears to offer a reasonable rate of facial nerve preservation and tumor control for patients with small-to-medium-sized FNS. Considering the published outcomes achieved with resection, RS may be the preferred first-line treatment for these tumors.