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Marec von Lehe and Johannes Schramm

Object

In this paper, the authors' goal was to summarize their experience with the surgical treatment of gliomas arising from the cingulate gyrus.

Methods

The authors analyzed preoperative data, surgical strategies, complications, and functional outcome in a series of 34 patients (mean age 42 years, range 12–69 years; 14 females) who underwent 38 operations between May 2001 and November 2008.

Results

In 7 cases (18%) the tumor was located in the posterior (parietal) part of the cingulate gyrus, and in 31 (82%) the tumor was in the anterior (frontal) part. In 10 cases (26%) the glioma was solely located in the cingulate gyrus, and in 28 cases (74%) the tumor extended to the supracingular frontal/parietal cortex. Most cases (23 [61%]) had seizures as the presenting symptom, 8 patients (24%) suffered from a hemiparesis/hemihypesthesia, and 4 patients (12%) had aphasic symptoms.

The authors chose an interhemispheric approach for tumor resection in 11 (29%) and a transcortical approach in 27 (71%) cases; intraoperative electrophysiological monitoring was applied in 23 (61%) and neuronavigation in 15 (39%) cases. A > 90% resection was achieved in 32 (84%) and > 70% in another 5 (13%) cases. Tumors were classified as low-grade gliomas in 11 cases (29%). A glioblastoma multiforme (WHO Grade IV, 10 cases [26%]) and oligoastrocytoma (WHO Grade III, 9 cases [24%]) were the most frequent histopathological results.

Postoperatively, patients in 13 cases suffered from a transient supplementary motor area syndrome (34%), all of whom had tumors in the anterior cingulate gyrus. In the early postoperative period (30 days) a new deficit occurred in 5 cases (13%, mild motor deficits or aphasic symptoms). One patient had a major bleeding episode 2 days after surgery and was in a persistent vegetative state.

Conclusions

Gliomas arising from the cingulate gyrus are rare. A gross-total resection is often possible and acceptably safe; intraoperative monitoring and neuronavigation are helpful adjuncts. In case of resection of gliomas arising from the anterior cingulate gyrus a supplementary motor area syndrome has to be considered, particularly when the tumor extends to the supracingular cortex

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Marec von Lehe and Johannes Schramm

Object

In this paper, the authors' goal was to summarize their experience with the surgical treatment of gliomas arising from the cingulate gyrus.

Methods

The authors analyzed preoperative data, surgical strategies, complications, and functional outcome in a series of 34 patients (mean age 42 years, range 12–69 years; 14 females) who underwent 38 operations between May 2001 and November 2008.

Results

In 7 cases (18%) the tumor was located in the posterior (parietal) part of the cingulate gyrus, and in 31 (82%) the tumor was in the anterior (frontal) part. In 10 cases (26%) the glioma was solely located in the cingulate gyrus, and in 28 cases (74%) the tumor extended to the supracingular frontal/parietal cortex. Most cases (23 [61%]) had seizures as the presenting symptom, 8 patients (24%) suffered from a hemiparesis/hemihypesthesia, and 4 patients (12%) had aphasic symptoms.

The authors chose an interhemispheric approach for tumor resection in 11 (29%) and a transcortical approach in 27 (71%) cases; intraoperative electrophysiological monitoring was applied in 23 (61%) and neuronavigation in 15 (39%) cases. A > 90% resection was achieved in 32 (84%) and > 70% in another 5 (13%) cases. Tumors were classified as low-grade gliomas in 11 cases (29%). A glioblastoma multiforme (WHO Grade IV, 10 cases [26%]) and oligoastrocytoma (WHO Grade III, 9 cases [24%]) were the most frequent histopathological results.

Postoperatively, patients in 13 cases suffered from a transient supplementary motor area syndrome (34%), all of whom had tumors in the anterior cingulate gyrus. In the early postoperative period (30 days) a new deficit occurred in 5 cases (13%, mild motor deficits or aphasic symptoms). One patient had a major bleeding episode 2 days after surgery and was in a persistent vegetative state.

Conclusions

Gliomas arising from the cingulate gyrus are rare. A gross-total resection is often possible and acceptably safe; intraoperative monitoring and neuronavigation are helpful adjuncts. In case of resection of gliomas arising from the anterior cingulate gyrus a supplementary motor area syndrome has to be considered, particularly when the tumor extends to the supracingular cortex

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Konstantinos Gousias, Johannes Schramm and Matthias Simon

OBJECTIVE

Recent advances in radiotherapy and neuroimaging have called into question the traditional role of aggressive resections in patients with meningiomas. In the present study the authors reviewed their institutional experience with a policy based on maximal safe resections for meningiomas, and they analyzed the impact of the degree of resection on functional outcome and progression-free survival (PFS).

METHODS

The authors retrospectively analyzed 901 consecutive patients with primary meningiomas (716 WHO Grade I, 174 Grade II, and 11 Grade III) who underwent resections at the University Hospital of Bonn between 1996 and 2008. Clinical and treatment parameters as well as tumor characteristics were analyzed using standard statistical methods.

RESULTS

The median follow-up was 62 months. PFS rates at 5 and 10 years were 92.6% and 86.0%, respectively. Younger age, higher preoperative Karnofsky Performance Scale (KPS) score, and convexity tumor location, but not the degree of resection, were identified as independent predictors of a good functional outcome (defined as KPS Score 90–100). Independent predictors of PFS were degree of resection (Simpson Grade I vs II vs III vs IV), MIB-1 index (< 5% vs 5%–10% vs >10%), histological grade (WHO I vs II vs III), tumor size (≤ 6 vs > 6 cm), tumor multiplicity, and location. A Simpson Grade II rather than Grade I resection more than doubled the risk of recurrence at 10 years in the overall series (18.8% vs 8.5%). The impact of aggressive resections was much stronger in higher grade meningiomas.

CONCLUSIONS

A policy of maximal safe resections for meningiomas prolongs PFS and is not associated with increased morbidity.

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Johannes Schramm, Karl Schaller, Jonas Esche and Azize Boström

OBJECTIVE

The objective of this study was to review the outcomes after microsurgical resection of cerebral arteriovenous malformations (AVMs) from a consecutive single-surgeon series. Clinical and imaging data were analyzed to address the following questions concerning AVM treatment in the post-ARUBA (A Randomized Trial of Unruptured Brain Arteriovenous Malformations) era. 1) Are the patients who present with unruptured or ruptured AVMs doing better at long-term follow-up? 2) Is the differentiation between Ponce Class A (Spetzler-Martin Grade I and II) patients versus Ponce Class B and C patients (Spetzler-Martin Grade III and IV) meaningful and applicable to surgical practice? 3) How did the ARUBA-eligible patients of this surgical series compare with the results reported in ARUBA?

METHODS

Two hundred eighty-eight patients with cerebral AVMs underwent microsurgical resection between 1983 and 2012 performed by the same surgeon (J.S.). This is a prospective case collection study that represents a consecutive series. The results are based on prospectively collected, early-outcome data that were supplemented by retrospectively collected, follow-up data for 94% of those cases. The analyzed data included the initial presentation, Spetzler-Martin grade, obliteration rates, surgical and neurological complications, and frequency of pretreatment with embolization or radiosurgery. The total cohort was compared using “small-AVM,” Spetzler-Martin Grade I and II, and ARUBA-eligible AVM subgroups.

RESULTS

The initial presentation was hemorrhage in 50.0% and seizures in 43.1% of patients. The series included 53 Spetzler-Martin Grade I (18.4%), 114 Spetzler-Martin Grade II (39.6%), 90 Spetzler-Martin Grade III (31.3%), 28 Spetzler-Martin Grade IV (9.7%), and 3 Spetzler-Martin Grade V (1.0%) AVMs. There were 144 unruptured and 104 ARUBA-eligible cases. Preembolization was used in 39 cases (13.5%). The occlusion rates for the total series and small AVM subgroup were 99% and 98.7%, respectively. The mean follow-up duration was 64 months. Early neurological deterioration was seen in 39.2% of patients, of which 12.2% had permanent and 5.6% had permanent significant deficits, and the mortality rate was 1.7% (n = 5). Outcome was better for patients with AVMs smaller than 3 cm (permanent deficit in 7.8% and permanent significant deficit in 3.2% of patients) and Ponce Class A status (permanent deficit in 7.8% and significant deficit in 3.2% of patients). Unruptured AVMs showed slightly higher new deficit rates (but 0 instances of mortality) among all cases, and in the small AVM and Ponce Class A subgroups. Unruptured Spetzler-Martin Grade I and II lesions had the best outcome (1.8% permanent significant deficit), and ARUBA-eligible Spetzler-Martin Grade I and II lesions had a slightly higher rate of permanent significant deficits (3.2%).

CONCLUSIONS

Microsurgery has a very high cure rate. Focusing microsurgical AVM resection on unruptured lesions smaller than 3 cm or on Spetzler-Martin Grade I and II lesions is a good strategy for minimizing long-term morbidity. Well-selected microsurgical cases lead to better outcomes than with multimodal interventions, as in the ARUBA treatment arm, or conservative treatment alone. Long-term prospective data collection is valuable.

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Daniel Delev, Anna Pavlova, Alexander Grote, Azize Boström, Anke Höllig, Johannes Schramm, Rolf Fimmers, Johannes Oldenburg and Matthias Simon

OBJECTIVE

Arteriovenous malformations (AVMs) of the brain are a frequent and important cause of intracranial hemorrhage in young adults. Little is known about the molecular-genetic pathomechanisms underlying AVM development. Genes of the NOTCH family control the normal development of vessels and proper arteriovenous specification. Transgenic mice with constitutive expression of active NOTCH4 frequently develop AVMs. Here, the authors report a genetic association study investigating possible associations between NOTCH4 gene polymorphisms and formation and clinical presentation of AVMs.

METHODS

After PCR amplification and direct DNA sequencing or restriction digests, 10 single-nucleotide polymorphisms (SNPs) of the NOTCH4 gene were used for genotyping 153 AVM patients and 192 healthy controls (i.e., blood donors). Pertinent clinical data were available for 129 patients. Uni- and multivariate single-marker and explorative haplotype analyses were performed to identify potential genetic risk factors for AVM development and for hemorrhagic or epileptic presentation.

RESULTS

Eleven calculated haplotypes consisting of 3–4 SNPs (most of which were located in the epidermal growth factor–like domain of the NOTCH4 gene) were observed significantly more often among AVM patients than among controls. Univariate analysis indicated that rs443198_TT and rs915895_AA genotypes both were significantly associated with hemorrhage and that an rs1109771_GG genotype was associated with epilepsy. The association between rs443198_TT and AVM bleeding remained significant in the multivariate regression analysis.

CONCLUSIONS

The authors' results suggest NOTCH4 SNPs as possible genetic risk factors for the development and clinical presentation of AVMs and a role of NOTCH4 in the pathogenesis of this disease.