Gangliogliomas are rare glioneuronal tumors that typically cause refractory seizures during the first 3 decades of life.
To determine the prognosticators of seizure outcome after surgery for ganglioglioma.
We reviewed the cases of 66 patients who underwent resection of gangliogliomas at the University of California, San Francisco. Demographic, seizure history, and operative data were examined for statistical association with postoperative seizure outcomes.
Of the 66 patients who underwent surgical resection of ganglioglioma, 49 patients (74%) presented with a history of seizures. Of those 49 patients, 50% presented with intractable epilepsy. Temporal lobe gangliogliomas were present in 76% of the patients who presented with a history of seizures. Electrocorticography was performed on 35% of the patients, and of those patients, 82% underwent extended lesionectomy to remove abnormally epileptogenic extralesional tissue. The median follow-up duration was 6.9 years, during which tumor progression occurred in 38% of patients who underwent subtotal resection and in 8% of patients who underwent gross total resection (P = .02). Overall, 85% of patients were seizure free (International League Against Epilepsy class I or II) 5 years after surgery. Subtotal resection was associated with poor seizure outcomes 1 year after resection (odds ratio = 14.6; 95% confidence interval = 2.4-87.7): rates of seizure freedom were 54% after subtotal resection, 96% after gross total resection, and 93% after gross total resection with intraoperative electrocorticography-guided extended lesionectomy.
We report excellent long-term seizure control outcomes after surgery for gangliogliomas. Intraoperative electrocorticography may be a useful adjunct for guiding extended resection in certain pharmacoresistant epilepsy patients with gangliogliomas. Subtotal resection is associated with higher rates of tumor progression and nonoptimal seizure outcomes.
[Show abstract][Hide abstract] ABSTRACT: Purpose
To investigate the value of intraoperative MR imaging (iopMRI) combined with neuronavigation to avoid intraoperative underestimation of the resection amount during surgery of lesional temporal lobe epilepsy (LTLE) patients.
We retrospectively investigated 88 patients (40 female, 48 male, mean age 37.2 yrs., from 12 to 69 yrs., 41 left sided lesions) with LTLE operated at our department, including 40.9% gangliogliomas (GG), 26.1% cavernomas (CM), 10.2% dysembryoplastic neuroepithelial tumours (DNT) and 11.4% focal cortical dysplasias (FCD), excluding hippocampal sclerosis.
Complete resection was achieved in 85 of 88 patients (96.6%), as proven by postoperative MRI 6 months after surgery. In contrast, the routine first iopMR imaging before closure revealed radical resection in only 66 of these 88 patients (75%). After re-intervention, the second iopMR imaging demonstrated complete resection in 19 more patients. Thus, as a direct effect of iopMRI and neuronavigation, overall resection rate was increased by 21.6%. An excellent seizure outcome Engel Class I was found in 76.1% of patients during a mean follow-up of 26.4 months, irrespective of histological entity (74% in CM, 75% in GG, 78% in DNT and 60% in FCD). No severe postoperative complications occurred; permanent superior visual field defects were detected in 10.2% and permanent dysphasia/dyscalculia in 1.1%.
Refined surgery using neuronavigation combined with iopMR imaging in LTLE surgery led to radical resection in 96.6% of the patients, due to immediate correction of underestimated resection in 21.6% of patients. This protocol resulted in a favourable seizure outcome and a low complication rate.
[Show abstract][Hide abstract] ABSTRACT: Reports on seizure outcomes following surgery for lesional epilepsy consistently cite extent of resection as a significant predictor of outcome. Unfortunately, gross-total resection is not technically feasible in all cases of medically refractory tumor-associated epilepsy. Here, the authors present the case of a 4-year-old girl whose epilepsy was medically controlled after 1-stage electrocorticography-guided subtotal resection (STR) of a large diffuse protoplasmic astrocytoma. They also review the modern literature on epilepsy associated with brain tumors. Outcomes are compared with those following surgical treatment of focal cortical dysplasia and vascular lesions. Gross-total lesional resection shows significant superiority across pathologies and anatomical regions. Despite a considerable number of STRs yielding seizure freedom, other favorable treatment factors have not been defined. Although gross-total lesional resection, if possible, is clearly superior, tailored surgery may still offer patients a significant opportunity for a good outcome. Treatment factors yielding successful seizure control following STR remain to be fully elucidated.
[Show abstract][Hide abstract] ABSTRACT: Focal malformations of cortical development (FMCD) are highly associated with several neurological disorders including intractable epilepsy and neurocognitive disabilities. Over the past decade, several FMCD subtypes have been linked to hyperactivation of the mammalian target of rapamycin (mTOR) signaling cascade. In view of the roles that mTOR plays in cell proliferation, size, motility, and stem cell phenotype, many of the features of FMCD such as cytomegaly, disorganized lamination, and expression of stem cell markers can be explained by enhanced mTOR signaling. FMCD result from several distinct and fascinating molecular mechanisms including biallelic gene inactivation, somatic mutation, and potentially, viral infection. These mechanisms have been directly linked to mTOR activation. Perhaps most compelling, pharmacological inhibition of mTOR has been implemented successfully in clinical trials for select FMCD and provides a new vista for treatment.
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