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Epilepsia Open Dec 2022This study aimed to evaluate the surgical outcomes and relevant prognostic factors in patients with low-grade epilepsy-associated neuroepithelial tumors (LEAT) and,...
OBJECTIVE
This study aimed to evaluate the surgical outcomes and relevant prognostic factors in patients with low-grade epilepsy-associated neuroepithelial tumors (LEAT) and, especially, to develop a scoring system to predict postoperative seizure outcomes.
METHODS
The clinical data of patients who underwent epilepsy surgery for LEAT were retrospectively studied. The surgical outcomes of seizure and neurological statuses in patients were evaluated using Engel classification and modified Rankin Scale (mRS) scoring, respectively. A scoring system of seizure outcomes was constructed based on the weight of the β-coefficient estimate of each predictor in the final multivariate predicting model of seizure outcomes.
RESULTS
Of the 287 patients (106 female) enrolled, the median age was 19 years at surgery and 10 years at seizure onset, with a median duration of epilepsy of 60 months. Among 258 patients who were followed up for at least 12 months, 215 (83.3%) patients had a favorable seizure outcome (Engel class I) after surgery, and 43 (16.7%) patients had an unfavorable seizure outcome; longer duration of epilepsy, discordant magnetoencephalography (MEG) findings, and acute postoperative seizures were significantly included in the scoring system to predict unfavorable seizure outcomes, and in the scoring system, accumulated scoring of 0-19 scores was recorded, which were finally grouped into three risk levels: low risk (risk < 30%), medium risk (30% ≤ risk < 70%), and high risk (risk ≥ 70%). In addition, favorable neurological outcomes (mRS score 0-1) were recorded in 187 (72.5%) patients, while unfavorable neurological outcomes were recorded in 71 (27.5%) patients, which were significantly related to poor seizure control, older age at surgery, and longer duration of epilepsy and hospitalization time.
SIGNIFICANCE
The long-term surgical outcomes of LEAT after surgery were satisfactory. A scoring system for predicting unfavorable seizure outcomes with different risk levels was developed, which could partly guide clinical treatments of LEAT.
Topics: Humans; Female; Young Adult; Adult; Retrospective Studies; Epilepsy; Seizures; Neoplasms, Neuroepithelial; Treatment Outcome
PubMed: 36081402
DOI: 10.1002/epi4.12648 -
Acta Neuropathologica Communications Feb 2020The discovery of fibroblast growth factor receptor (FGFR) gene family alterations as drivers of primary brain tumors has generated significant excitement, both as... (Review)
Review
The discovery of fibroblast growth factor receptor (FGFR) gene family alterations as drivers of primary brain tumors has generated significant excitement, both as potential therapeutic targets as well as defining hallmarks of histologic entities. However, FGFR alterations among neuroepithelial lesions are not restricted to high or low grade, nor to adult vs. pediatric-type tumors. While it may be tempting to consider FGFR-altered tumors as a unified group, this underlying heterogeneity poses diagnostic and interpretive challenges. Therefore, understanding the underlying biology of tumors harboring specific FGFR alterations is critical. In this review, recent evidence for recurrent FGFR alterations in histologically and biologically low-grade neuroepithelial tumors (LGNTs) is examined (namely FGFR1 tyrosine kinase domain duplication in low grade glioma, FGFR1-TACC1 fusions in extraventricular neurocytoma [EVN], and FGFR2-CTNNA3 fusions in polymorphous low-grade neuroepithelial tumor of the young [PLNTY]). Additionally, FGFR alterations with less well-defined prognostic implications are considered (FGFR3-TACC3 fusions, FGFR1 hotspot mutations). Finally, a framework for practical interpretation of FGFR alterations in low grade glial/glioneuronal tumors is proposed.
Topics: Animals; Brain Neoplasms; Fetal Proteins; Humans; Microtubule-Associated Proteins; Mutation; Neoplasms, Neuroepithelial; Nuclear Proteins; Receptor, Fibroblast Growth Factor, Type 1; Receptor, Fibroblast Growth Factor, Type 2; Receptor, Fibroblast Growth Factor, Type 3; alpha Catenin
PubMed: 32085805
DOI: 10.1186/s40478-020-00898-6 -
Cancer Nov 2002Gliomatosis cerebri (GC) is a rare primary brain tumor characterized by proliferation of neoplastic glial cells that typically involve multiple brain areas, with... (Review)
Review
BACKGROUND
Gliomatosis cerebri (GC) is a rare primary brain tumor characterized by proliferation of neoplastic glial cells that typically involve multiple brain areas, with preservation of brain structures and sparing of neurons. The optimal therapeutic strategy is not well established. The impact of radiotherapy on survival in patients with GC remains undefined.
METHODS
Between 1980 and 2001, 12 patients with GC were identified, representing less than 1% of all patients with primary brain neoplasms treated at the Cleveland Clinic.
RESULTS
All 12 patients had brain biopsies between March 1986 and July 2001 (seven males, five females, with a median age of 53 years [range, 13-85 years]). Median Karnofsky performance status at the time of presentation was 70 (range, 40-90). Eight patients had low-grade gliomas and four patients had anaplastic astrocytomas. Eight patients received radiation treatment to the brain as the only treatment, and four received neither radiation nor chemotherapy. The median dose of megavoltage radiation was 55.4 Gy (range, 45-61.2 Gy). Of the eight patients who received brain radiotherapy, the clinical and radiologic follow-up findings improved in three patients, stabilized in three patients, and worsened in two patients. Median follow-up was 10.3 months (range, 1-55 months). The median survival for the eight patients who received brain radiotherapy was 11.4 months. The one- and two-year survival rates were 45% and 30%, respectively. Two of the eight patients who received radiotherapy were alive at the time of writing. The four patients who did not receive radiotherapy died of the disease at 0.6, 1.0, 1.9, and 2.4 months after diagnosis.
CONCLUSIONS
Gliomatosis cerebri is associated with poor survival. Although brain radiotherapy controlled or stabilized disease progression in most patients, the overall survival after brain radiotherapy alone was not satisfactory. More aggressive therapy may be needed.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Astrocytoma; Brain Neoplasms; Female; Glioma; Humans; Male; Middle Aged; Neoplasms, Neuroepithelial; Radiotherapy Dosage; Radiotherapy, High-Energy; Retrospective Studies; Survival Rate
PubMed: 12404298
DOI: 10.1002/cncr.10909 -
Journal of Medical Case Reports Aug 2023Dysembryoplastic neuroepithelial tumors are rare benign supratentotrial epilepsy-associated glioneuronal tumors of children and young adults. Patients have a long... (Review)
Review
BACKGROUND
Dysembryoplastic neuroepithelial tumors are rare benign supratentotrial epilepsy-associated glioneuronal tumors of children and young adults. Patients have a long history of seizures. Proper surgical resection achieves long term seizure control. We describe the clinicopathological features of dysembryoplastic neuroepithelial tumor cases reported in our practice and review the published literature.
METHODS
All cases of Pakistani ethnicity were diagnosed between 2015 and 2021 were included. Slides were reviewed and clinicopathological features were recorded. Follow-up was obtained. Extensive literature review was conducted.
RESULTS
Fourteen cases were reported. There were 12 males and 2 females. Age range was 9-45 years (mean 19 years). Majority were located in the temporal and frontal lobes. Duration of seizures prior to resection ranged from 2 months to 9 years with mean and median duration of 3.2 and 3 years, respectively. Histologically, all cases demonstrated a multinodular pattern, specific glioneuronal component, and floating neurons. Simple and complex forms comprised seven cases each. No significant nuclear atypia, mitotic activity, or necrosis was seen. Ki-67 proliferative index was very low. Cortical dysplasia was noted in adjacent glial tissue in four cases. Follow-up ranged from 20 to 94 months. Seizures continued following resection in all but one case but were reduced in frequency and intensity. In one case, seizures stopped completely following surgery.
CONCLUSION
Clinicopathological features were similar to those in published literature. However, a marked male predominance was noted in our series. Seizures continued following resection in all but one case but were reduced in frequency and intensity. This series will help raise awareness among clinicians and pathologists in our part of the world about this seizure-associated tumor of children and young adults.
Topics: Adolescent; Adult; Child; Female; Humans; Male; Middle Aged; Young Adult; Brain Neoplasms; Epilepsy; Glioma; Neoplasms, Neuroepithelial; Seizures
PubMed: 37525202
DOI: 10.1186/s13256-023-04062-1 -
Brain Pathology (Zurich, Switzerland) Jul 2000Astroblastomas are uncommon brain tumors whose classification and histogenesis have been debated. Precise criteria for diagnosis have been described only recently, but...
Astroblastomas are uncommon brain tumors whose classification and histogenesis have been debated. Precise criteria for diagnosis have been described only recently, but have not found wide acceptance. We report the clinical, radiographic, and histopathologic features of 20 astroblastomas, and the chromosomal alterations in seven cases as detected by comparative genomic hybridization (CGH). The tumors occurred both in children and young adults (average age, 14 years), most often as well circumscribed, peripheral, cerebral hemispheric masses. Radiographically, the lesions were contrast-enhancing and solid, often with a cystic component. All were characterized histologically by astroblastic pseudorosettes, and most displayed prominent perivascular hyalinization, regional hyaline changes, and pushing borders in regard to adjacent brain. Tumor cells were strongly immunoreactive for S-100 protein, GFAP, and vimentin. Staining for EMA was focal. Ten of 20 astroblastomas were classified as "well differentiated" and 10 were classified as "malignant," largely on the basis of hypercellular zones with increased mitotic indices, vascular proliferation, and necrosis with pseudopalisading. All 10 well differentiated lesions and 8 of 10 malignant lesions were completely resected. None of the well differentiated astroblastomas recurred within the limited follow-up period. Three malignant astroblastomas recurred, including two incompletely resected tumors, and one that had been totally resected. One patient died of disease following recurrence. The most frequent chromosomal alterations detected by CGH were gains of chromosome arm 20q (4/7 tumors) and chromosome 19 (3/7). The combination of these gains occurred in three, including two well differentiated and one malignant astroblastoma. Other alterations noted in two tumors each were losses on 9q, 10, and X. These chromosomal alterations are not typical of ependymoma or infiltrating astrocytic neoplasms, and suggest that astroblastomas may have a characteristic cytogenetic profile in addition to their distinctive clinical, radiographic, and histopathologic features.
Topics: Adolescent; Adult; Brain Neoplasms; Child; Chromosome Aberrations; Chromosome Disorders; Combined Modality Therapy; Female; Follow-Up Studies; Humans; Male; Middle Aged; Neoplasms, Neuroepithelial; Nucleic Acid Hybridization; Radiography
PubMed: 10885653
DOI: 10.1111/j.1750-3639.2000.tb00266.x -
AJNR. American Journal of Neuroradiology May 2003
Topics: Astrocytoma; Brain Chemistry; Brain Neoplasms; Choline; Creatine; Diagnosis, Differential; Humans; Inositol; Magnetic Resonance Spectroscopy; Neoplasms, Neuroepithelial
PubMed: 12748070
DOI: No ID Found -
Epilepsia Jan 2012Gangliogliomas (GGs) and dysembryoplastic neuroepithelial tumors (DNETs) are low-grade brain tumors of glioneuronal origin that commonly present with seizures. Achieving... (Review)
Review
PURPOSE
Gangliogliomas (GGs) and dysembryoplastic neuroepithelial tumors (DNETs) are low-grade brain tumors of glioneuronal origin that commonly present with seizures. Achieving seizure control in patients with glioneuronal tumors remains underappreciated, as tumor-related epilepsy significantly affects patients' quality-of-life.
METHODS
We performed a quantitative and comprehensive systematic literature review of seizure outcomes after surgical resection of GGs and DNETs associated with seizures. We evaluated 910 patients from 39 studies, and stratified outcomes according to several potential prognostic variables.
KEY FINDINGS
Overall, 80% of patients were seizure-free after surgery (Engel class I), whereas 20% continued to have seizures (Engel class II-IV). We observed significantly higher rates of seizure-freedom in patients with ≤1 year duration of epilepsy compared to those with >1 year of seizures [odds ratio (OR) 9.48; 95% confidence interval (CI) 2.26-39.66], and with gross-total resection over subtotal lesionectomy (OR 5.34; 95% CI 3.61-7.89). In addition, the presence of secondarily generalized seizures preoperatively predicted a lower rate of seizure-freedom after surgery (OR 0.40; 95% CI 0.24-0.66). Outcomes did not differ significantly between adults and children, patients with temporal lobe versus extratemporal tumors, pathologic diagnosis of GG versus DNET, medically controlled versus refractory seizures, or with the use of electrocorticography (ECoG). Extended resection of temporal lobe tumors, with hippocampectomy and/or corticectomy, conferred additional benefit.
SIGNIFICANCE
These results suggest that early operative intervention and gross-total resection are critically important factors in achieving seizure-freedom, and thus improving quality-of-life, in patients with glioneuronal tumors causing epilepsy.
Topics: Brain Neoplasms; Electroencephalography; Ganglioglioma; Humans; Neoplasms, Neuroepithelial; Seizures; Treatment Outcome
PubMed: 21933181
DOI: 10.1111/j.1528-1167.2011.03269.x -
AJNR. American Journal of Neuroradiology Sep 1997
Topics: Brain Neoplasms; Cerebral Cortex; Diagnosis, Differential; Diagnostic Imaging; Hippocampus; Humans; Neuroectodermal Tumors, Primitive, Peripheral; Temporal Lobe
PubMed: 9296207
DOI: No ID Found -
Journal of Korean Medical Science Oct 2004Astroblastoma is one of the very unusual type of tumors, whose histogenesis has not been clarified. It occurs mainly among children or young adults. Astroblastoma is...
Astroblastoma is one of the very unusual type of tumors, whose histogenesis has not been clarified. It occurs mainly among children or young adults. Astroblastoma is grossly well-demarcated, and shows histologically characteristic perivascular pseudorosettes with frequent vascular hyalinization. Perivascular pseudorosettes in astroblastoma have short and thick cytoplasmic processes and blunt-ended foot plates. A 15-yr-old girl presented with headache and diplopia for one and a half year. A well-demarcated mass, 9.7 cm in diameter, was found in the right frontal lobe in brain MRI, and it was a well-enhanced inhomogenous mass. Cystic changes of various sizes were observed inside the tumor mass as well as in the posterior part of the mass, but no peritumoral edema was found. Histologically, this mass belongs to a typical astroblastoma, and no sign of anaplastic astrocytoma, gemistocytic astrocytoma or glioblastoma was found in any part of the tumor. Immunohistochemically, the tumor cells showed diffuse strong positivity for glial fibrillary acidic protein, S-100 protein, vimentin and neuron specific enolase, and focal positivity for epithelial membrane antigen and CAM 5.2, while showing negativity for synaptophysin, neurofilament protein, pan-cytokeratin and high molecular weight keratin.
Topics: Adolescent; Biomarkers; Brain Neoplasms; Diagnosis, Differential; Female; Glial Fibrillary Acidic Protein; Humans; Keratins; Magnetic Resonance Imaging; Neoplasms, Neuroepithelial; Phosphopyruvate Hydratase; S100 Proteins; Vimentin
PubMed: 15483362
DOI: 10.3346/jkms.2004.19.5.772 -
The American Surgeon Sep 2023Neuroblastomas are the most common extracranial solid malignancy in children with variable manifestations and complications depending on the presence of paraneoplastic...
BACKGROUND
Neuroblastomas are the most common extracranial solid malignancy in children with variable manifestations and complications depending on the presence of paraneoplastic syndromes.
MATERIALS AND METHODS
We performed a single institution retrospective cohort study of all patients less than 18 years old diagnosed with neuroblastoma or ganglioneuroblastoma between January 2002 and July 2022. Patients were identified through the pathology and cancer registry and cross-referenced with pediatric records. Patient demographics, clinical presentation, treatment, and outcomes were collected. A univariate descriptive analysis of the collected data was conducted.
RESULTS
In our study period, 130 children were diagnosed with neuroblastoma, and 15 were diagnosed with ganglioneuroblastoma. There were 12 children with a paraneoplastic syndrome identified, 8 with NBL and 4 with ganglioneuroblastoma (GNBL). The average age at diagnosis was 22 months. All but 1 underwent resection prior to treatment of paraneoplastic syndrome, and 4 children required neoadjuvant therapy. Neurological complications were the most common with 10 children (83%). The average time from symptom onset to diagnosis was 0.7 months. Eight children had complete resolution of their symptoms after treatment and resection, 2 children recently started treatment within a year, 1 had partial resolution, and 1 died during treatment. The presence of tumor-infiltrating lymphocytes occurred in 4 children with neurologic paraneoplastic syndromes. Six children had neuropil rich tumors.
CONCLUSION
The histological profile of paraneoplastic syndromes of neuroblastoma and ganglioneuroblastoma and their treatment across a single institution can be highly variable. The presence of tumor-infiltrating lymphocytes and neuropil may have an impact on paraneoplastic pathology.
Topics: Humans; Child; Infant; Adolescent; Ganglioneuroblastoma; Retrospective Studies; Neuroblastoma; Paraneoplastic Syndromes; Nervous System Diseases
PubMed: 37150742
DOI: 10.1177/00031348231175112