Cortical ependymoma: an unusual epileptogenic lesion

Object Supratentorial cortical ependymomas (CE) are rare, with 7 cases reported. The lesions, typically occurring in the superficial cortex in young adults and associated with a history of seizures, are not fully characterized. Furthermore, their relationship with the recently described angiocentric glioma (AG) is still being debated. This study was undertaken to summarize the authors' experience with CEs. Methods Between 1997 and 2009, 202 cases of ependymoma were surgically treated at the Mayo Clinic, 49 of which were supratentorial. Among these, 9 CE cases were retrospectively identified. Clinical, imaging, and pathological features of each case were reviewed. Results Tumors arose from the frontal (5 cases), parietal (3), and occipital (1) lobes. No tumor occurred in the temporal lobe, despite its reported association with seizures. The mean age at presentation was 27 ± 19 years (± SD) and age at resection was 36 ± 16 years. The mean size of the lesion was 16 ± 14 cm3. Seizures were the presenting symptom in 78%. Cross-sectional imaging in 8 cases was characterized by a heterogeneous mass with multiple cystlike areas and enhancement of the soft-tissue component. Gross-total resection was achieved in 8 of 9 tumors. Pathologically, 6 were low-grade (WHO Grade II) and 3 were anaplastic (WHO Grade III) ependymomas. All tumors exhibited the focal presence of perivascular pseudorosettes, but only 1 (11%) exhibited the focal presence of a true rosette. A bipolar spindle cell component resembling AG was present in 3 (33%) and “Schwannian-like” nodules in 2 (22%). Subpial aggregation and peripheral infiltration were present in 4 cases (44%). With a mean postsurgery follow-up of 62 ± 38 months, only 2 lesions recurred locally after imaging-confirmed gross-total resection, both being Grade III. In 5 (71%) of 7 patients presenting with seizures an Engel Class I outcome was achieved. Conclusions Cortical ependymomas represent a rare type of ependymoma occurring superficially in the cortex. Morphologically, these tumors are protean, varying from classic to epithelioid, clear cell, and tanycytic. Some also exhibited features typical of AG. Most tumors were low grade and cured with resection. Anaplastic tumors occur and may recur locally despite provision of radiation therapy. Cortical ependymomas frequently, but not always, present with seizures, but despite their high association with epilepsy, none occurred in the temporal lobe in any of the authors' 9 patients. Overall, CEs appear to have a relatively favorable prognosis compared with other supratentorial ependymomas.

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Gross-total resection of temporal low grade gliomas is a critically important factor in achieving seizure-freedom

Arquivos de Neuro-Psiquiatria ◽

10.1590/0004-282x20150141 ◽

2015 ◽

Vol 73 (11) ◽

pp. 924-928 ◽

Cited By ~ 10

Author(s):

Lucas Crociati Meguins ◽

Rodrigo Antônio Rocha da Cruz Adry ◽

Sebastião Carlos da Silva Júnior ◽

Carlos Umberto Pereira ◽

Jean Gonçalves de Oliveira ◽

...

Keyword(s):

Intractable Epilepsy ◽

Gross Total Resection ◽

Seizure Outcome ◽

Seizure Freedom ◽

Low Grade ◽

Total Resection ◽

Low Grade Gliomas ◽

Significant Difference ◽

The Mean ◽

One Year

Objective To present a surgical series of patients with low grade temporal gliomas causing intractable epilepsy, focusing on long-term seizure outcome.Method A retrospective study was conducted with patients with temporal low-grade gliomas (LGG).Results Sixty five patients with were operated in our institution. Males were more affected than females and the mean age at surgery was 32.3 ± 8.4 (9-68 years). The mean age at seizure onset was 25.7 ± 9.2 (11-66 years). Seizure outcome was classified according with Engel classification. After one year of follow up, forty two patients (64.6%) were Engel I; seventeen (26.2%) Engel II; four (6.2%) Engel III and two (3.1%) Engel IV. Statistically significant difference in seizure outcome was obtained when comparing the extension of resection. Engel I was observed in 39 patients (69.6%) with total resection and in only 3 (33.3%) patients with partial resection.Conclusion Gross-total resection of temporal LGGs is a critically important factor in achieving seizure-freedom.

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Independent association of extent of resection with survival in patients with malignant brain astrocytoma

Journal of Neurosurgery ◽

10.3171/2008.4.17536 ◽

2009 ◽

Vol 110 (1) ◽

pp. 156-162 ◽

Cited By ~ 444

Author(s):

Matthew J. McGirt ◽

Kaisorn L. Chaichana ◽

Muraya Gathinji ◽

Frank J. Attenello ◽

Khoi Than ◽

...

Keyword(s):

Median Survival ◽

Extent Of Resection ◽

Primary Resection ◽

Gross Total Resection ◽

Total Resection ◽

Who Grade ◽

Factors Associated ◽

Independent Association ◽

Who Grade Iii ◽

Grade Iii

Object With recent advances in the adjuvant treatment of malignant brain astrocytomas, it is increasingly debated whether extent of resection affects survival. In this study, the authors investigate this issue after primary and revision resection of these lesions. Methods The authors retrospectively reviewed the cases of 1215 patients who underwent surgery for malignant brain astrocytomas (World Health Organization [WHO] Grade III or IV) at a single institution from 1996 to 2006. Patients with deep-seated or unresectable lesions were excluded. Based on MR imaging results obtained < 48 hours after surgery, gross-total resection (GTR) was defined as no residual enhancement, near-total resection (NTR) as having thin rim enhancement of the resection cavity only, and subtotal resection (STR) as having residual nodular enhancement. The independent association of extent of resection and subsequent survival was assessed via a multivariate proportional hazards regression analysis. Results Magnetic resonance imaging studies were available for review in 949 cases. The mean age and mean Karnofsky Performance Scale (KPS) score at time of surgery were 51 ± 16 years and 80 ± 10, respectively. Surgery consisted of primary resection in 549 patients (58%) and revision resection for tumor recurrence in 400 patients (42%). The lesion was WHO Grade IV in 700 patients (74%) and Grade III in 249 (26%); there were 167 astrocytomas and 82 mixed oligoastrocytoma. Among patients who underwent resection, GTR, NTR, and STR were achieved in 330 (35%), 388 (41%), and 231 cases (24%), respectively. Adjusting for factors associated with survival (for example, age, KPS score, Gliadel and/or temozolomide use, and subsequent resection), GTR versus NTR (p < 0.05) and NTR versus STR (p < 0.05) were independently associated with improved survival after both primary and revision resection of glioblastoma multiforme (GBM). For primary GBM resection, the median survival after GTR, NTR, and STR was 13, 11, and 8 months, respectively. After revision resection, the median survival after GTR, NTR, and STR was 11, 9, and 5 months, respectively. Adjusting for factors associated with survival for WHO Grade III astrocytoma (age, KPS score, and revision resection), GTR versus STR (p < 0.05) was associated with improved survival. Gross-total resection versus NTR was not associated with an independent survival benefit in patients with WHO Grade III astrocytomas. The median survival after primary resection of WHO Grade III (mixed oligoastrocytomas excluded) for GTR, NTR, and STR was 58, 46, and 34 months, respectively. Conclusions In the authors' experience with both primary and secondary resection of malignant brain astrocytomas, increasing extent of resection was associated with improved survival independent of age, degree of disability, WHO grade, or subsequent treatment modalities used. The maximum extent of resection should be safely attempted while minimizing the risk of surgically induced neurological injury.

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18F-FET-PET-guided gross total resection improves overall survival in patients with WHO grade III/IV glioma: moving towards a multimodal imaging-guided resection

Journal of Neuro-Oncology ◽

10.1007/s11060-021-03844-1 ◽

2021 ◽

Author(s):

Jonas Ort ◽

Hussam Aldin Hamou ◽

Julius M. Kernbach ◽

Karlijn Hakvoort ◽

Christian Blume ◽

...

Keyword(s):

Multimodal Imaging ◽

Tumor Volume ◽

Oncological Outcome ◽

Gross Total Resection ◽

Treatment Modalities ◽

Total Resection ◽

Who Grade ◽

Fet Pet ◽

Who Grade Iii ◽

Grade Iii

Abstract Purpose PET using radiolabeled amino acid [18F]-fluoro-ethyl-L-tyrosine (FET-PET) is a well-established imaging modality for glioma diagnostics. The biological tumor volume (BTV) as depicted by FET-PET often differs in volume and location from tumor volume of contrast enhancement (CE) in MRI. Our aim was to investigate whether a gross total resection of BTVs defined as < 1 cm3 of residual BTV (PET GTR) correlates with better oncological outcome. Methods We retrospectively analyzed imaging and survival data from patients with primary and recurrent WHO grade III or IV gliomas who underwent FET-PET before surgical resection. Tumor overlap between FET-PET and CE was evaluated. Completeness of FET-PET resection (PET GTR) was calculated after superimposition and semi-automated segmentation of pre-operative FET-PET and postoperative MRI imaging. Survival analysis was performed using the Kaplan–Meier method and the log-rank test. Results From 30 included patients, PET GTR was achieved in 20 patients. Patients with PET GTR showed improved median OS with 19.3 compared to 13.7 months for patients with residual FET uptake (p = 0.007; HR 0.3; 95% CI 0.12–0.76). This finding remained as independent prognostic factor after performing multivariate analysis (HR 0.19, 95% CI 0.06–0.62, p = 0.006). Other survival influencing factors such as age, IDH-mutation, MGMT promotor status, and adjuvant treatment modalities were equally distributed between both groups. Conclusion Our results suggest that PET GTR improves the OS in patients with WHO grade III or IV gliomas. A multimodal imaging approach including FET-PET for surgical planning in newly diagnosed and recurrent tumors may improve the oncological outcome in glioma patients.

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Outcome and survival following primary and repeat surgery for World Health Organization Grade III meningiomas

Journal of Neurosurgery ◽

10.3171/2010.1.jns091114 ◽

2010 ◽

Vol 113 (2) ◽

pp. 202-209 ◽

Cited By ~ 76

Author(s):

Michael E. Sughrue ◽

Nader Sanai ◽

Gopal Shangari ◽

Andrew T. Parsa ◽

Mitchel S. Berger ◽

...

Keyword(s):

Overall Survival ◽

Clinical Data ◽

World Health ◽

Gross Total Resection ◽

Total Resection ◽

Repeat Surgery ◽

Who Grade ◽

Kaplan Meier ◽

Who Grade Iii ◽

Grade Iii

Object Despite an increased understanding of the biology of malignant meningioma tumor progression, there is a paucity of published clinical data on factors affecting outcomes following treatment for these lesions. The authors present the largest case series to date dealing with these tumors, providing analysis of 63 patients. Methods The authors identified all patients undergoing resection of WHO Grade III tumors at their institution over a 16-year period. They analyzed clinical data from these patients, and performed Kaplan-Meier and Cox regression analyses to determine the impact of different clinical characteristics and different treatment modalities on survival following initial and repeat surgery for these lesions. Results Sixty-three patients met inclusion criteria and were analyzed further. The median clinical follow-up time was 5 years (range 1–22 years). The 2-, 5-, and 10-year overall survival rates following initial operation were 82, 61, and 40%, respectively. Kaplan-Meier analysis demonstrated a marked survival benefit with repeat operation (53 vs 25 months, p = 0.02). Interestingly, patients treated with near-total resection experienced improved overall survival when compared with patients treated with gross-total resection at initial (p = 0.035) and repeat operations (p = 0.005). Twelve (19%) of 63 patients experienced significant neurological morbidity referable to the resection of their tumors. Conclusions Surgery is an effective treatment for WHO Grade III meningiomas at presentation and recurrence; however, aggressive attempts to achieve gross-total resection can be associated with significant neurological risk.

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Impact of gross total resection in patients with WHO grade III glioma harboring the IDH 1/2 mutation without the 1p/19q co-deletion

Journal of Neuro-Oncology ◽

10.1007/s11060-016-2201-2 ◽

2016 ◽

Vol 129 (3) ◽

pp. 505-514 ◽

Cited By ~ 62

Author(s):

Tomohiro Kawaguchi ◽

Yukihiko Sonoda ◽

Ichiyo Shibahara ◽

Ryuta Saito ◽

Masayuki Kanamori ◽

...

Keyword(s):

Gross Total Resection ◽

Total Resection ◽

Who Grade ◽

Who Grade Iii ◽

Grade Iii

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Transcavernous Approach for Gross Total Resection of a Dumbbell-Shaped Giant Trigeminal Schwannoma

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0041-1729995 ◽

2021 ◽

Author(s):

Sima Sayyahmelli ◽

Emel Avci ◽

Burak Ozaydin ◽

Mustafa K. Başkaya

Keyword(s):

Skull Base ◽

Cavernous Sinus ◽

Tumor Resection ◽

Mass Effect ◽

World Health ◽

Gross Total Resection ◽

Petrous Apex ◽

Total Resection ◽

Who Grade ◽

Trigeminal Schwannoma

AbstractTrigeminal schwannomas are rare nerve sheet tumors that represent the second most common intracranial site of occurrence after vestibular nerve origins. Microsurgical resection of giant dumbbell-shaped trigeminal schwannomas often requires complex skull base approaches. The extradural transcavernous approach is effective for the resection of these giant tumors involving the cavernous sinus.The patient is a 72-year-old man with headache, dizziness, imbalance, and cognitive decline. Neurological examination revealed left-sided sixth nerve palsy, a diminished corneal reflex, and wasting of temporalis muscle. Magnetic resonance imaging (MRI) showed a giant homogeneously enhancing dumbbell-shaped extra-axial mass centered within the left cavernous sinus, Meckel's cave, and the petrous apex, with extension to the cerebellopontine angle. There was a significant mass effect on the brain stem causing hydrocephalus. Computed tomography (CT) scan showed erosion of the petrous apex resulting in partial anterior autopetrosectomy (Figs. 1 and 2).The decision was made to proceed with tumor resection using a transcavernous approach. Gross total resection was achieved. The surgery and postoperative course were uneventful, and the patient woke up the same as in the preoperative period. MRI confirmed gross total resection of the tumor. The histopathology was a trigeminal schwannoma, World Health Organization (WHO) grade I. The patient continues to do well without any recurrence at 15-month follow-up.This video demonstrates important steps of the microsurgical skull base techniques for resection of these challenging tumors.The link to the video can be found at https://youtu.be/TMK5363836M

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Profound hearing loss following surgery in pediatric patients with posterior fossa low-grade glioma

Neuro-Oncology Practice ◽

10.1093/nop/npx025 ◽

2017 ◽

Vol 5 (2) ◽

pp. 96-103 ◽

Cited By ~ 1

Author(s):

Yahya Ghazwani ◽

Ibrahim Qaddoumi ◽

Johnnie K Bass ◽

Shengjie Wu ◽

Jason Chiang ◽

...

Keyword(s):

Hearing Loss ◽

Posterior Fossa ◽

Extent Of Resection ◽

Tumor Location ◽

Low Grade Glioma ◽

Gross Total Resection ◽

Subtotal Resection ◽

Low Grade ◽

Total Resection ◽

Profound Hearing Loss

Abstract Background Hearing loss may occur in patients with posterior fossa low-grade glioma who undergo surgery. Methods We retrospectively reviewed 217 patients with posterior fossa low-grade glioma, including 115 for whom results of hearing tests performed after surgery and before chemotherapy or radiation therapy were available. We explored the association of UHL with age at diagnosis, sex, race, tumor location, extent of resection, posterior fossa syndrome, ventriculoperitoneal shunt placement, and histology. Results Of the 115 patients, 15 (13.0%: 11 male, 6 black, 8 white, 1 multiracial; median age 7 years [range, 1.3–17.2 years]) had profound UHL after surgery alone or before receiving ototoxic therapy. Median age at tumor diagnosis was 6.8 years (range, 0.7–14.1 years), and median age at surgery was 6.8 years (range, 0.7–14.1 years). Patients with UHL had pathology characteristic of pilocytic astrocytoma (n = 10), ganglioglioma (n = 4), or low-grade astrocytoma (n = 1). Of these 15 patients, 4 underwent biopsy, 1 underwent gross total resection, 1 underwent near-total resection, and 9 underwent subtotal resection. UHL was more frequent in black patients than in white patients (OR 7.3, P = .007) and less frequent in patients who underwent gross total resection or near-total resection than in those who underwent subtotal resection (OR 0.11, P = .02). Conclusions Children undergoing surgery for posterior fossa low-grade glioma are at risk for UHL, which may be related to race or extent of resection. These patients should receive postoperative audiologic testing, as earlier intervention may improve outcomes.

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Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children

Journal of Neurosurgery Spine ◽

10.3171/2013.6.spine12927 ◽

2013 ◽

Vol 19 (4) ◽

pp. 471-476 ◽

Cited By ~ 46

Author(s):

William B. Feldman ◽

Aaron J. Clark ◽

Michael Safaee ◽

Christopher P. Ames ◽

Andrew T. Parsa

Keyword(s):

Recurrence Rate ◽

Adjuvant Radiotherapy ◽

Pediatric Patients ◽

Cauda Equina ◽

Extent Of Resection ◽

Gross Total Resection ◽

Tumor Control ◽

Recurrence Rates ◽

Total Resection ◽

Who Grade

Object Myxopapillary ependymomas (MPEs) are rare WHO Grade I tumors found in the conus medullaris, cauda equina, and filum terminale. Treatment generally consists of resection with or without adjuvant radiotherapy. Evidence-based guidelines for surgical management are lacking due to the rarity of this tumor. Methods An English-language PubMed search was performed using the key words “myxopapillary” and “ependymoma.” Reports describing fewer than 3 patients or those lacking data on the extent of resection or radiotherapy were excluded. A total of 28 articles describing 475 patients met the authors' inclusion criteria. Patients were grouped by extent of resection and whether or not they underwent adjuvant radiotherapy. Differences in recurrence rates were assessed by chi-square test. Results The overall recurrence rate was 15.5% in patients treated by gross-total resection (GTR) and 32.6% in patients treated by subtotal resection (STR), irrespective of whether they underwent adjuvant therapy (p < 0.001). Regardless of the extent of resection, adjuvant radiotherapy was not associated with a decrease in recurrence rates. The overall recurrence rate was 15.6% in patients who underwent GTR and radiotherapy compared with 15.9% in patients who underwent GTR alone (p = 0.58), and it was 29.3% in patients who underwent STR and radiotherapy compared with 35.1% in those who underwent STR alone (p = 0.53). The difference between recurrence rates for patients who underwent GTR alone versus STR and radiotherapy was statistically significant (p = 0.02). Subgroup analysis demonstrated significantly higher recurrence rates in pediatric patients compared with adults (40.5% vs 23.4%, respectively; p = 0.02). Even in the setting of GTR alone, recurrence rates were higher in pediatric patients (65% vs 7.6%; p < 0.001). Conclusions Gross-total resection alone is associated with decreased recurrence rates compared with STR with or without radiotherapy. The authors' results suggest that treatment goals should include attempted GTR whenever possible. The observation that children benefitted from radiation therapy to a greater extent than did adults suggests that biological differences between tumors in these patient populations warrants more rigorous scientific studies.

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Ependymomas in infancy: underlying genetic alterations, histological features, and clinical outcome

Child s Nervous System ◽

10.1007/s00381-020-04655-x ◽

2020 ◽

Vol 36 (11) ◽

pp. 2693-2700

Author(s):

Stephanie T. Jünger ◽

Felipe Andreiuolo ◽

Martin Mynarek ◽

Evelyn Dörner ◽

Anja zur Mühlen ◽

...

Keyword(s):

Posterior Fossa ◽

Genetic Alterations ◽

Gross Total Resection ◽

Older Children ◽

Total Resection ◽

Who Grade ◽

Chromosome 1Q ◽

Stable Genome ◽

1Q Gain

Abstract Introduction Young age is an adverse prognostic factor in children with ependymomas. Treatment of these infants is challenging since beneficial therapeutic options are limited. As ependymomas are considered a biologically heterogeneous group, we aimed to characterize infant ependymomas with regard to their histological and genetic features. Materials and methods We analyzed 28 ependymomas occurring in children younger than 18 months at diagnosis enrolled into the HIT2000-E protocols with the aim to postpone irradiation until the age of 18 months if possible. All cases underwent neuropathological review, including immunohistochemical characterization. Genome-wide copy number alterations (CNA) were assessed by molecular inversion probe assays, and RELA and YAP1 fusions were detected by RT-PCR and sequencing. Results All infant ependymomas were anaplastic (WHO grade III). Twenty-one (75%) cases were located in the posterior fossa. Gross total resection was accomplished in 12 (57%) of these cases. All posterior fossa tumors showed loss of H3-K27me3 characteristic of PFA ependymomas. CNA analysis showed a stable genome in all cases with lack of chromosome 1q gain, an adverse prognostic marker in PFA ependymomas of older children. However, after a median follow-up of 5.4 years, 15 (71%) relapsed, and 9 (43%) died. Seven ependymomas (25%) occurred in the supratentorial region. Gross total resection could be achieved in only two of these cases. Four tumors carried C11orf95-RELA fusions, and two cases had typical YAP1-MAMLD1 fusions (one case was not analyzable). The RELA-fused cases did not display CDKN2A loss as an adverse indicator of prognosis in this disease entity. Although three infants (43%) with supratentorial ependymomas relapsed, all patients survived (median follow-up, 8.0 years). Conclusion Infant ependymomas seem to fall into three biological entities, with supratentorial tumors carrying RELA or YAP fusions and PFA posterior fossa ependymomas. The latter showed a poor outcome even though chromosome 1q gain was absent.

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Analysis of Prognostic Factors of World Health Organization Grade Ⅲ Meningiomas

Frontiers in Oncology ◽

10.3389/fonc.2020.593073 ◽

2020 ◽

Vol 10 ◽

Author(s):

Weidong Tian ◽

Jingdian Liu ◽

Kai Zhao ◽

Junwen Wang ◽

Wei Jiang ◽

...

Keyword(s):

Multivariate Analysis ◽

Prognostic Factors ◽

Cox Regression ◽

Postoperative Radiotherapy ◽

World Health ◽

Low Grade ◽

Ki 67 ◽

Who Grade ◽

Who Grade Iii ◽

Grade Iii

ObjectiveWHO grade III meningiomas are highly aggressive and lethal. However, there is a paucity of clinical information because of a low incidence rate, and little is known for prognostic factors. The aim of this work is to analyze clinical characteristics and prognosis in patients diagnosed as WHO grade III meningiomas.Methods36 patients with WHO grade III meningiomas were enrolled in this study. Data on gender, age, clinical presentation, preoperative Karnofsky Performance Status (KPS), histopathologic features, tumor size, location, radiologic findings, postoperative radiotherapy (RT), surgical treatment, and prognosis were retrospectively analyzed. Progression-free survival (PFS) and overall survival (OS) were evaluated using the Kaplan-Meier method. Univariate and multivariate analysis were conducted by the Cox regression model.ResultsMedian PFS is 20 months and median OS is 36 months in 36 patients with WHO grade III meningiomas. Patients with secondary tumors which transformed from low grade meningomas had lower PFS (p=0.0014) compared with primary group. Multivariate analysis revealed that tumors location (PFS, p=0.016; OS, p=0.013), Ki-67 index (PFS, p=0.004; OS, p<0.001) and postoperative radiotherapy (PFS, p=0.006; OS, p<0.001) were associated with prognosis.ConclusionWHO grade III meningiomas which progressed from low grade meningiomas were more prone to have recurrences or progression. Tumors location and Ki-67 index can be employed to predict patient outcomes. Adjuvant radiotherapy after surgery can significantly improve patient prognosis.

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