Clinical, radiological, and histological features and treatment outcomes of supratentorial extraventricular ependymoma: 14 cases from a single center

OBJECTIVEReports on supratentorial extraventricular ependymoma (STE) are relatively rare. The object of this study was to analyze the clinical, radiological, and histological features and treatment outcomes of 14 patients with STE.METHODSOverall, 227 patients with ependymoma underwent surgical treatment in the authors’ department between January 2010 and June 2015; 14 of these patients had STE. Data on clinical presentation, radiological studies, histopathological findings, surgical strategies, and treatment outcomes in these 14 cases were retrospectively analyzed.RESULTSThe patients consisted of 6 women and 8 men (sex ratio 0.75). Mean age at diagnosis was 24.5 ± 13.5 years (range 3–48 years). Tumors were predominantly located in the frontal and temporal lobes (5 and 4 cases, respectively). Typical radiological features were mild to moderate heterogeneous tumor enhancements on contrast-enhanced MRI. Other radiological features included well-circumscribed, “popcorn” enhancement and no distinct adjoining brain edema. Gross-total resection was achieved in 12 patients, while subtotal removal was performed in 2. Radiotherapy was administered in 7 patients after surgery. Seven tumors were classified as WHO Grade II and the other 7 were verified as WHO Grade III. The mean follow-up period was 22.6 months (range 8–39 months). There were 3 patients with recurrence, and 2 of these patients died.CONCLUSIONSSupratentorial extraventricular ependymoma has atypical clinical presentations, various radiological features, and heterogeneous histological forms; therefore, definitive diagnosis can be difficult. Anaplastic STE shows malignant biological behavior, a higher recurrence rate, and a relatively poor prognosis. Gross-total resection with or without postoperative radiotherapy is currently the optimal treatment for STE.

Download Full-text

Diagnosis and Predictors of Treatment Outcomes in Meningiomas with Atypical or Anaplastic Histology

JBNC - JORNAL BRASILEIRO DE NEUROCIRURGIA ◽

10.22290/jbnc.v27i2.751 ◽

2018 ◽

Vol 27 (2) ◽

pp. 100-104 ◽

Cited By ~ 1

Author(s):

Gustavo Simiano Jung ◽

Ricardo Ramina ◽

Erasmo Barros Da Silva Jr ◽

Maurício Coelho Neto

Keyword(s):

Treatment Outcomes ◽

Treatment Options ◽

Gross Total Resection ◽

Total Resection ◽

Who Grade ◽

Grade Ii ◽

Anaplastic Histology ◽

Biological Nature

Atypical and anaplastic meningiomas (WHO grade II and III) are uncommon tumors with poorer prognosis than benign meningiomas. They represent a small and heterogeneous subgroup of meningiomas that has more aggressive biological nature and higher frequency of recurrence. Treatment of these tumors remains challenging and recurrence is common even after gross total resection. We report five year experience of an experienced neurosurgical center (INC) reviewing treatment options and predictor of treatment outcomes for malignant meningiomas.

Download Full-text

The role of radiotherapy following gross-total resection of atypical meningiomas

Journal of Neurosurgery ◽

10.3171/2012.7.jns112113 ◽

2012 ◽

Vol 117 (4) ◽

pp. 679-686 ◽

Cited By ~ 86

Author(s):

Ricardo J. Komotar ◽

J. Bryan Iorgulescu ◽

Daniel M. S. Raper ◽

Eric C. Holland ◽

Kathryn Beal ◽

...

Keyword(s):

Postoperative Radiotherapy ◽

Prospective Trial ◽

Gross Total Resection ◽

Recurrence Rates ◽

Total Resection ◽

Who Grade ◽

Strong Trend ◽

Time To Recurrence ◽

Atypical Meningiomas

Object Atypical (WHO Grade II) meningiomas comprise a heterogeneous group of tumors, with histopathology delineated under the guidance of the WHO and a spectrum of clinical outcomes. The role of postoperative radiotherapy for patients with atypical meningiomas who have undergone gross-total resection (GTR) remains unclear. In this paper, the authors sought to clarify this role by reviewing their experience over the past 2 decades. Methods The authors retrospectively analyzed all patients at their institution who underwent GTR between 1992 and 2011 with a final histology demonstrating atypical meningioma. Information regarding patients, tumor characteristics, and postoperative adjuvant therapy was gleaned from medical records. Time to recurrence and overall survival were analyzed using univariate, multivariate, and Kaplan-Meier survival analyses. Results Forty-five patients who met the inclusion criteria underwent GTR for atypical meningiomas. By a median follow-up of 44.1 months, 22% of atypical meningiomas had recurred. There was no recurrence in 12 (92%) of 13 patients who received postoperative radiotherapy or in 19 (59%) of 32 patients who did not undergo postoperative radiotherapy (p = 0.085), demonstrating a strong trend toward improved local control with postoperative radiotherapy. No other factors were significantly associated with recurrence in univariate or multivariate analyses. Conclusions This retrospective series supports the observation that postoperative radiotherapy likely results in lower recurrence rates of gross totally resected atypical meningiomas. Although a multicenter prospective trial will ultimately be needed to fully define the role of radiotherapy in managing gross totally resected atypical meningiomas, the authors' results contribute to a growing number of series that support routine postoperative radiotherapy as an adjuvant treatment for these lesions.

Download Full-text

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

Download Full-text

Long-term efficacy of surgical resection with or without adjuvant therapy for treatment of secondary glioblastoma in adults

Neuro-Oncology Advances ◽

10.1093/noajnl/vdaa098 ◽

2020 ◽

Vol 2 (1) ◽

Author(s):

Ruoyu Huang ◽

Guanzhang Li ◽

Yiming Li ◽

Yinyan Wang ◽

Pei Yang ◽

...

Keyword(s):

Survival Analysis ◽

Prognostic Factors ◽

Adjuvant Therapy ◽

Clinical Outcomes ◽

Cox Regression ◽

Postoperative Radiotherapy ◽

Gross Total Resection ◽

Subtotal Resection ◽

Total Resection ◽

Secondary Glioblastoma

Abstract Background There are limited studies on treatment strategies and associated clinical outcomes in patients with secondary glioblastoma (sGBM). We sought to investigate the prognostic factors and treatment decisions in a retrospective cohort of patients with sGBM. Methods One hundred and seventy-one patients with sGBM who met the screening criteria were included in this study. Kaplan–Meier survival analysis and Cox survival analysis were used to detect prognostic factors. R (v3.5.0) and SPSS software (v25.0, IBM) were used to perform statistical analyses. Results The median overall survival was 303 days (range 23–2237 days) and the median progression-free survival was 229 days (range 33–1964 days) in patients with sGBM. When assessing the relationship between adjuvant treatment outcome and extent of resection (EOR), the results showed that patients underwent gross total resection can benefit from postoperative radiotherapy and chemotherapy, but not in patients underwent subtotal resection. In addition, we also found that aggressive adjuvant therapy can significantly improve clinical outcomes of IDH1-mutated patients but no significant prognostic value for IDH1-wildtyped patients. The univariate Cox regression analyses demonstrated that EOR, adjuvant therapy, and postoperative Karnofsky Performance Scores were prognostic factors for patients with sGBM, and multivariate COX analysis confirmed that adjuvant therapy and EOR were independent prognostic factors. Conclusions For patients with sGBM, aggressive postoperative adjuvant therapy after gross total resection was recommended. However, we did not detect a benefit in IDH1-wildtype patients in our cohort.

Download Full-text

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.

Download Full-text

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.

Download Full-text

Factors affecting outcome following treatment of patients with cavernous sinus meningiomas

Journal of Neurosurgery ◽

10.3171/2010.3.jns091807 ◽

2010 ◽

Vol 113 (5) ◽

pp. 1087-1092 ◽

Cited By ~ 43

Author(s):

Michael E. Sughrue ◽

Martin J. Rutkowski ◽

Derick Aranda ◽

Igor J. Barani ◽

Michael W. McDermott ◽

...

Keyword(s):

Cavernous Sinus ◽

Postoperative Radiotherapy ◽

Meta Analysis ◽

Survival Rates ◽

Extent Of Resection ◽

Gross Total Resection ◽

Cranial Neuropathy ◽

Tumor Control ◽

Subtotal Resection ◽

Total Resection

Object Although there is a considerable volume of literature available on the treatment of patients with cavernous sinus meningiomas (CSMs), most of the data regarding tumor control and survival come from case studies or single-institution series. The authors performed a meta-analysis of reported tumor control and survival rates of patients described in the published literature, with an emphasis on specific prognostic factors. Methods The authors systematically analyzed the published literature and found more than 3000 patients treated for CSMs. Separate meta-analyses were performed to calculate pooled rates of recurrence and cranial neuropathy after 1) gross-total resection, 2) subtotal resection without adjuvant postoperative radiotherapy or radiosurgery, and 3) stereotactic radiosurgery (SRS) alone. Results were expressed as pooled proportions, and random-effects models were used to incorporate any heterogeneity present to generate a pooled proportion. Individual studies were weighted using the inverse variance method, and 95% CIs for each group were calculated from the pooled proportions. Results A total of 2065 nonduplicated patients treated for CSM met inclusion criteria for the analysis. Comparisons of the 95% CIs for recurrence of these 3 cohorts revealed that SRS-treated patients experienced improved rates of recurrence (3.2% [95% CI 1.9–4.5%]) compared with either gross-total resection (11.8% [95% CI 7.4–16.1%]) or subtotal resection alone (11.1% [95% CI 6.6–15.7%]) (p < 0.01). The authors found that the pooled mixed-effects rate of cranial neuropathy was markedly higher in patients undergoing resection (59.6% [95% CI 50.3–67.5%]) than for those undergoing SRS alone (25.7% [95% CI 11.5–38.9%]) (p < 0.05). Conclusions Radiosurgery provided improved rates of tumor control compared with surgery alone, regardless of the subjective extent of resection.

Download Full-text

Microsurgical resection of giant T11/T12 conus cauda equine schwannoma

Bosnian Journal of Basic Medical Sciences ◽

10.17305/bjbms.2020.5153 ◽

2020 ◽

Author(s):

Alisa Arnautovic ◽

Mirza Pojskic ◽

Kenan Arnautovic

Keyword(s):

Motor Evoked Potentials ◽

Tumor Resection ◽

Cauda Equina ◽

Sensory Nerve ◽

Gross Total Resection ◽

Nerve Roots ◽

Total Resection ◽

Who Grade ◽

Extramedullary Tumor ◽

Microsurgical Resection

In this video, we highlight the anatomy involved with microsurgical resection of a giant T11/T12 conus cauda equine schwannoma. Spinal schwannoma remains the third most common intradural spinal tumor. Tumors undergoing gross total resection usually do not recur. To our knowledge, this is the first video case report of giant cauda equina schwannoma resection. A 55-year-old female presented with paraparesis and urinary retention. Lumbar spine MRI revealed contrast-enhancing intradural extramedullary tumor at the T11/T12 level. Surgery was performed in a prone position with intraoperative neurophysiology monitoring (somatosensory and motor evoked potentials - SSEP and MEP). T11/T12 laminectomies were performed. After opening the dura and arachnoid, the tumor was found covered with cauda equina nerve roots. We delineated the inferior pole of the tumor, followed by opening of the capsule and debulking the tumor. Subsequently, the cranial pole was dissected from the corresponding cauda equina nerve roots. Finally, the tumor nerve origin was identified and divided after nerve stimulation confirmed the tumor arose from a sensory nerve root. The tumor was removed; histological analysis revealed a schwannoma (WHO Grade I). Postoperative MRI revealed complete resection. The patient fully recovered her neurological function. This case highlights the importance of careful microsurgical technique and gross total resection of the tumor in the view of favorable postoperative neurological recovery of the patient. Intraoperative use of ultrasound is helpful to delineate preoperatively tumor extension and confirm postoperative tumor resection.

Download Full-text

Choroid plexus carcinoma in an adolescent male: a case report

Journal of Medical Case Reports ◽

10.1186/s13256-021-02801-w ◽

2021 ◽

Vol 15 (1) ◽

Author(s):

Patel Zeeshan Jameel ◽

Ashish Varma ◽

Pooja Kumari ◽

Keta Vagha ◽

Jayant Vagha ◽

...

Keyword(s):

Choroid Plexus ◽

Postoperative Radiotherapy ◽

Central India ◽

Clonic Seizure ◽

Choroid Plexus Carcinoma ◽

Gross Total Resection ◽

Adolescent Male ◽

Pathological Examination ◽

Total Resection ◽

Neck Stiffness

Abstract Introduction/background Although central nervous system tumors are the most common etiology of malignancies in the pediatric age group, choroid plexus carcinomas are rare, with an annual incidence rate of 0.10 per 100,000 children. Case presentation We report the case of an adolescent male belonging to central India who had presented with a history of persistent headache, projectile vomiting, neck stiffness, and an episode of generalized tonic-clonic seizure. Neurological examination was suggestive of a space-occupying lesion. Further neuroimaging was suggestive of a large left-sided choroid plexus carcinoma, later confirmed on pathological examination. Gross total resection was achieved and followed by radiation therapy. His recovery was satisfactory without any major events despite suffering from such a malignancy with a poor prognosis. Conclusion In the absence of a global consensus on choroid plexus carcinoma management, our patient underwent a successful gross total resection and received postoperative radiotherapy. He made a satisfactory recovery with a further plan to review with gadolinium-enhanced neuroimaging at a later date. We conclude that, when possible, achieving gross total resection is of utmost importance.

Download Full-text

Spinal ependymoma in adults: a multicenter investigation of surgical outcome and progression-free survival

Journal of Neurosurgery Spine ◽

10.3171/2017.9.spine17494 ◽

2018 ◽

Vol 28 (6) ◽

pp. 654-662 ◽

Cited By ~ 22

Author(s):

Maria Wostrack ◽

Florian Ringel ◽

Sven O. Eicker ◽

Max Jägersberg ◽

Karl Schaller ◽

...

Keyword(s):

Radiation Therapy ◽

Progression Free Survival ◽

Adult Patients ◽

Gross Total Resection ◽

Adjuvant Radiation ◽

Total Resection ◽

Ki 67 ◽

Who Grade ◽

Free Survival ◽

Spinal Ependymoma

OBJECTIVESpinal ependymomas are rare glial neoplasms. Because their incidence is low, only a few larger studies have investigated this condition. There are no clear data concerning prognosis and therapy. The aim of the study was to describe the natural history, perioperative clinical course, and local tumor control of adult patients with spinal ependymomas who were surgically treated under modern treatment standards.METHODSThe authors performed a multicenter retrospective study. They identified 158 adult patients with spinal ependymomas who had received surgical treatment between January 2006 and June 2013. The authors analyzed the clinical and histological aspects of these cases to identify the predictive factors for postoperative morbidity, tumor resectability, and recurrence.RESULTSGross-total resection (GTR) was achieved in 80% of cases. At discharge, 37% of the patients showed a neurological decline. During follow-up the majority recovered, whereas 76% showed at least preoperative status. Permanent functional deterioration remained in 2% of the patients. Transient deficits were more frequent in patients with cervically located ependymomas (p = 0.004) and in older patients (p = 0.002). Permanent deficits were independently predicted only by older age (p = 0.026). Tumor progression was observed in 15 cases. The 5-year progression-free survival (PFS) rate was 80%, and GTR (p = 0.037), WHO grade II (p = 0.009), and low Ki-67 index (p = 0.005) were independent prognostic factors for PFS. Adjuvant radiation therapy was performed in 15 cases. No statistically relevant effects of radiation therapy were observed among patients with incompletely resected ependymomas (p = 0.079).CONCLUSIONSDue to its beneficial value for PFS, GTR is important in the treatment of spinal ependymoma. Gross-total resection is feasible in the majority of cases, with acceptable rates of permanent deficits. Also, Ki-67 appears to be an important prognostic factor and should be included in a grading scheme for spinal ependymomas.

Download Full-text