scholarly journals Cerebellopontine angle ependymoma presenting as isolated hearing loss in an elderly patient: A case report and literature review

2021 ◽  
Vol 12 ◽  
pp. 572
Author(s):  
Khaled Dibs ◽  
Rahul Neal Prasad ◽  
Kajal Madan ◽  
Kevin Liu ◽  
Will Jiang ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Local Recurrence ◽  
Cerebellopontine Angle ◽  
Case Reports ◽  
Disease Recurrence ◽  
World Health ◽  
Adjuvant Radiation ◽  
Who Grade ◽  
Imaging Characteristics ◽  
Grade Ii

Background: Ependymoma is an uncommon tumor accounting for approximately 1.9% of all adult central nervous system tumors. Ependymomas at the cerebellopontine angle (CPA) are even more rare and only previously described in isolated case reports. Typically, acoustic neuromas and meningiomas represent the bulk of adult CPA tumors. Diagnosis can be challenging, as ependymomas have clinical findings and imaging characteristics that overlap with more common tumor histologies at the CPA. Case Description: We present the case of a 70-year-old male patient with progressive, isolated left-sided hearing loss found to have a World Health Organization (WHO) Grade II CPA ependymoma, representing one of the oldest recorded patients presenting with this primarily pediatric malignancy in this unique location. The patient presentation with isolated hearing loss was particularly unusual. When associated with neurologic deficits, CPA ependymomas more characteristically result in facial nerve impairment with fully preserved hearing, while vestibular schwannomas tend to present with isolated hearing loss. The standard of care for pediatric ependymomas is maximal safe resection with adjuvant radiotherapy, but treatment paradigms in adult CPA ependymoma are not well defined particularly for WHO Grade II disease. After resection, he received adjuvant radiation to decrease the risk of local recurrence. Twenty-nine months after resection, the patient remains free of treatment-related toxicity or disease recurrence. Conclusion: We review this patient’s clinical course in the context of the literature to highlight the challenges associated with timely diagnosis of this rare tumor and the controversial role of adjuvant therapy in preventing local recurrence in these patients.

scholarly journals PATH-35. A SCALABLE MOLECULARLY INTEGRATED CLASSIFIER FOR MENINGIOMA OUTPERFORMS WHO CLASSIFICATION

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii172-ii172
Author(s):  
Wenya Linda Bi ◽  
Joseph Driver ◽  
Sherwin Tavakol ◽  
Samantha Hoffman ◽  
Eleanor Woodward ◽  
...  
Keyword(s):  
Copy Number ◽  
Who Classification ◽  
Statistical Tests ◽  
Extent Of Resection ◽  
World Health ◽  
Brier Score ◽  
Adjuvant Radiation ◽  
Who Grade ◽  
Genome Wide ◽  
Grade Ii

Abstract The clinical behavior of meningiomas often belies their histopathologic grade. Increasing data suggest that molecular features augment classic World Health Organization (WHO) classification in predicting recurrent risk. We investigated the influence of histopathology, chromosome copy number, and treatment on meningioma outcome to construct a simple, scalable, molecularly integrated classifier. METHODS: We analyzed 684 meningiomas with genome-wide chromosomal copy-number profiling for clinical features, pre-/post-operative tumor volume, histopathology, and recurrence. We devised a point-based molecularly-integrated classification system (IC 1-3), incorporating mitotic count and nine common molecular alterations consistently associated with risk of recurrence across four independent statistical tests. We used brier curves, time-dependent area-under-the-curve, and average precision to assess model performance. We added treatment variables, including primary or recurrent status, tumor size, and extent of resection, to the Integrated class to formulate a nomogram of recurrence risk at 5 years. RESULTS: The Integrated Class significantly associated with recurrence in a multivariate model (IC Class 2 vs 1: HR 3.60, 95% CI 2.19-5.91; Class 3 vs 1: HR 5.15, 95% CI 3.28-8.09) and outperformed WHO grade in predicting recurrence (by integrated brier score, 0.093 vs 0.178, internally and independently validated). WHO Grade I and IC-1 exhibited 86% concordance, 31.5% between Grade II and IC-2, and 64% between Grade III and IC-3. WHO grade I meningiomas with IC 2-3 fared significantly worse than WHO grade II-III meningiomas with IC-1 designation. Each additional molecular feature incrementally strengthens the classifier, allowing for application of single-arm FISH or combinatorial genome-wide signatures based on available resources. Intriguingly, receipt of adjuvant radiation in newly diagnosed WHO Grade II-III or IC 2-3 meningiomas was associated with greater propensity for recurrence, after controlling for extent of resection. CONCLUSION: We present a scalable, molecularly-integrated classification for meningioma that better predicts recurrence compared to classic histopathologic grades to aid in clinical management.

scholarly journals Extracranial metastases in secondary glioblastoma multiforme: a case report

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Jessica Rossi ◽  
Lucia Giaccherini ◽  
Francesco Cavallieri ◽  
Manuela Napoli ◽  
Claudio Moratti ◽  
...  
Keyword(s):  
Brain Lesion ◽  
Rare Event ◽  
Subsequent Development ◽  
World Health ◽  
Who Grade ◽  
Systemic Involvement ◽  
Grade Ii ◽  
The Stability ◽  
Health Organization ◽  
Extracranial Metastases

Abstract Background Glioblastoma (GBM) is known for its devastating intracranial infiltration and its unfavorable prognosis, while extracranial involvement is a very rare event, more commonly attributed to IDH wild-type (primary) GBM evolution. Case presentation We present a case of a young woman with a World Health Organization (WHO) grade II Astrocytoma evolved to WHO grade IV IDH mutant glioblastoma, with subsequent development of lymphatic and bone metastases, despite the favorable biomolecular pattern and the stability of the primary brain lesion. Conclusions Our case highlights that grade II Astrocytoma may evolve to a GBM and rarely lead to a secondary metastatic diffusion, which can progress quite rapidly; any symptoms referable to a possible systemic involvement should be carefully investigated.

scholarly journals The Role of Extent of Resection in IDH1 Wild-Type or Mutant Low-Grade Gliomas

Neurosurgery ◽  
2017 ◽  
Vol 82 (6) ◽  
pp. 808-814 ◽  
Author(s):  
Toral Patel ◽  
Evan D Bander ◽  
Rachael A Venn ◽  
Tiffany Powell ◽  
Gustav Young-Min Cederquist ◽  
...  
Keyword(s):  
Cox Regression ◽  
Extent Of Resection ◽  
World Health ◽  
Low Grade ◽  
Wild Type ◽  
Who Grade ◽  
Cox Regression Analysis ◽  
Low Grade Gliomas ◽  
Grade Ii ◽  
The Impact

Abstract BACKGROUND Maximizing extent of resection (EOR) improves outcomes in adults with World Health Organization (WHO) grade II low-grade gliomas (LGG). However, recent studies demonstrate that LGGs bearing a mutation in the isocitrate dehydrogenase 1 (IDH1) gene are a distinct molecular and clinical entity. It remains unclear whether maximizing EOR confers an equivalent clinical benefit in IDH mutated (mtIDH) and IDH wild-type (wtIDH) LGGs. OBJECTIVE To assess the impact of EOR on malignant progression-free survival (MPFS) and overall survival (OS) in mtIDH and wtIDH LGGs. METHODS We performed a retrospective review of 74 patients with WHO grade II gliomas and known IDH mutational status undergoing resection at a single institution. EOR was assessed with quantitative 3-dimensional volumetric analysis. The effect of predictor variables on MPFS and OS was analyzed with Cox regression models and the Kaplan–Meier method. RESULTS Fifty-two (70%) mtIDH patients and 22 (30%) wtIDH patients were included. Median preoperative tumor volume was 37.4 cm3; median EOR of 57.6% was achieved. Univariate Cox regression analysis confirmed EOR as a prognostic factor for the entire cohort. However, stratifying by IDH status demonstrates that greater EOR independently prolonged MPFS and OS for wtIDH patients (hazard ratio [HR] = 0.002 [95% confidence interval {CI} 0.000-0.074] and HR = 0.001 [95% CI 0.00-0.108], respectively), but not for mtIDH patients (HR = 0.84 [95% CI 0.17-4.13] and HR = 2.99 [95% CI 0.15-61.66], respectively). CONCLUSION Increasing EOR confers oncologic and survival benefits in IDH1 wtLGGs, but the impact on IDH1 mtLGGs requires further study.

scholarly journals Huge heterogeneity in survival in a subset of adult patients with resected, wild-type isocitrate dehydrogenase status, WHO grade II astrocytomas

2019 ◽  
Vol 130 (4) ◽  
pp. 1289-1298 ◽  
Author(s):  
Gaëtan Poulen ◽  
Catherine Gozé ◽  
Valérie Rigau ◽  
Hugues Duffau
Keyword(s):  
Radiation Therapy ◽  
Malignant Transformation ◽  
Isocitrate Dehydrogenase ◽  
Adult Patients ◽  
World Health ◽  
Wild Type ◽  
Who Grade ◽  
Grade Ii ◽  
The Individual

OBJECTIVEWorld Health Organization grade II gliomas are infiltrating tumors that inexorably progress to a higher grade of malignancy. However, the time to malignant transformation is quite unpredictable at the individual patient level. A wild-type isocitrate dehydrogenase (IDH-wt) molecular profile has been reported as a poor prognostic factor, with more rapid progression and a shorter survival compared with IDH-mutant tumors. Here, the oncological outcomes of a series of adult patients with IDH-wt, diffuse, WHO grade II astrocytomas (AII) who underwent resection without early adjuvant therapy were investigated.METHODSA retrospective review of patients extracted from a prospective database who underwent resection between 2007 and 2013 for histopathologically confirmed, IDH-wt, non–1p19q codeleted AII was performed. All patients had a minimum follow-up period of 2 years. Information regarding clinical, radiographic, and surgical results and survival were collected and analyzed.RESULTSThirty-one consecutive patients (18 men and 13 women, median age 39.6 years) were included in this study. The preoperative median tumor volume was 54 cm3 (range 3.5–180 cm3). The median growth rate, measured as the velocity of diametric expansion, was 2.45 mm/year. The median residual volume after surgery was 4.2 cm3 (range 0–30 cm3) with a median volumetric extent of resection of 93.97% (8 patients had a total or supratotal resection). No patient experienced permanent neurological deficits after surgery, and all patients resumed a normal life. No immediate postoperative chemotherapy or radiation therapy was given. The median clinical follow-up duration from diagnosis was 74 months (range 27–157 months). In this follow-up period, 18 patients received delayed chemotherapy and/or radiotherapy for tumor progression. Five patients (16%) died at a median time from radiological diagnosis of 3.5 years (range 2.6–4.5 years). Survival from diagnosis was 77.27% at 5 years. None of the 21 patients with a long-term follow-up greater than 5 years have died. There were no significant differences between the clinical, radiological, or molecular characteristics of the survivors relative to the patients who died.CONCLUSIONSHuge heterogeneity in the survival data for a subset of 31 patients with resected IDH-wt AII tumors was observed. These findings suggest that IDH mutation status alone is not sufficient to predict risk of malignant transformation and survival at the individual level. Therefore, the therapeutic management of AII tumors, in particular the decision to administer early adjuvant chemotherapy and/or radiation therapy following surgery, should not solely rely on routine molecular markers.

Pediatric thalamic incidentalomas: an international retrospective multicenter study

2021 ◽  
pp. 1-9
Author(s):  
Danil A. Kozyrev ◽  
Jehuda Soleman ◽  
Deki Tsering ◽  
Robert F. Keating ◽  
David S. Hersh ◽  
...  
Keyword(s):  
Multicenter Study ◽  
Thalamic Lesion ◽  
High Grade ◽  
Who Grade ◽  
Imaging Characteristics ◽  
Surgical Complexity ◽  
Grade Ii ◽  
Thalamic Lesions ◽  
International Multicenter Study ◽  
Immediate Surgery

OBJECTIVE Widespread use of modern neuroimaging has led to a surge in diagnosing pediatric brain incidentalomas. Thalamic lesions have unique characteristics such as deep location, surgical complexity, and proximity to eloquent neuronal structures. Currently, the natural course of incidental thalamic lesions is unknown. Therefore, the authors present their experience in treating such lesions. METHODS A retrospective, international multicenter study was carried out in 8 tertiary pediatric centers from 5 countries. Patients were included if they had an incidental thalamic lesion suspected of being a tumor and were diagnosed before the age of 20 years. Treatment strategy, imaging characteristics, pathology, and the outcome of operated and unoperated cases were analyzed. RESULTS Overall, 58 children (23 females and 35 males) with a mean age of 10.8 ± 4.0 years were included. The two most common indications for imaging were nonspecific reasons (n = 19; e.g., research and developmental delay) and headache unrelated to small thalamic lesions (n = 14). Eleven patients (19%) underwent early surgery and 47 were followed, of whom 10 underwent surgery due to radiological changes at a mean of 11.4 ± 9.5 months after diagnosis. Of the 21 patients who underwent surgery, 9 patients underwent resection and 12 underwent biopsy. The two most frequent pathologies were pilocytic astrocytoma and WHO grade II astrocytoma (n = 6 and n = 5, respectively). Three lesions were high-grade gliomas. CONCLUSIONS The results of this study indicate that pediatric incidental thalamic lesions include both low- and high-grade tumors. Close and long-term radiological follow-up is warranted in patients who do not undergo immediate surgery, as tumor progression may occur.

Stereotactic radiation treatment for recurrent nonbenign meningiomas

2007 ◽  
Vol 106 (5) ◽  
pp. 846-854 ◽  
Author(s):  
Carlos A. Mattozo ◽  
Antonio A. F. De Salles ◽  
Ivan A. Klement ◽  
Alessandra Gorgulho ◽  
David McArthur ◽  
...  
Keyword(s):  
Radiation Treatment ◽  
Progression Free Survival ◽  
Malignant Progression ◽  
World Health ◽  
Who Grade ◽  
Stereotactic Radiation ◽  
Grade Ii ◽  
Health Organization ◽  
Grade Iii

Object The authors analyzed the results of stereotactic radiosurgery (SRS) and stereotactic radiotherapy (SRT) for the treatment of recurrent meningiomas that were described at initial resection as showing aggressive, atypical, or malignant features (nonbenign). Methods Twenty-five patients who underwent SRS and/or SRT for nonbenign meningiomas between December 1992 and August 2004 were included. Thirteen of these patients underwent treatment for multiple primary or recurrent lesions. In all, 52 tumors were treated. All histological sections were reviewed and reclassified according to World Health Organization (WHO) 2000 guidelines as benign (Grade I), atypical (Grade II), or anaplastic (Grade III) meningiomas. The median follow-up period was 42 months. Seventeen (68%) of the cases were reclassified as follows: WHO Grade I (five cases), Grade II (11 cases), and Grade III (one case). Malignant progression occurred in eight cases (32%) during the follow-up period; these cases were considered as a separate group. The 3-year progression-free survival (PFS) rates for the Grades I, II, and III, and malignant progression groups were 100, 83, 0, and 11%, respectively (p < 0.001). In the Grade II group, the 3-year PFS rates for patients treated with SRS and SRT were 100 and 33%, respectively (p = 0.1). After initial treatment, 22 new tumors required treatment using SRS or SRT; 17 (77%) of them occurred inside the original resection cavity. Symptomatic edema developed in one patient (4%). Conclusions Stereotactic radiation treatment provided effective local control of “aggressive” Grade I and Grade II meningiomas, whereas Grade III lesions were associated with poor outcome. The outcome of cases in the malignant progression group was intermediate between that of the Grade II and Grade III groups, with the lesions showing a tendency toward malignancy.

scholarly journals Oligodendroglioma with Sarcomatous Transformation: Case Report and Literature Review

2019 ◽  
Vol 38 (02) ◽  
pp. 128-136
Author(s):  
Gonçalo Cerdeira Figueiredo ◽  
Célia Maria Pinheiro ◽  
Alfredo Luís Calheiros
Keyword(s):  
Spinal Metastasis ◽  
Poor Response ◽  
World Health ◽  
Rapid Progression ◽  
Who Grade ◽  
Genetic Characteristics ◽  
Oligodendroglial Component ◽  
The Central Nervous System ◽  
Grade Ii ◽  
Health Organization

AbstractOligodendrogliomas are infiltrative tumors of the central nervous system considered to be morphologically stable and to offer a better prognosis. Here, we describe the case of a 36-year-old man with an initial diagnosis of oligodendroglioma, World Health Organization (WHO) grade II, who presented transformation to a sarcomatous form, while maintaining the oligodendroglial component as well as the genetic characteristics of the initial tumor without having undergone any complementary treatments previously. Despite the favorable genetic characteristics, the tumor presented poor response to complementary treatments, and rapid progression, including spinal metastasis.

The role of radiosurgery in the management of WHO Grade II and III intracranial meningiomas

2013 ◽  
Vol 35 (6) ◽  
pp. E16 ◽  
Author(s):  
Dale Ding ◽  
Robert M. Starke ◽  
John Hantzmon ◽  
Chun-Po Yen ◽  
Brian J. Williams ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Stereotactic Radiosurgery ◽  
Case Reports ◽  
Case Series ◽  
External Beam Radiation ◽  
Beam Radiation ◽  
Who Grade ◽  
Intracranial Meningiomas ◽  
Grade Ii ◽  
Grade Iii

Object WHO Grade II and III intracranial meningiomas are uncommon, but they portend a significantly worse prognosis than their benign Grade I counterparts. The mainstay of current management is resection to obtain cytoreduction and histological tissue diagnosis. The timing and benefit of postoperative fractionated external beam radiation therapy and stereotactic radiosurgery remain controversial. The authors review the stereotactic radiosurgery outcomes for Grade II and III meningiomas. Methods A comprehensive literature search was performed using PubMed to identify all radiosurgery series reporting the treatment outcomes for Grade II and III meningiomas. Case reports and case series involving fewer than 10 patients were excluded. Results From 1998 to 2013, 19 radiosurgery series were published in which 647 Grade II and III meningiomas were treated. Median tumor volumes were 2.2–14.6 cm3. The median margin doses were 14–21 Gy, although generally the margin doses for Grade II meningiomas were 16–20 Gy and the margin doses for Grade III meningiomas were 18–22 Gy. The median 5-year PFS was 59% for Grade II tumors and 13% for Grade III tumors, which may have been affected by patient age, prior radiation therapy, tumor volume, and radiosurgical dose and timing. The median complication rate following radiosurgery was 8%. Conclusions The current data for radiosurgery suggest that it has a role in the management of residual or recurrent Grade II and III meningiomas. However, better studies are needed to fully define this role. Due to the relatively low prevalence of these tumors, it is unlikely that prospective studies will be feasible. As such, well-designed retrospective analyses may improve our understanding of the effect of radiosurgery on tumor recurrence and patient survival and the incidence and impact of treatment-induced complications.

scholarly journals Benign meningiomas (WHO Grade I) with atypical histological features: correlation of histopathological features with clinical outcomes

2016 ◽  
Vol 124 (1) ◽  
pp. 106-114 ◽  
Author(s):  
Ariel E. Marciscano ◽  
Anat O. Stemmer-Rachamimov ◽  
Andrzej Niemierko ◽  
Mykol Larvie ◽  
William T. Curry ◽  
...  
Keyword(s):  
Prognostic Significance ◽  
Risk Groups ◽  
World Health ◽  
Who Grade ◽  
Simpson Grade ◽  
Atypical Features ◽  
Additional Surgery ◽  
Increased Risk ◽  
Grade Ii ◽  
Health Organization

OBJECT World Health Organization (WHO) Grade I (benign) meningiomas with atypical features may behave more aggressively than similarly graded tumors without atypical features. Here, the prognostic significance of atypical features in benign meningiomas was determined. METHODS Data from patients diagnosed with WHO Grade I benign meningiomas per the 2007 WHO criteria and who underwent surgery between 2002 and 2012 were retrospectively reviewed. Patients were stratified by the absence or presence of 1 to 2 atypical features with review of the clinical and histological factors. RESULTS A total of 148 patients met the inclusion criteria (n = 77 with atypia; n = 71 without atypia). The median follow-up duration after pathological diagnosis was 37.5 months. Thirty patients had progression/recurrence (P/R) after initial treatment, and 22 (73%) of 30 patients with P/R had 1–2 atypical features. The presence of atypical features was significantly associated with P/R (p = 0.03) and independent of the MIB-1 labeling index. The 1-year and 5-year actuarial rates of P/R were 9.6% versus 1.4% and 30.8% versus 13.8% fortumors with and without atypical features, respectively. Higher Simpson grade resection (II–IV vs I) was associated with the increased risk of P/R (p < 0.001). Stratification of patients into low-risk (Simpson Grade I), intermediate-risk (Simpson Grade II–IV with no atypical features), and high-risk groups (Simpson Grade II–IV with atypical features) was significantly correlated with increased risk of P/R (p < 0.001). CONCLUSIONS Patients with benign meningiomas with atypical features and those undergoing Simpson Grade II–IV resection are at significantly increased risk of P/R. Patients with these features may benefit from the consideration of additional surgery and/or radiation therapy.

scholarly journals Absence of contrast enhancement in a petroclival meningioma: Case report and systematic literature review

2020 ◽  
Vol 11 ◽  
pp. 418
Author(s):  
Adedamola Adepoju ◽  
Ananth Narayan ◽  
Mahmoud Aldyab ◽  
David Foyt ◽  
Maria Peris-Celda
Keyword(s):  
Contrast Enhancement ◽  
Cerebellopontine Angle ◽  
Case Reports ◽  
Tumor Location ◽  
Foramen Magnum ◽  
Tumor Vascularity ◽  
Who Grade ◽  
Facial Numbness ◽  
Magnetic Resonance Imaging Contrast ◽  
Radiologic Features

Background: Meningioma is one of the most common intracranial tumors with well-established radiologic features such as contrast enhancement, dural tail, and hyperostosis on computed tomography and magnetic resonance imaging. Contrast enhancement is usually homogeneous or heterogeneous based on tumor vascularity and underlying histopathology. Even in this context, faint or nonenhancing meningioma is exceedingly rare. Case Description: A 57-year-old male presented with progressive right hearing loss, disequilibrium, occasional difficulty swallowing, and facial numbness. Imaging demonstrated an extensive hypodense, nonenhancing right cerebellopontine angle mass extending from the interpeduncular, and ambient cisterns to the foramen magnum. The pathological analysis demonstrated a microcystic meningioma WHO Grade I. There are few reported case reports or series of minimal or nonenhancing meningiomas, and a systematic review was performed for these cases. Seven peer-reviewed articles with 14 verifiable cases were identified and reviewed for radiologic features, tumor location, and tumor classification. The majority of minimal or nonenhancing meningiomas were microcystic, and most of them located at the convexity. This is the second case reported of a nonenhancing meningioma at the cerebellopontine angle and petroclival region Conclusion: Meningioma should be considered a differential diagnosis for nonenhancing lesion at the cerebellopontine and petroclival regions.

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