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Journal of Computer Assisted TomographyTo improve the understanding and the diagnosis of intracranial ependymal tumors.
OBJECTIVE
To improve the understanding and the diagnosis of intracranial ependymal tumors.
METHODS
The clinical, radiological and prognostic features of 48 supratentorial extraventricular ependymomas and 74 intraventricular ependymomas were summarized and compared.
RESULTS
Supratentorial extraventricular ependymomas, most often located in the frontal lobe (33.3%) and classified as grade III (75.0%), had relatively large eccentric cysts (3.07 ± 2.03 cm), significant enhancement (84.8%), low apparent diffusion coefficient (ADC) values, and associated with higher mortality (41.3%). The majority of intraventricular lesions occurred in the fourth ventricle (86.5%) and classified as grade II (78.4%), had relatively small and multiple cystic changes (1.04 ± 0.87 cm), slight or moderate enhancement (76.9%), high ADC values and associated with lower mortality (20.7%). There were few significant differences between grade II and grade III tumors in these 2 groups, respectively. Young age, high grade and low ADC values are worse prognostic indicators for patients with supratentorial extraventricular ependymomas, but not for those with intraventricular ependymomas.
CONCLUSIONS
Conventional radiological features, combined with clinical manifestations and quantitative information provided by diffusion-weighted imaging, may not only enhance the diagnosis and assist in determining prognosis but also provide a better pathophysiological understanding of intracranial ependymal tumors.
Topics: Adolescent; Adult; Age Factors; Brain Neoplasms; Diffusion Magnetic Resonance Imaging; Ependymoma; Female; Humans; Male; Middle Aged; Mortality; Neoplasm Grading; Prognosis; Supratentorial Neoplasms; Tomography, X-Ray Computed; Young Adult
PubMed: 34297516
DOI: 10.1097/RCT.0000000000001164 -
Brain Pathology (Zurich, Switzerland) Mar 2019Ependymoma with YAP1-MAMLD1 fusion is a rare, recently described supratentorial neoplasm of childhood, with few cases published so far. We report on 15 pediatric...
Ependymoma with YAP1-MAMLD1 fusion is a rare, recently described supratentorial neoplasm of childhood, with few cases published so far. We report on 15 pediatric patients with ependymomas carrying YAP1-MAMLD1 fusions, with their characteristic histopathology, immunophenotype and molecular/cytogenetic, radiological and clinical features. The YAP1-MAMLD1 fusion was documented by RT-PCR/Sanger sequencing, and tumor genomes were studied by molecular inversion probe (MIP) analysis. Significant copy number alterations were identified by GISTIC (Genomic Identification of Significant Targets in Cancer) analysis. All cases showed similar histopathological features including areas of high cellularity, presence of perivascular pseudo-rosettes, small to medium-sized nuclei with characteristic granular chromatin and strikingly abundant cells with dot-like cytoplasmic expression of epithelial membrane antigen. Eleven cases presented features of anaplasia, corresponding to WHO grade III. MRI showed large supratentorial multinodular tumors with cystic components, heterogeneous contrast enhancement, located in the ventricular or periventricular region. One of two variants of YAP1-MAMLD1 fusions was detected in all cases. The MIP genome profiles showed balanced profiles, with focal alterations of the YAP1 locus at 11q22.1-11q21.2 (7/14), MAMLD1 locus (Xp28) (10/14) and losses of chromosome arm 22q (5/14). Most patients were female (13/15) and younger than 3 years at diagnosis (12/15; median age, 8.2 months). Apart from one patient who died during surgery, all patients are alive without evidence of disease progression after receiving different treatment protocols, three without postoperative further treatment (median follow-up, 4.84 years). In this to date, largest series of ependymomas with YAP1-MAMLD1 fusions we show that they harbor characteristic histopathological, cytogenetic and imaging features, occur mostly in young girls under 3 years and are associated with good outcome. Therefore, this genetically defined neoplasm should be considered a distinct disease entity. The diagnosis should be confirmed by demonstration of the specific fusion. Further studies on large collaborative series are warranted to confirm our findings.
Topics: Adaptor Proteins, Signal Transducing; Child; Child, Preschool; DNA Copy Number Variations; DNA-Binding Proteins; Ependymoma; Female; Humans; Infant; Male; Nuclear Proteins; Phosphoproteins; Retrospective Studies; Supratentorial Neoplasms; Transcription Factors; YAP-Signaling Proteins
PubMed: 30246434
DOI: 10.1111/bpa.12659 -
Clinical Neuroradiology Dec 2019Epigenetic profiling has recently identified clinically and molecularly distinct subgroups of ependymoma. The 2016 World Health Organization (WHO) classification...
PURPOSE
Epigenetic profiling has recently identified clinically and molecularly distinct subgroups of ependymoma. The 2016 World Health Organization (WHO) classification recognized supratentorial ependymomas (ST-EPN) with REL-associated protein/p65 (RELA) fusion as a clinicopathological entity. These tumors represent 70% of pediatric ST-EPN characterized by recurrent C11orf95-RELA fusion transcripts, which lead to pathological activation of the nuclear factor 'kappa-light-chain-enhancer' of activated B-cells (NF-κB) signaling pathway. Cyclin-dependent kinase inhibitor 2A (CDKN2A) inactivation has also been reported to correlate with poor prognosis. Here, we systematically describe magnetic resonance imaging (MRI) characteristics of RELA-fused ST-EPN, with respect to CDKN2A deletion status.
METHODS
Our cohort of patients with ST-EPN (n = 57) was obtained from the database of the German Brain Tumor Reference Center of the German Society for Neuropathology and Neuroanatomy (DGNN), and tumors were diagnosed according to the 2016 WHO classification. Molecular characterization identified 47 RELA-fused tumors. We analyzed the preoperative MRI according to standardized criteria, and comparison was performed between CDKN2A altered (n = 21) and CDKN2A wild type (n = 26) tumors.
RESULTS
The RELA-fused ST-EPN showed a spectrum of predominantly hemispheric tumors with cysts and necrosis. Statistical analysis on CDKN2A status revealed significant differences in terms of younger manifestation age (p =0.002) and more intratumoral hemorrhage in T2-weighted imaging (T2WI) (p =0.010) in wild type tumors; however, the location was not a parameter for differentiation.
CONCLUSION
This study first provides comprehensive MRI data for RELA-fused ST-EPN as a distinct entity, with further interest on CDKN2A genomic status. Patient stratification by morphological MRI alone seems difficult at present. The results may support ongoing research in ST-EPN within the framework of the radiogenomics concept.
Topics: Adolescent; Child; Child, Preschool; Databases, Factual; Ependymoma; Female; Gene Deletion; Genes, p16; Humans; Image Interpretation, Computer-Assisted; Magnetic Resonance Imaging; Male; Neoplasm Grading; Oncogene Proteins, Fusion; Phenotype; Supratentorial Neoplasms; Transcription Factor RelA
PubMed: 30027327
DOI: 10.1007/s00062-018-0704-2 -
World Neurosurgery Oct 2018Supratentorial extraventricular ependymoma (STEE) is an extremely rare central nervous system (CNS) neoplasm, the clinical characteristics and optimal treatment of which...
BACKGROUND
Supratentorial extraventricular ependymoma (STEE) is an extremely rare central nervous system (CNS) neoplasm, the clinical characteristics and optimal treatment of which remain unclear. We retrospectively analyzed the clinical characteristics and treatment outcomes of 15 patients with STEE.
METHODS
Fifteen patients with STEE were identified, and their clinical, radiologic, and surgical records were reviewed.
RESULTS
The 15 patients included 6 males (40%) and 9 females (60%), with a median age of 15 years. Nine patients (60%) underwent gross total resection, 5 patients (33.3%) underwent subtotal resection, and 1 patient (6.7%) underwent biopsy. Eight patients received adjuvant radiotherapy, and 3 received adjuvant chemotherapy. The 5-year overall survival (OS) rates of patients with World Health Organization (WHO) grade II and grade III STEE were 100% and 30%, respectively, and the corresponding 2-year progression-free survival (PFS) rates were 77.8% and 20%. The differences in OS and PFS rates between WHO grades II and III STEE were statistically significant (P < 0.01 and 0.02, respectively). Anaplastic histology (WHO grade III) was a poor prognostic factor for PFS (P = 0.04). The recurrence pattern was local recurrence in all patients; all cases were treated primarily by redo surgery. Three patients with recurrent WHO grade II STEE were alive at more than 64, 52, and 44 months after redo surgery.
CONCLUSIONS
STEE is an extremely rare CNS neoplasm. The histological grade of STEE is an important prognostic factor. Microsurgical resection might play a major role in both initial treatment and treatment of recurrent tumors.
Topics: Adolescent; Adult; Child; Child, Preschool; Ependymoma; Female; Follow-Up Studies; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Retrospective Studies; Supratentorial Neoplasms; Young Adult
PubMed: 29879510
DOI: 10.1016/j.wneu.2018.05.192 -
World Neurosurgery Mar 2020Dermoid cysts are benign, congenital rare lesions, frequently occurring in or near the midline. Rare localizations and variable radiologic findings have been described...
BACKGROUND
Dermoid cysts are benign, congenital rare lesions, frequently occurring in or near the midline. Rare localizations and variable radiologic findings have been described but remain exceptional.
CASE DESCRIPTION
The authors present a rare case of a giant temporoparietal dermoid cyst in an adult female who was paucisymptomatic. No sign of rupture was identified, with a clinical presentation suggesting a slow-growing lesion. Gross total excision was performed with a favorable clinical and radiologic outcome.
CONCLUSIONS
Rare localizations previously published in the literature are discussed for dermoid cysts. To our best knowledge, this is the first giant dermoid cyst presenting with this localization.
Topics: Brain; Dermoid Cyst; Diagnosis, Differential; Female; Humans; Middle Aged; Supratentorial Neoplasms
PubMed: 31821914
DOI: 10.1016/j.wneu.2019.12.007 -
Neurosurgery Apr 1999Ependymomas arise from different areas in the neuraxis and have variable outcomes that depend on tumor location and patient age at the time of presentation. The...
OBJECTIVE
Ependymomas arise from different areas in the neuraxis and have variable outcomes that depend on tumor location and patient age at the time of presentation. The predictive value of histology for these tumors is unresolved. We report a series of adult patients with supratentorial ependymomas to characterize the roles of surgery, histology, ploidy, and proliferation index in tumor control.
METHODS
Fourteen of the 23 supratentorial ependymomas were in the region of the third ventricle and the remainder were located in the hemispheres. Resections were gross total in 12 patients, subtotal in 8, and biopsy in 3. A single pathologist reviewed all slides and quantitated the deoxyribonucleic acid. The mean follow-up duration was 95 months (+/-75 mo).
RESULTS
All of the malignant ependymomas were hemispheric (n = 4). Mortality occurred only in patients with third ventricular tumors; two patients died as a result of surgical complications and three as a result of tumor progression. Kaplan-Meier estimates of 5- and 10-year survival rates were 100% for hemispheric and 72.5% for third ventricular tumors (62.5% including the two perioperative deaths). The median time to recurrence was 53 months, with a 10-year progression-free survival rate of 27%. Univariate analysis revealed that recurrence was associated with malignant histology, including mitoses, cellularity, and aneuploidy. For nonmalignant ependymomas, recurrence was associated with subtotal resection and metastases. S-phase fraction did not correlate with recurrence. Only malignant histology correlated with recurrence on multivariate analysis.
CONCLUSION
Although the numbers are too small to draw any definite conclusions, treatment of ependymomas that arise in the supratentorial compartment in adult patients results in excellent outcomes despite frequent recurrences. Association with the third ventricle and metastases seem to have a negative impact on survival, whereas malignant histology, subtotal resection, and metastases may be predictors of recurrence.
Topics: Adolescent; Adult; Aged; Cell Division; Combined Modality Therapy; Disease-Free Survival; Ependymoma; Humans; Middle Aged; Multivariate Analysis; Neoplasm Recurrence, Local; Ploidies; Prognosis; Retrospective Studies; Supratentorial Neoplasms; Survival Rate
PubMed: 10201296
DOI: 10.1097/00006123-199904000-00018 -
Turkish Neurosurgery Apr 2009Astroblastoma is a rare glial tumor with uncertain histopathological origin and unpredictable clinical behavior. In this report, the authors present two cases of high... (Review)
Review
Astroblastoma is a rare glial tumor with uncertain histopathological origin and unpredictable clinical behavior. In this report, the authors present two cases of high grade intracerebral astroblastomas. Both tumors occurred in children as supratentorial, well-circumscribed, peripheral masses. The lesions differed radigraphically; one contained a huge cystic component and heterogeneously enhancing mural nodule while the other appeared as a prominently contrastenhancing solid mass lesion. Both patients were treated with surgery and postoperative radiotherapy. They were followed-up long-term and no recurrence of the tumor was detected in either case. We also discussed the radiological and histological characteristics with prognostic features.
Topics: Biopsy; Child; Combined Modality Therapy; Female; Humans; Magnetic Resonance Imaging; Neoplasms, Neuroepithelial; Supratentorial Neoplasms
PubMed: 19431125
DOI: No ID Found -
Clinical Neurology and Neurosurgery Feb 2018Supratentorial extraventricular ependymomas are relatively rare. Long-term outcomes and prognostic factor for this rare tumor have not been well established. The purpose...
OBJECTIVE
Supratentorial extraventricular ependymomas are relatively rare. Long-term outcomes and prognostic factor for this rare tumor have not been well established. The purpose of this study was to demonstrateprogression-freesurvival(PFS),overallsurvival(OS), and prognostic factors of such tumor.
PATIENTS AND METHODS
Fifty-five patients with supratentorial extraventricular ependymomas from our hospital were included in this study. Epidemiological characteristics, clinical features, treatment,long-term outcomes, and prognostic factors for PFS and OS were reviewed retrospectively.
RESULTS
The patients consisted of 30 males and 25 females with mean age of 30.0 ± 23.6 years (range, 1-74 years). Twenty-nine tumors were located in the right hemisphere, and 26 in the left side. The 2 most common tumor locations were the frontal (n = 19; 35%) and parietal lobe (n = 11; 20%). All patients underwent surgical resection. Gross-total resection (GTR) was achieved in 42 cases (76%) and subtotal resection (STR) was performed in 13 patients (24%). According to the WHO classification system, 38 tumors (69%) were Grade III (anaplastic ependymoma), and 17 (31%) were Grade II (ependymoma). Three-,5-, and 10 year PFS rates were 60%, 49%, and 36%, respectively. Three-,5-, and 10 year OS rates were 79%, 64%, and 49%, respectively. EOR and tumor grade were identified as prognostic factors for PFS and OS on univariate analysis, multivariate analysis, and Kaplan-Meierlog-rank testing. Subtotal resection (STR) predicted a worse PFS (HR = 4.808; 95%, 1.942-11.905; P = .001) and OS (HR = 5.650; 95%, 2.114-15.152; P = .001). WHO Grade III tumors also had worse PFS (HR = 3.922; 95%, 1.429-18.182; P = .012) and OS (HR = 6.329; 95%, 1.328-30.303; P = 0.021). For patients with tumor recurrence, reoperation was significant prognostic factors for OS (HR = 2.091; 95%, 0.939-4.654; p = .000). Age, sex, tumor side, and postoperativeradiotherapy were not prognostic factors for PFS and OS.
CONCLUSIONS
Most supratentorial extraventricular ependymomas are WHO grade III tumors. STRandWHO Grade III pathology predicted worse PFS and OS. Gross-total resection remains the optimal treatment for patients with supratentorial extraventricular ependymoma. Reoperation should be considered first in cases of recurrence. The role of postoperative radiotherapy as an adjuvant treatment for supratentorial extraventricular ependymoma needs further investigation.
Topics: Adolescent; Adult; Aged; Brain Neoplasms; Child; Child, Preschool; Ependymoma; Female; Humans; Infant; Male; Middle Aged; Neoplasm Recurrence, Local; Neurosurgical Procedures; Prognosis; Retrospective Studies; Supratentorial Neoplasms; Time; Young Adult
PubMed: 29253745
DOI: 10.1016/j.clineuro.2017.12.013 -
Journal of the American Medical... Mar 1948
Topics: Brain; Brain Diseases; Brain Neoplasms; Humans; Supratentorial Neoplasms
PubMed: 18900426
DOI: 10.1001/jama.1948.72890270004008 -
Current Opinion in Neurology Dec 2019The purpose of this review is to discuss how a new treatment modality, tumor treating fields, may be incorporated into the oncologic care for patients with glioblastoma. (Review)
Review
PURPOSE OF REVIEW
The purpose of this review is to discuss how a new treatment modality, tumor treating fields, may be incorporated into the oncologic care for patients with glioblastoma.
RECENT FINDINGS
Tumor treating fields are a new treatment modality available to patients with newly diagnosed and recurrent glioblastoma. Alternating electric fields are delivered via a wearable, removable device affixed to the scalp of patients with supratentorial glioblastoma. With continuous use, the application of tumor treating fields combined with temozolomide chemotherapy has been shown to improve overall survival compared with temozolomide alone in patients with newly diagnosed glioblastoma. Adverse events attributable to the device are limited to localized skin reactions. Despite compendium guidelines in support of its use and Food and Drug Administration (FDA) approval, tumor treating fields have been slow to be adopted in the neuro-oncology community. Critics have raised concerns about the generalizability of the study data, patient quality of life, and mechanism of action of this therapy.
SUMMARY
Tumor treating fields are available for the treatment of both newly diagnosed and recurrent glioblastoma and represent a new category of treatment modalities in oncologic therapy. This novel device has received FDA approval but has been slow to be adopted into clinical practice.
Topics: Electric Stimulation Therapy; Glioblastoma; Humans; Neoplasm Recurrence, Local; Supratentorial Neoplasms
PubMed: 31609738
DOI: 10.1097/WCO.0000000000000762