scholarly journals PATZ1 Is Overexpressed in Pediatric Glial Tumors and Correlates with Worse Event-Free Survival in High-grade Gliomas

Cancers ◽  
2019 ◽  
Vol 11 (10) ◽  
pp. 1537
Author(s):  
Annalisa Passariello ◽  
Maria Elena Errico ◽  
Vittoria Donofrio ◽  
Manuela Maestrini ◽  
Alia Zerbato ◽  
...  
Keyword(s):  
Mrna Level ◽  
Young Patients ◽  
Molecular Heterogeneity ◽  
Low Grade ◽  
Normal Brain ◽  
High Grade ◽  
Event Free Survival ◽  
Free Survival ◽  
Glial Tumors ◽  
High Grade Gliomas

Glial tumors are the leading cause of cancer-related death and morbidity in children. Their diagnosis, mainly based on clinical and histopathological factors, is particularly challenging because of their high molecular heterogeneity. Thus, tumors with identical histotypes could result in variable biological behaviors and prognoses. The PATZ1 gene has been recently shown to be expressed in adult gliomas, including glioblastomas, where it correlates with the proneural subtype and with a better prognosis. Here, we analyzed the expression of PATZ1 in pediatric gliomas, first at mRNA level in a public database, and then at protein level, by immunohistochemistry, in a cohort of 52 glial brain tumors from young patients aged from 6 months to 16 years. As for adult tumors, we show that PATZ1 is enriched in glial tumors compared to the normal brain, where it correlates positively and negatively with a proneural and mesenchymal signature, respectively. Moreover, we show that PATZ1 is expressed at variable levels in our cohort of tumors. Higher expression was detected in high-grade than low-grade gliomas, suggesting a correlation with the malignancy. Among high-grade gliomas, higher levels of PATZ1 have consistently been found to correlate with worse event-free survival. Therefore, our study may imply new diagnostic opportunities for pediatric gliomas.

scholarly journals NIMG-42. HOROS SOFTWARE FOR POSTOPERATIVE ANALYSIS OF PEDIATRIC HIGH-GRADE GLIOMAS

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii157-ii157
Author(s):  
Yasmeen Elsawaf ◽  
Stephanie Anetsberger ◽  
Ana Aguilar-Bonilla ◽  
Amy Smith ◽  
Samer Elbabaa
Keyword(s):  
Pediatric Patients ◽  
Tumor Volume ◽  
Tumor Resection ◽  
Region Of Interest ◽  
Extent Of Resection ◽  
High Grade ◽  
Residual Volume ◽  
Event Free Survival ◽  
Free Survival ◽  
High Grade Gliomas

Abstract INTRODUCTION Horos (LGPL 3.0) is a free, open source medical image viewer that has gained attention in the neurosurgical community because of the familiar OsiriX-based interface and its useful three-dimensional (3D) volumetric rendering capabilities. We present the use of Horos software as a postoperative tool for residual tumor volume analysis in children with high-grade gliomas. METHODS A retrospective study of 11 pediatric patients with histologically confirmed HGG underwent tumor resection (n=8) or biopsy (n=3) as definitive treatment from 6/2011 to 6/2019. Volumetric data and extent of resection were obtained via region of interest-based 3D analysis using Horos image-processing software. Age, initial tumor volume, extent of resection, and postoperative residual volume were assessed as predictors of overall survival or event free survival. TECHNIQUE Region of interest (ROI) segmentation was performed utilizing the “Closed Polygon Tool” to outline the tumor and the “Generate Missing ROIs” function to capture the entirety of the tumor within the MRI series. The “Computer Volume” function was used to render the 3D tumor volumes. The preoperative and postoperative tumor volumes were compared per patient to yield the percent extent of resection and residual volume. RESULTS The Horos software is a highly effective means of volumetric analysis for high-grade gliomas depicted in T1 and T2 MRI series. In our series, eight (73%) patients underwent tumor resection and three (27%) underwent biopsy. Patients who underwent resection were older than biopsy patients [12 (8-18) vs. 9 (8-21) years old]. Age, initial tumor volume, extent of resection, and postoperative residual volume were not significant predictors of overall survival or event free survival. CONCLUSION Horos software provides increased accuracy and confidence in determining post-operative volume and is useful in assessing the impact of residual volume on outcome after maximal safe resection in pediatric patients with high-grade gliomas.

The Added Value of Inflow-Based Vascular-Space-Occupancy and Diffusion-Weighted Imaging in Preoperative Grading of Gliomas

2021 ◽  
pp. 1-8
Author(s):  
Haimei Cao ◽  
Xiang Xiao ◽  
Jun Hua ◽  
Guanglong Huang ◽  
Wenle He ◽  
...  
Keyword(s):  
Diagnostic Accuracy ◽  
Roc Curve ◽  
Diffusion Weighted Imaging ◽  
Low Grade ◽  
High Grade ◽  
Mr Images ◽  
Vascular Space ◽  
Diffusion Weighted ◽  
High Grade Gliomas ◽  
And Diffusion

Objectives: The present study aimed to study whether combined inflow-based vascular-space-occupancy (iVASO) MR imaging (MRI) and diffusion-weighted imaging (DWI) improve the diagnostic accuracy in the preoperative grading of gliomas. Methods: Fifty-one patients with histopathologically confirmed diffuse gliomas underwent preoperative structural MRI, iVASO, and DWI. We performed 2 qualitative consensus reviews: (1) structural MR images alone and (2) structural MR images with iVASO and DWI. Relative arteriolar cerebral blood volume (rCBVa) and minimum apparent diffusion coefficient (mADC) were compared between low-grade and high-grade gliomas. Receiver operating characteristic (ROC) curve analysis was performed to compare the tumor grading efficiency of rCBVa, mADC, and the combination of the two parameters. Results: Two observers diagnosed accurate tumor grade in 40 of 51 (78.4%) patients in the first review and in 46 of 51 (90.2%) in the second review. Both rCBVa and mADC showed significant differences between low-grade and high-grade gliomas. ROC analysis gave a threshold value of 1.52 for rCBVa and 0.85 × 10−3 mm2/s for mADC to provide a sensitivity and specificity of 88.0 and 81.2% and 100.0 and 68.7%, respectively. The area under the ROC curve (AUC) was 0.87 and 0.85 for rCBVa and mADC, respectively. The combination of rCBVa and mADC values increased the AUC to 0.92. Conclusion: The combined application of iVASO and DWI may improve the diagnostic accuracy of glioma grading.

scholarly journals Validation of a clinicopathological score for the prediction of post-surgical evolution of pituitary adenoma: retrospective analysis on 566 patients from a tertiary care centre

2019 ◽  
Vol 180 (2) ◽  
pp. 127-134 ◽  
Author(s):  
S Asioli ◽  
A Righi ◽  
M Iommi ◽  
C Baldovini ◽  
F Ambrosi ◽  
...  
Keyword(s):  
Pituitary Adenomas ◽  
Proliferative Activity ◽  
Disease Free Survival ◽  
Low Grade ◽  
High Grade ◽  
Tumour Type ◽  
Disease Evolution ◽  
Free Survival ◽  
Disease Free

Objective and design A clinicopathological score has been proposed by Trouillas et al. to predict the evolution of pituitary adenomas. Aim of our study was to perform an independent external validation of this score and identify other potential predictor of post-surgical outcome. Methods The study sample included 566 patients with pituitary adenomas, specifically 253 FSH/LH-secreting, 147 GH-secreting, 85 PRL-secreting, 72 ACTH-secreting and 9 TSH-secreting tumours with at least 3-year post-surgical follow-up. Results In 437 cases, pituitary adenomas were non-invasive, with low (grade 1a: 378 cases) or high (grade 1b: 59 cases) proliferative activity. In 129 cases, tumours were invasive, with low (grade 2a: 87 cases) or high (grade 2b: 42 cases) proliferative activity. During the follow-up (mean: 5.8 years), 60 patients developed disease recurrence or progression, with a total of 130 patients with pituitary disease at last follow-up. Univariate analysis demonstrated a significantly higher risk of disease persistence and recurrence/progression in patients with PRL-, ACTH- and FSH/LH-secreting tumours as compared to those with somatotroph tumours, and in those with high proliferative activity (grade 1b and 2b) or >1 cm diameter. Multivariate analysis confirmed tumour type and grade to be independent predictors of disease-free-survival. Tumour invasion, Ki-67 and tumour type were the only independent prognostic factors of disease-free survival. Conclusions Our data confirmed the validity of Trouillas’ score, being tumour type and grade independent predictors of disease evolution. Therefore, we recommend to always consider both features, together with tumour histological subtype, in the clinical setting to early identify patients at higher risk of recurrence.

scholarly journals Everolimus and Bevacizumab in the Management of Recurrent, Progressive Intracranial NF2 Mutated Meningioma

2019 ◽  
Vol 12 (1) ◽  
pp. 126-130 ◽  
Author(s):  
Vinay Mathew Thomas ◽  
Poorva Bindal ◽  
James J. Vredenburgh
Keyword(s):  
Survival Time ◽  
Progression Free Survival ◽  
Genetic Alteration ◽  
Cns Tumors ◽  
Good Effect ◽  
Genomic Sequencing ◽  
Low Grade ◽  
High Grade ◽  
Free Survival

Meningiomas are primary CNS tumors that arise from the arachnoid layer of the meninges. Genomic sequencing has revealed that NF2 mutations are the most common genetic alteration seen in meningiomas. Meningiomas although usually low grade, can sometimes progress to high grade. A patient who had several recurrences of meningiomas since childhood presented with recurrent headaches. Imaging showed that he had another recurrence of a meningioma. He underwent surgery for resection of the meningioma and histopathology showed NF2 mutation. He was started on everolimus and bevacizumab with good effect. Studies have shown that NF-2 mutated meningiomas have a good response to everolimus and bevacizumab with increased progression-free survival time and progression-free survival time at 6 months.

scholarly journals Urothelial Tumors of the Urinary Bladder in Young Patients: A Clinicopathologic Study of 59 Cases

2013 ◽  
Vol 137 (10) ◽  
pp. 1337-1341 ◽  
Author(s):  
Melissa L. Stanton ◽  
Li Xiao ◽  
Bogdan A. Czerniak ◽  
Charles C. Guo
Keyword(s):  
Urinary Bladder ◽  
Clinical Outcomes ◽  
Young Patients ◽  
Muscularis Propria ◽  
Low Grade ◽  
High Grade ◽  
Urothelial Carcinomas ◽  
Pathologic Features ◽  
Urothelial Tumors

Context.—Urothelial tumors are rare in young patients. Because of their rarity, the natural history of the disease in young patients remains poorly understood. Objective.—To understand the pathologic and clinical features of urothelial tumors of the urinary bladder in young patients. Design.—We identified 59 young patients with urothelial tumors of the urinary bladder treated at our institution and analyzed the tumors' pathologic features and the patients' clinical outcomes. Results.—All patients were 30 years or younger, with a mean age of 23.5 years (range, 4–30). Thirty-eight patients (64%) were male, and 21 (36%) were female. Most tumors were noninvasive, papillary urothelial tumors (49 of 59; 83%), including papillary urothelial neoplasms of low malignant potential (7 of 49; 14%), low-grade papillary urothelial carcinomas (38 of 49; 78%), and high-grade papillary urothelial carcinomas (4 of 49; 8%). Only a few (n = 10) of the urothelial tumors were invasive, invading the lamina propria (n = 5; 50%), muscularis propria (n = 4; 40%), or perivesical soft tissue (n = 1; 10%). Clinical follow-up information was available for 41 patients (69%), with a mean follow-up time of 77 months. Of 31 patients with noninvasive papillary urothelial tumors, only 1 patient (3%) later developed an invasive urothelial carcinoma and died of the disease, and 30 of these patients (97%) were alive at the end of follow-up, although 10 (32%) had local tumor recurrences. In the 10 patients with invasive urothelial carcinomas, 3 patients (30%) died of the disease and 5 others (50%) were alive with metastases (the other 2 [20%] were alive with no recurrence). Conclusion.—Urothelial tumors in young patients are mostly noninvasive, papillary carcinomas and have an excellent prognosis; however, a small subset of patients may present with high-grade invasive urothelial carcinomas that result in poor clinical outcomes.

scholarly journals Low-Grade Gemistocytic Morphology in H3 G34R-Mutant Gliomas and Concurrent K27M Mutation: Clinicopathologic Findings

2020 ◽  
Vol 79 (10) ◽  
pp. 1038-1043
Author(s):  
Meaghan Morris ◽  
Meghan Driscoll ◽  
John W Henson ◽  
Charles Cobbs ◽  
LiQun Jiang ◽  
...  
Keyword(s):  
Histone H3 ◽  
Low Grade ◽  
High Grade ◽  
K27m Mutation ◽  
Gemistocytic Astrocytoma ◽  
First Case ◽  
Clinicopathologic Findings ◽  
Tumor Phenotype ◽  
High Grade Gliomas ◽  
H3 K27m Mutations

Abstract Mutations in histone H3 are key molecular drivers of pediatric and young adult high-grade gliomas. Histone H3 G34R mutations occur in hemispheric high-grade gliomas and H3 K27M mutations occur in aggressive, though histologically diverse, midline gliomas. Here, we report 2 rare cases of histologically low-grade gliomas with gemistocytic morphology and sequencing-confirmed histone H3 G34R mutations. One case is a histologically low-grade gemistocytic astrocytoma with a G34R-mutation in H3F3A. The second case is a histologically low-grade gemistocytic astrocytoma with co-occurring K27M and G34R mutations in HIST1H3B. Review of prior histone H3-mutant gliomas sequenced at our institution shows a divergent clinical and immunohistochemical pattern in the 2 cases. The first case is similar to prior histone H3 G34R-mutant tumors, while the second case most closely resembles prior histone H3 K27M-mutant gliomas. These represent novel cases of sequencing-confirmed histone H3 G34R-mutant gliomas with low-grade histology and add to the known rare cases of G34R-mutant tumors with gemistocytic morphology. Although K27M and G34R mutations are thought to be mutually exclusive, we document combined K27M and G34R mutations in HIST1H3B and present evidence suggesting the K27M-mutation drove tumor phenotype in this dual mutant glioma.

scholarly journals Progression-free survival: An important end point in evaluating therapy for recurrent high-grade gliomas

2008 ◽  
Vol 10 (2) ◽  
pp. 162-170 ◽  
Author(s):  
Kathleen R. Lamborn ◽  
W. K. Alfred Yung ◽  
Susan M. Chang ◽  
Patrick Y. Wen ◽  
Timothy F. Cloughesy ◽  
...  
Keyword(s):  
Progression Free Survival ◽  
High Grade ◽  
Free Survival ◽  
End Point ◽  
High Grade Gliomas

scholarly journals Assessment of diffusion tensor imaging metrics in differentiating low-grade from high-grade gliomas

2016 ◽  
Vol 29 (5) ◽  
pp. 400-407 ◽  
Author(s):  
Lamiaa El-Serougy ◽  
Ahmed Abdel Khalek Abdel Razek ◽  
Amani Ezzat ◽  
Hany Eldawoody ◽  
Ahmad El-Morsy
Keyword(s):  
Diffusion Tensor Imaging ◽  
Diffusion Tensor ◽  
Low Grade ◽  
High Grade ◽  
High Grade Gliomas

scholarly journals The Identification of Prognostic Factors and Survival Statistics of Conventional Central Chondrosarcoma

Sarcoma ◽  
2015 ◽  
Vol 2015 ◽  
pp. 1-11 ◽  
Author(s):  
Sjoerd P. F. T. Nota ◽  
Yvonne Braun ◽  
Joseph H. Schwab ◽  
C. Niek van Dijk ◽  
Jos A. M. Bramer
Keyword(s):  
Prognostic Factors ◽  
Survival Rates ◽  
Disease Free Survival ◽  
Selection Procedure ◽  
Tumor Location ◽  
Low Grade ◽  
High Grade ◽  
Dedifferentiated Chondrosarcoma ◽  
Free Survival ◽  
Disease Free

Introduction. Chondrosarcomas are malignant bone tumors that are characterized by the production of chondroid tissue. Since radiation therapy and chemotherapy have limited effect on chondrosarcoma, treatment of most patients depends on surgical resection. We conducted this study to identify independent predictive factors and survival characteristics for conventional central chondrosarcoma and dedifferentiated central chondrosarcoma.Methods. A systematic literature review was performed in September 2014 using the Pubmed, Embase, and Cochrane databases. Subsequent to a beforehand-composed selection procedure we included 13 studies, comprising a total of 1114 patients.Results. The prognosis of central chondrosarcoma is generally good for the histologically low-grade tumors. Prognosis for the high-grade chondrosarcoma and the dedifferentiated chondrosarcoma is poor with lower survival rates. Poor prognostic factors in conventional chondrosarcoma for overall survival are high-grade tumors and axial/pelvic tumor location. In dedifferentiated chondrosarcoma the percentage of dedifferentiated component has significant influence on disease-free survival.Conclusion. Despite the fact that there are multiple prognostic factors identified, as shown in this study, there is a need for prospective and comparative studies. The resulting knowledge about prognostic factors and survival can give direction in the development of better therapies. This could eventually lead to an evidence-based foundation for treating chondrosarcoma patients.

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