scholarly journals Pleomorphic xanthoastrocytoma

2021 ◽  
Author(s):  
Luis Camarillo
Keyword(s):  
Pleomorphic Xanthoastrocytoma

scholarly journals Predictors of outcome in pleomorphic xanthoastrocytoma

2020 ◽  
Author(s):  
Antonio Dono ◽  
Victor Lopez-Rivera ◽  
Ankush Chandra ◽  
Cole T Lewis ◽  
Rania Abdelkhaleq ◽  
...  
Keyword(s):  
Tumor Size ◽  
Postoperative Radiotherapy ◽  
Treatment Strategies ◽  
Pleomorphic Xanthoastrocytoma ◽  
Survival Outcomes ◽  
Factors Affecting ◽  
Cox Proportional Hazard ◽  
Kaplan Meier ◽  
Cox Proportional Hazard Models ◽  
Male Patients

Abstract Background Pleomorphic xanthoastrocytomas (PXA) are circumscribed gliomas that typically have a favorable prognosis. Limited studies have revealed factors affecting survival outcomes in PXA. Here, we analyzed the largest PXA dataset in the literature and identify factors associated with outcomes. Methods Using the Surveillance, Epidemiology, and End Results (SEER) 18 Registries database, we identified histologically confirmed PXA patients between 1994 and 2016. Overall survival (OS) was analyzed using Kaplan-Meier survival and multivariable Cox proportional hazard models. Results In total, 470 patients were diagnosed with PXA (males = 53%; median age = 23 years [14-39 years]), the majority were Caucasian (n = 367; 78%). The estimated mean OS was 193 months [95% CI: 179-206]. Multivariate analysis revealed that greater age at diagnosis (≥39 years) (3.78 [2.16-6.59], P < .0001), larger tumor size (≥30 mm) (1.97 [1.05-3.71], P = .034), and postoperative radiotherapy (RT) (2.20 [1.31-3.69], P = .003) were independent predictors of poor OS. Pediatric PXA patients had improved survival outcomes compared to their adult counterparts, in which chemotherapy (CT) was associated with worse OS. Meanwhile, in adults, females and patients with temporal lobe tumors had an improved survival; conversely, tumor size ≥30 mm and postoperative RT were associated with poor OS. Conclusions In PXA, older age and larger tumor size at diagnosis are risk factors for poor OS, while pediatric patients have remarkably improved survival. Postoperative RT and CT appear to be ineffective treatment strategies while achieving GTR confer an improved survival in male patients and remains the cornerstone of treatment. These findings can help optimize PXA treatment while minimizing side effects. However, further studies of PXAs with molecular characterization are needed.

scholarly journals A Case of Pleomorphic Xanthoastrocytoma with Intracranial Hemorrhage in a Child

2019 ◽  
Vol 6 (1) ◽  
pp. 39-42
Author(s):  
Yusuke Takamine ◽  
Shun Yamamuro ◽  
Koichiro Sumi ◽  
Takashi Ohta ◽  
Katsunori Shijo ◽  
...  
Keyword(s):  
Intracranial Hemorrhage ◽  
Pleomorphic Xanthoastrocytoma

scholarly journals HGG-02. ADOLESCENT AND YOUNG ADULT (AYA) GLIOMA WITH BRAF V600E-MUTANTATION

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii344-iii344
Author(s):  
Yui Kimura ◽  
Yukitomo Ishi ◽  
Yuko Watanabe ◽  
Yoshiko Nakano ◽  
Shigeru Yamaguchi ◽  
...  
Keyword(s):  
Braf Inhibitor ◽  
Clinical Information ◽  
Early Recurrence ◽  
Pleomorphic Xanthoastrocytoma ◽  
Adolescent And Young Adult ◽  
Braf V600e ◽  
Low Grade ◽  
Diffuse Astrocytoma ◽  
Genetic Features ◽  
Epithelioid Glioblastoma

Abstract BACKGROUND Biological features of pediatric glioma differ significantly from those of adult glioma, and limited data are available on those of AYA patients. Here, we focused on AYA patients with glioma, especially those harboring BRAF V600E mutation, and investigated their clinical and genetic features. METHOD: We retrospectively analyzed AYA patients with brain tumors harboring BRAF V600E, who were treated in two hospitals in Japan. RESULTS Clinical information was available for 14 patients. The median age at diagnosis was 25 years (range: 15–38). Five patients were diagnosed with glioblastoma (GBM), including one epithelioid type. These patients were over 25. Although one patient with GBM died of the disease 6.9 years after initial diagnosis, the remaining patients were alive. Two patients were alive without recurrence at 38 and 51 months after the treatment. The patient with epithelioid glioblastoma experienced early recurrence. The remaining nine patients (64%) were diagnosed with low-grade glioma, including ganglioglioma, pilocytic astrocytoma, diffuse astrocytoma, oligodendroglioma, pleomorphic xanthoastrocytoma, and polymorphous low-grade neuroepithelial tumor of the young. No patients died of the disease, and four patients are alive without recurrence after initial operation without adjuvant treatment. Two patients are (epithelioid glioblastoma and ganglioglioma) currently undergoing treatment with a BRAF inhibitor for recurrent tumors. DISCUSSION Although the number of this study is limited, our study suggested that the prognosis of AYA patients with BRAF-V600E positive GBM may not be as dismal as that of children or adults.

scholarly journals RARE-30. ANAPLASTIC PLEOMORPHIC XANTHOASTROCYTOMA WITH LEPTOMENINGEAL DISSEMINATION RESPONSIVE TO BRAF INHIBITION AND BEVACIZUMAB

2017 ◽  
Vol 19 (suppl_6) ◽  
pp. vi216-vi216 ◽  
Author(s):  
Alissa Thomas ◽  
Suzanne Tucker ◽  
Carl Nelson ◽  
Joshua Nickerson ◽  
Susan Durham ◽  
...  
Keyword(s):  
Pleomorphic Xanthoastrocytoma ◽  
Leptomeningeal Dissemination ◽  
Braf Inhibition

Malignant transformation in pleomorphic xanthoastrocytoma–cfa report of two cases

1999 ◽  
Vol 13 (5) ◽  
pp. 516-519 ◽  
Author(s):  
A. Chakrabarty ◽  
P. Mitchell ◽  
L. R. Bridges ◽  
A. J. Franks
Keyword(s):  
Malignant Transformation ◽  
Pleomorphic Xanthoastrocytoma

scholarly journals PATH-13. PLEOMORPHIC XANTHOASTROCYTOMA INTEGRATED GENOMIC CHARACTERIZATION - WHAT HAVE WE LEARNED?

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii426-iii427
Author(s):  
Rachael Vaubel ◽  
Valentina Zschernack ◽  
Alissa Caron ◽  
Dragana Milosevic ◽  
Robert Jenkins ◽  
...  
Keyword(s):  
Overall Survival ◽  
Strong Predictor ◽  
Progression Free Survival ◽  
Genetic Alterations ◽  
Pleomorphic Xanthoastrocytoma ◽  
Who Grade ◽  
Targeted Next Generation Sequencing ◽  
Genomic Characterization ◽  
Promoter Mutations ◽  
Methylation Profiling

Abstract Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytoma occurring predominantly in children and young adults. It is characterized histologically by large pleomorphic, spindled and lipidized cells with frequent eosinophilic granular bodies and pericellular reticulin deposition. BRAF p.V600E mutation and CKDN2A/B deletion are the most common genetic alterations. We report the integrated genomic characterization of a cohort of 67 patients (37 F, 30 M; median age 20.3 years (interquartile 13.4–32.9) with histologically defined PXA (52, 78%) or anaplastic PXA (A-PXA) (15, 22%), using genome-wide cytogenetic (ThermoFisher Oncoscan, n=67), methylation profiling (Illumina EPIC array, n=43), and targeted next generation sequencing (n=32). BRAF p.V600E mutation (n=51, 76.1%) and CDKN2A/B deletion (n=63; 94%) were the most frequent alterations. Of 16 BRAF p.V600E negative cases, 7 showed an alternative BRAF activating mutation (n=2), NF1 (n=3) mutation or ATG7-RAF1 fusion (n=2). Targeted TERT analysis found promoter mutations in 3 (of 58) cases, but TERT amplification was absent. Supervised and unsupervised methylation profiling against a comprehensive reference cohort demonstrated consensus grouping with the PXA class in 36 of 43 cases; while the minority grouped with a ganglioglioma class (n=3), with reactive brain or had no resolvable subgroup (n=4). Follow-up was available in 61 patients (91.0%) (median 63 months). Overall survival was significantly different between PXA and A-PXA (5-year:80.4% vs. 55.1%; p=0.001), but not progression-free survival (5-year:61.7% vs. 39.8%; p=0.128). Our data confirm the high frequency of MAP-K abnormalities and CDKN2A/B deletion in PXA. WHO grade remains a strong predictor of patient overall survival.

scholarly journals Intra-operative squash cytodiagnosis of pleomorphic xanthoastrocytoma : A diagnostic challenge

2017 ◽  
Vol 4 (4) ◽  
pp. 279-283
Author(s):  
Dr. Rachana Binayke ◽  
◽  
Dr. Shubhangi Vinayak Agale ◽  
Dr. Geeta Kumari ◽  
Dr. Grace. F. D'Costa ◽  
...  
Keyword(s):  
Pleomorphic Xanthoastrocytoma ◽  
Diagnostic Challenge

scholarly journals Pleomorphic xanthoastrocytoma at the pineal region: rare case report with successful surgical management

2021 ◽  
Vol 36 (1) ◽  
Author(s):  
Saraj Kumar Singh ◽  
Krishan Kumar Sharma ◽  
Tarun Kumar
Keyword(s):  
Surgical Management ◽  
Rare Case ◽  
Case Reports ◽  
Internal Capsule ◽  
Pleomorphic Xanthoastrocytoma ◽  
Pineal Region ◽  
Low Grade ◽  
Pineal Region Tumors ◽  
Pediatric Age Group ◽  
Rare Case Report

Abstract Background Pineal region tumors are commonly present in the pediatric age group. However, pleomorphic xanthoastrocytoma (PXA) is very rare at this region, and only few case reports have been reported till now in literature. Case presentation Here, we report a rare case of pineal region, juxta-thalamic, pleomorphic xanthoastrocytoma (PXA) in an 11-year-old male child. The child presented with severe headache after which MRI was done. It was suggestive of pineal region low-grade tumor. The patient was operated in Parkbench position with SCIT (supracerebellar approach) in a retractor-free manner. Gross total resection was done. However, the patient developed postoperative left-sided hemiparesis. It got improved in 1 month, and the patient became ambulatory. Histopathology came out as pleomorphic xanthoastrocytoma. Conclusion Surgical management should include careful resection near the internal capsule to avoid postoperative hemiparesis. Also, shunt should be delayed in the cystic cavity created by resection of tumor.

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