Anaplastic pleomorphic xanthoastrocytoma with leptomeningeal dissemination responsive to BRAF inhibition and bevacizumab

2018 ◽  
Vol 66 (1) ◽  
pp. e27465 ◽  
Author(s):  
Alissa A. Thomas ◽  
Suzanne M. Tucker ◽  
Carl J. Nelson ◽  
Joshua P. Nickerson ◽  
Susan R. Durham ◽  
...  
Keyword(s):  
Pleomorphic Xanthoastrocytoma ◽  
Leptomeningeal Dissemination ◽  
Braf Inhibition

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

Successful Targeting of an ATG7-RAF1 Gene Fusion in Anaplastic Pleomorphic Xanthoastrocytoma With Leptomeningeal Dissemination

2019 ◽  
pp. 1-7 ◽  
Author(s):  
Mehdi Touat ◽  
Nadia Younan ◽  
Philipp Euskirchen ◽  
Maxime Fontanilles ◽  
Karima Mokhtari ◽  
...  
Keyword(s):  
Gene Fusion ◽  
Pleomorphic Xanthoastrocytoma ◽  
Leptomeningeal Dissemination

Non-anaplastic pleomorphic xanthoastrocytoma with neuroradiological evidences of leptomeningeal dissemination

2005 ◽  
Vol 22 (6) ◽  
pp. 614-618 ◽  
Author(s):  
Eva Passone ◽  
Stefano Pizzolitto ◽  
Serena D'Agostini ◽  
Miran Skrap ◽  
Maria Paola Gardiman ◽  
...  
Keyword(s):  
Pleomorphic Xanthoastrocytoma ◽  
Leptomeningeal Dissemination

Response to BRAF/MEK Inhibition After Progression With BRAF Inhibition in a Patient With Anaplastic Pleomorphic Xanthoastrocytoma

2018 ◽  
Vol 23 (5) ◽  
pp. 163-166 ◽  
Author(s):  
Faiz Hussain ◽  
Craig M. Horbinski ◽  
Steven J. Chmura ◽  
Bakhtiar Yamini ◽  
Rimas V. Lukas
Keyword(s):  
Pleomorphic Xanthoastrocytoma ◽  
Mek Inhibition ◽  
Braf Inhibition

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.

Sign in / Sign up

Export Citation Format

Share Document