Daniel R Klinger, Rebecca L Dillon, Nathan F Clement, Francis J Cloran, Iren Horkayne-Szakaly
Background: Pleomorphic xanthoastrocytomas (PXAs) are uncommon intradural and typically intramedullary astrocytic central nervous system tumors. Although they commonly occur supratentorially, they are rarely seen in the spine. Case Description: A 43-year-old male presented with cervical neck pain and right-sided radicular symptoms. He was found to have an intradural extramedullary mass at the C5-C6 level. The lesion was fully excised and proved to be a PXA. Of interest, the lesion did not recur on postoperative MR imaging studies obtained 7 months later...
2020: Surgical Neurology International
Mansour Mathkour, Somdeb Banerjee, Cassidy Werner, Joshua Hanna, Hussam Abou-Al-Shaar, Rishawn Dindial, Tyler Scullen, Lauren Boehm, R Shane Tubbs, Marcus L Ware
BACKGROUND: Pleomorphic xanthoastrocytoma (PXA) is a rare brain tumor occurring supra- and infra-tentorially in both young adults and children. PXA is a benign tumor with a favorable prognosis. It is not traditionally considered as a neurofibromatosis type 1 (NF-1)-associated lesion, and its prognosis remains largely unknown, on the contrary to non-NF-1 PXA tumors. OBJECTIVE: Herein, we present a rare case of cerebellar PXA in a patient with NF-1 and performed systematic review of NF-1-associated PXA...
November 2, 2020: Clinical Neurology and Neurosurgery
Madhivanan Karthigeyan, Pankaj Kumar, Pravin Salunke, Manish Rohilla, Debjyoti Chatterjee, Chirag Kamal Ahuja
BACKGROUND: Pleomorphic xanthoastrocytoma (PXA), a relatively uncommon, World Health Organization grade II tumor of astrocytic origin, occurs in children and in general carries good prognosis. The recently identified anaplastic PXA (grade III) type shows poor outcome. Very rarely, the above said tumors can display an aggressive clinical course with features of leptomeningeal spread. Such cases are mostly seen in adults, with rare instances reported in pediatric age group. CASE DESCRIPTION: In this report, we describe an 8-year-old child with anaplastic PXA with a rapid downhill course due to cerebrospinal fluid (CSF) spread...
October 16, 2020: World Neurosurgery
Takahiro Ono, Toshio Sasajima, Hiroaki Shimizu, Manabu Natsumeda, Masayuki Kanamori, Kenichiro Asano, Takaaki Beppu, Kenichiro Matsuda, Masahiro Ichikawa, Yukihiko Fujii, Hiroki Ohkuma, Kuniaki Ogasawara, Yukihiko Sonoda, Kiyoshi Saito, Sumihito Nobusawa, Yoichi Nakazato, Chifumi Kitanaka, Takamasa Kayama, Teiji Tominaga
Pleomorphic xanthoastrocytoma (PXA) is a rare glial tumor, however, its histological differentiation from high-grade gliomas is often difficult. Molecular characteristics may contribute to a better diagnostic discrimination. Prognostic factors of PXA are also important but few relevant reports have been published. This study investigated the molecular features and prognostic factors of PXAs. Seven university hospitals participated in this study by providing retrospective clinical data and tumor samples of PXA cases between 1993 and 2014...
October 16, 2020: Neurologia Medico-chirurgica
Shuang-Lin Deng, Ri-Hua Jin, Yi-Ming Liu, Yi Jing, Yi Guan
RATIONALE: Pleomorphic xanthoastrocytoma (PXA) is a rare low-grade glial neoplasm of the central nervous system, which is difficult to distinguish from other neoplastic and non-neoplastic entities. Herein, we report 2 cases of PXA that had been misdiagnosed as an inflammatory granuloma. PATIENT CONCERNS: The first case was a 22-year-old man who originally presented with a generalized seizure 7 years previously. Magnetic resonance imaging (MRI) revealed a lesion in the right parietal lobe, leading to a diagnosis of inflammatory granuloma...
October 9, 2020: Medicine (Baltimore)
Antonio Dono, Jennifer Vu, Molly Anapolsky, Gabriella Hines, Takeshi Takayasu, Yuanqing Yan, Nitin Tandon, Jay-Jiguang Zhu, Meenakshi B Bhattacharjee, Yoshua Esquenazi, Leomar Y Ballester
INTRODUCTION: Recently, the term "Diffuse glioma, BRAF V600E-mutant" has been recommended for IDH-wildtype gliomas with BRAF p.V600E mutation and without CDKN2A/B deletion. However, additional alterations in gliomas that coexist with BRAF-mutations are poorly defined. METHODS: We analyzed next-generation sequencing results in 315 cancer-associated genes for 372 gliomas from our institution (2010 to 2017). In addition, we reviewed IDH-WT gliomas with mutation and copy-number alterations available in cBioPortal, to further characterize BRAF-mutant gliomas...
September 2020: Journal of Neuro-oncology
Modhi Alhussinan, Turki Elarjani, Mohammed Jawharri, Mohammed Albrahim, Faisal Farrash
Background: Idiopathic intracranial hypertension (IIH) and normal pressure hydrocephalus (NPH) are disorders of the cerebrospinal fluid (CSF) flow dynamics. Pleomorphic xanthoastrocytoma (PXA) is a rare low-grade astrocytoma (World Health Organization grade II) representing <1% of astrocytomas. Combination of IIH and NPH with PXA is unheard of, with few published cases discussing the association of CNS tumors with either IIH or NPH, but never combined. We present a case of a 51-year-old woman with such a rare combination...
2020: Case Reports in Surgery
M Hope Robinson, Juan Vasquez, Akhilesh Kaushal, Tobey J MacDonald, José E Velázquez Vega, Matthew Schniederjan, Kavita Dhodapkar
Brain tumors are the leading cause of cancer-related mortality in children and have distinct genomic and molecular features compared with adult glioma. However, the properties of immune cells in these tumors has been vastly understudied compared with their adult counterparts. We combined multiplex immunofluorescence immunohistochemistry coupled with machine learning and single-cell mass cytometry to evaluate T-cells infiltrating pediatric glial tumors. We show that low-grade tumors are characterized by greater T-cell density compared with high-grade glioma (HGG)...
August 2020: Journal for Immunotherapy of Cancer
Pierre Dal Col, Delphine Poncet, Romain Rivoirard, François Vassal, Emilie Bernichon, Claire Boutet, Michel Péoc'h, Fabien Forest
Pleomorphic xanthoastrocytoma (PXA) is classified as an astrocytic glioma occurring most often in children or young adults. Molecular alterations in PXA are not fully known, especially those associated with tumor progression. We describe a patient with several relapses of a PXA. The tumor showed an acquired ATRX loss through tumor evolution. We tested alternative lengthening of telomeres (ALT) with the C-circle test. While the test was negative in the first tumor, a high circle activity was detected in the last relapse, suggesting an acquired ALT phenotype...
August 8, 2020: Journal of Neuropathology and Experimental Neurology
Adam Kowalewski, Justyna Durślewicz, Marek Zdrenka, Dariusz Grzanka, Łukasz Szylberg
The possible application of BRAF-targeted therapy in brain tumors is growing continuously. We have analyzed clinical strategies that address BRAF activation in primary brain tumors and verified current recommendations regarding screening for BRAF mutations. There is preliminary evidence for a range of positive responses in certain brain tumor types harboring the BRAF V600E mutation. National Comprehensive Cancer Network Guidelines for central nervous system cancers recommend screening for the BRAF V600E mutation in pilocytic astrocytoma, pleomorphic xanthoastrocytoma, and ganglioglioma...
August 2020: Targeted Oncology
Rachael Vaubel, Valentina Zschernack, Quynh T Tran, Sarah Jenkins, Alissa Caron, Dragana Milosevic, James Smadbeck, George Vasmatzis, Daniela Kandels, Astrid Gnekow, Christof Kramm, Robert Jenkins, Benjamin R Kipp, Fausto J Rodriguez, Brent A Orr, Torsten Pietsch, Caterina Giannini
Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytoma predominantly affecting children and young adults. We performed comprehensive genomic characterization on a cohort of 67 patients with histologically defined PXA (n=53, 79%) or anaplastic PXA (A-PXA, n=14, 21%), including copy number analysis (ThermoFisher Oncoscan, n=67), methylation profiling (Illumina EPIC array, n=43), and targeted next generation sequencing (n=32). The most frequent alterations were CDKN2A/B deletion (n=63; 94%) and BRAF p.V600E (n=51, 76...
July 3, 2020: Brain Pathology
Joshua A Hanna, Mansour Mathkour, Edna E Gouveia, JonMark Lane, Lauren Boehm, Joseph R Keen, Erin E Biro, Olawale A Sulaiman, Cuong J Bui
Background: Pleomorphic xanthoastrocytoma (PXA) is a rare and often focal glioma that most commonly affects children and young adults. Lesions are preferentially supratentorial and superficial, although infratentorial masses have been described, along with occasional involvement of the leptomeninges. The World Health Organization (WHO) categorizes these tumors as grade II, with surgical resection carrying a favorable prognosis. However, these tumors may undergo malignant degeneration and must be identified for appropriate treatment and prognosis...
2020: Ochsner Journal
Adham M Khalafallah, Maureen Rakovec, Debraj Mukherjee
BACKGROUND: Pleomorphic Xanthoastrocytoma (PXA) is a low-grade central nervous system (CNS) tumor with a generally favorable prognosis. However, due to its rarity, optimal adjuvant treatment guidelines have not been established by large scale studies. In this study, we investigated the effect of adjuvant radiation therapy (RT) on overall survival (OS) in adult patients with PXA to help address this unanswered question. METHODS: The National Cancer Database (NCDB) was used to identify adult patients (age ≥ 18 years old) diagnosed with histologically confirmed grade II PXA (2004-2016)...
June 24, 2020: Clinical Neurology and Neurosurgery
Karen Tang, David Kurland, Varshini Vasudevaraja, Jonathan Serrano, Michael Delorenzo, Alireza Radmanesh, Cheddhi Thomas, Marissa Spino, Sharon Gardner, Jeffrey C Allen, Theodore Nicolaides, Diana S Osorio, Jonathan L Finlay, Daniel R Boué, Matija Snuderl
Pleomorphic xanthoastrocytoma (PXA) is a rare type of brain tumor that affects children and young adults. Molecular prognostic markers of PXAs remain poorly established. Similar to gangliogliomas, PXAs show prominent immune cell infiltrate, but its composition also remains unknown. In this study, we correlated DNA methylation and BRAF status with clinical outcome and explored the tumor microenvironment. We performed DNA methylation in 21 tumor samples from 18 subjects with a histological diagnosis of PXA. MethylCIBERSORT was used to deconvolute the PXA microenvironment by analyzing the associated immune cell-types...
June 28, 2020: Journal of Neuropathology and Experimental Neurology
Yuki Matsumoto, Mikiko Kobayashi, Kunihiko Shingu, Ayako Tateishi, Maki Ohya, Kenji Sano, Tatsuya Negishi, Shohei Shigeto, Tatsuya Kobayashi, Yosuke Hara, Yukinari Kakizawa, Hiroyuki Kanno
Pleomorphic xanthoastrocytomas (PXAs) are rare low-grade astrocytic tumors that typically present as superficial nodular cystic tumors of the cerebrum attached to the leptomeninx. Histologically, they are pleomorphic, hypercellular glial neoplasms. Despite the presence of microscopic pleomorphism, patients' postoperative prognosis is generally good. Anaplastic PXAs (APXAs) have a high mitotic index and patients with APXAs have a worse prognosis than patients with PXAs. Here, we report an autopsy case of APXA initially diagnosed as PXA...
June 23, 2020: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Iris Deitch-Harel, Eyal Raskin, Zohar Habot-Wilner, Ronit Friling, Radgonde Amer, Michal Kramer
PURPOSE: To report cases of uveitis induced by biological therapy given for cancer. METHODS: Retrospective review of medical charts of patients. RESULTS: Included were six patients aged 14-81 years. Three were treated with vemurafenib and one each with nivolumab, ipilimumab, and osimertinib. The oncological diagnoses were metastatic thyroid carcinoma, pleomorphic xanthoastrocytoma, metastatic melanoma, adenocarcinoma of the lung, and metastatic breast cancer...
June 16, 2020: Ocular Immunology and Inflammation
Wei Zhang, Junxi Chen, Gang Hua, Dan Zhu, Qinghua Tan, Liming Zhang, Genbo Wang, Meichao Ding, Xiangshu Hu, Hua Li, Hari Shanker Sharma, Qiang Guo
OBJECTIVE: To explore the strategy of surgical treatment of low-grade brain tumors associated with epilepsy. METHODS: Clinical data of 158 patients with low-grade brain tumors were collected from January 2011 to December 2017 in Guangdong Sanjiu brain hospital. All patients received Preoperative evaluation. Lesion site: 18 cases were located in multiple cerebral lobes, 10 cases were in the functional zones, 130 cases were in the non-functional zones (including 74 cases were in the medial of temporal lobe)...
2020: International Review of Neurobiology
Darius Tan, Leon T Lai, Christopher D Daly, Vu Tran, Julian Maingard, Craig Timms
Pleomorphic Xanthoastrocytoma (PXA) is an uncommon central nervous system neoplasm with an overall favourable survival prognosis. Metastatic spread of PXA to the spinal cord and the cauda equina is rare and may have a different clinicopathological course. Treatment and prognostic outcomes, in this context, is not well defined. We discussed a case of a 30-year-old patient with known cerebral PXA presenting with metastatic spinal anaplastic PXA and present a literature analysis of treatment outcomes.
May 19, 2020: World Neurosurgery
Sang Ho Kim, Kihwan Hwang, Kyu Sang Lee, Gheeyoung Choe, Chae-Yong Kim
BACKGROUND: Pleomorphic xanthoastrocytoma (PXA) is a rare type of astrocytoma with a peak incidence from 10 to 19 years of age. PXA is found most commonly in the supratentorium. When it is present in atypical areas like the infratentorium, it is often overlooked as a potential differential diagnosis. CASE DESCRIPTION: This report discusses the case of an 18-year-old girl with a pure PXA in the left cerebellum. Our case consists of interesting pathological and molecular features which do not align with the literature about PXA; a positive finding for BRAF V600E mutation and a negative finding for CD34 expression...
April 26, 2020: World Neurosurgery
Kohei Fukuoka, Yasin Mamatjan, Ruth Tatevossian, Michal Zapotocky, Scott Ryall, Ana Guerreiro Stucklin, Julie Bennett, Liana Figueiredo Nobre, Anthony Arnoldo, Betty Luu, Ji Wen, Kaicen Zhu, Alberto Leon, Dax Torti, Trevor J Pugh, Lili-Naz Hazrati, Normand Laperriere, James Drake, James T Rutka, Peter Dirks, Abhaya V Kulkarni, Michael D Taylor, Ute Bartels, Annie Huang, Gelareh Zadeh, Kenneth Aldape, Vijay Ramaswamy, Eric Bouffet, Matija Snuderl, David Ellison, Cynthia Hawkins, Uri Tabori
BACKGROUND: Both genetic and methylation analysis have been shown to provide insight into the diagnosis and prognosis of many brain tumors. However, the implication of methylation profiling and its interaction with genetic alterations in pediatric low-grade gliomas (PLGGs) are unclear. METHODS: We performed a comprehensive analysis of PLGG with long term clinical follow up. In total 152 PLGGs were analyzed from a range of pathological subtypes including 40 gangliogliomas...
April 3, 2020: Neuro-oncology
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