Citations 2
Molecular, histologic, and clinical characterization of methylation class pleomorphic xanthoastrocytoma: An analysis of 469 tumors
Dampier, Christopher H. , Shah, Niharika, Neto, Osório Lopes Abath , Abdullaev, Zied , Alexandrescu, Sanda , Armstrong, Terri, Baker, Tiffany , Cathcart, Sahara , Chung, Hye‐Jung, Cimino, Patrick J. , Conway, Kyle , Dazelle, Karen , Etminam, Nima, Fernandes, Igor, Ferrone, Christina K. , Gilani, Ahmed , Gilbert, Mark, Gregory, Jason V. , Lee, Han Sung, Lopes, Maria Beatriz, Neill, Stewart G. , Ozer, Byram H. , Paulus, Werner, Penas‐Prado, Marta, Prinz, Marco , Pytel, Peter , Quezado, Martha , Raffeld, Mark , Rajan, Sharika, Reifenberger, Guido, Robinson, Lorraina , Schittenhelm, Jens , Thomas-Ogunniyi, Jaiyeola , Toland, Angus , Turakulov, Rust , Aldape, Kenneth , Cimino, Patrick J , Mao, Qinwen , Prinz, Marco , Ratliff, Miriam , Robinson, Lorraina , Schittenhelm, Jens , Thomas, Christian , Vaubel, Rachael A , Wu, Jing , Giannini, Caterina , Brandner, Sebastian , von Deimling, Andreas , Aldape, Kenneth , Dampier, Christopher H , Galbraith, Kristyn , Ebrahimi, Azadeh , Andreiuolo, Felipe , Cotter, Jennifer A. , Costa, Felipe D’Almeida , Ferman, Sima , Fernandes, Igor, Gregory, Jason, Ketchum, Courtney , Lee, Ina , Lopes, M. Beatriz S. , Mao, Qinwen , Marshall, Michael S. , McCord, Matthew , Nirschl, Jeffrey , Peñas-Prado, Marta , Schrimpf, Daniel , Singh, Omkar , Thomas, Christian , Thomas, Diana , Vaubel, Rachael A. , Wadhwani, Nitin R. , Giannini, Caterina , Snuderl, Matija , Deimling, Andreas von
Neuro-Oncology Advances | July 21, 2025 | Volume: 7 | Issue: 1 | Pages: No page available
Abstract Background Methylation class pleomorphic xanthoastrocytoma (mcPXA) comprises tumors with the DNA methylation signature of classical PXA but with a wider histologic spectrum, including overlap with glioblastoma (GBM). Methods To clarify the histologic and molecular scope of mcPXA and characterize its clinical behavior, a cohort of 469 tumor samples from 458 patients matching to mcPXA by the DKFZ classifier (v12.6 score ≥0.85) was interrogated. Results Patient median age was 23 years (range 1-73 years) with a female predominance (259 female/199 male). CDKN2A/B homozygous deletion was observed in 406 of 469 (87%) samples. In samples tested for BRAF p.V600E mutations (n = 279), 240 (86%) harbored the mutation. A chr7+/chr10− pattern was observed in 103 of 469 (22%) samples. Among samples tested for TERT promoter mutations (n = 143), 32 (22%) harbored the mutation. Progression-free and overall survival of patients with mcPXA were comparable to patients with methylation class IDH-mutant astrocytoma, low grade, but a GBM-like subset (ie, cases with a pre-methylation working diagnosis of GBM) showed shorter survival. Histologic features of high grade, including palisading necrosis and microvascular proliferation, were prognostic in mcPXA. Compared to patients with BRAF p.V600E-altered GBM, patients with mcPXA were younger and had a lower frequency of TERT promoter mutations. Conclusion Tumors in mcPXA share molecular characteristics with histologically defined PXA, and high-grade histologic features can help predict their clinical behavior. The use of an epigenetic classification of PXA reveals that this group of tumors is more common than previously appreciated and warrants in-depth study to identify efficacious therapeutic options.
cIMPACT‐NOW update 6: new entity and diagnostic principle recommendations of the cIMPACT‐Utrecht meeting on future CNS tumor classification and grading
Louis, David N. , Wesseling, Pieter , Aldape, Kenneth , Capper, David , Cree, Ian A. , Figarella‐Branger, Dominique , Fouladi, Maryam, Haberler, Christine , Hawkins, Cynthia , Komori, Takashi, Ng, Ho‐Keung , Orr, Brent A. , Paulus, Werner, Pietsch, Torsten , Reifenberger, Guido , Rosenblum, Marc, Rous, Brian , Sahm, Felix , Sarkar, Chitra , Solomon, David A. , Tabori, Uri , Bent, Martin J. van den , Deimling, Andreas von , Weller, Michael , White, Valerie A. , Ellison, David W. , Figarella-Branger, Dominique , Pietsch, Torsten , Sarkar, Chitra , Solomon, David A , Weller, Michael , van den Bent, Martin J , Orr, Brent A , Wesseling, Pieter , von Deimling, Andreas , Tabori, Uri , Sahm, Felix , Capper, David , Brat, Daniel J. , Eberhart, Charles G. , Fouladi, Maryam , Fuller, Gregory N. , Giannini, Caterina , Kros, Johan M. , Park, Sung-Hye , Perry, Arie , Rosenblum, Marc
Brain Pathology | April 20, 2020 | Volume: 30 | Issue: 4 | Pages: 844-856
Abstract cIMPACT‐NOW (the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy) was established to evaluate and make practical recommendations on recent advances in the field of CNS tumor classification, particularly in light of the rapid progress in molecular insights into these neoplasms. For Round 2 of its deliberations, cIMPACT‐NOW Working Committee 3 was reconstituted and convened in Utrecht, The Netherlands, for a meeting designed to review putative new CNS tumor types in advance of any future World Health Organization meeting on CNS tumor classification. In preparatory activities for the meeting and at the actual meeting, a list of possible entities was assembled and each type and subtype debated. Working Committee 3 recommended that a substantial number of newly recognized types and subtypes should be considered for inclusion in future CNS tumor classifications. In addition, the group endorsed a number of principles—relating to classification categories, approaches to classification, nomenclature, and grading—that the group hopes will also inform the future classification of CNS neoplasms.