Survival of diffuse astrocytic glioma, IDH1/2 wildtype, with molecular features of glioblastoma, WHO grade IV: a confirmation of the cIMPACT-NOW criteria

• Published in: Neuro-oncology (Charlottesville, Va.) Va.), 2020-04, Vol.22 (4), p.515-523
• Main Authors: Tesileanu, C Mircea S, Dirven, Linda, Wijnenga, Maarten M J, Koekkoek, Johan A F, Vincent, Arnaud J P E, Dubbink, Hendrikus J, Atmodimedjo, Peggy N, Kros, Johan M, van Duinen, Sjoerd G, Smits, Marion, Taphoorn, Martin J B, French, Pim J, van den Bent, Martin J
• Format: Article
• Language: English
• Subjects: Astrocytoma - genetics; Brain Neoplasms - genetics; Clinical Investigations; Glioblastoma - genetics; Humans; Isocitrate Dehydrogenase - genetics; Mutation; Prognosis; Retrospective Studies; World Health Organization
• ISSN: 1522-8517; 1523-5866
• DOI: 10.1093/neuonc/noz200

Abstract Background The Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy (cIMPACT-NOW) has recommended that isocitrate dehydrogenase 1 and 2 wildtype (IDH1/2wt) diffuse lower-grade gliomas (LGGs) World Health Organization (WHO) grade II or III that present with (i) a telomerase reverse transcriptase promoter mutation (pTERTmt), and/or (ii) gain of chromosome 7 combined with loss of chromosome 10, and/or (iii) epidermal growth factor receptor (EGFR) amplification should be reclassified as diffuse astrocytic glioma, IDH1/2 wildtype, with molecular features of glioblastoma, WHO grade IV (IDH1/2wt astrocytomas WHO IV). This paper describes the overall survival (OS) of IDH1/2wt astrocytoma WHO IV patients, and more in detail patients with tumors with pTERTmt only. Methods In this retrospective multicenter study, we compared the OS of 71 IDH1/2wt astrocytomas WHO IV patients, with radiological characteristics of LGGs, with the OS of 197 IDH1/2wt glioblastoma patients. Moreover, we compared the OS of 22 pTERTmt only astrocytoma patients with the OS of the IDH1/2wt glioblastoma patients. Results Median OS was similar for IDH1/2wt astrocytoma WHO IV patients (23.8 mo) and IDH1/2wt glioblastoma patients (19.2 mo) (Cox proportional hazards model: hazard ratio [HR] 1.27, 95% CI: 0.85–1.88, P = 0.242). OS was also similar in patients with IDH1/2wt astrocytomas WHO IV, pTERTmt only, and IDH1/2wt glioblastomas (HR 1.15, 95% CI: 0.64–2.10, P = 0.641). Conclusions The presented data confirm the cIMPACT-NOW recommendation and we propose that IDH1/2wt astrocytomas WHO IV in the absence of other qualifying mutations should be classified as IDH1/2wt glioblastomas.