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Biczok, Annamaria; Kraus, Theo; Suchorska, Bogdana; Terpolilli, Nicole A.; Thorsteinsdottir, Jun; Giese, Armin; Tonn, Jörg C.; Schichor, Christian (2018): TERT promoter mutation is associated with worse prognosis in WHO grade II and III meningiomas. In: Journal of Neuro-Oncology, Vol. 139, No. 3: pp. 671-678
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Abstract

IntroductionTranscriptional activating mutations in the promoter region of the telomerase reverse transcriptase (TERT) gene occur at high frequency in various types of solid tumors and have also been reported for meningiomas. Especially for atypical and anaplastic meningiomas, the prognostic relevance of TERT promoter mutation is yet unclear. The present study aimed to analyze the frequency of TERT promoter mutation and define its long-term prognostic significance beyond clinical and histological factors in a cohort of meningiomas WHO grade II and III.Methods: Patients undergoing surgical resection of aggressive meningiomas were included. Analysis for C228T and C250T mutation in the TERT promoter region was performed using PCR method. Patients were stratified into two groups (TERT mutated vs. TERT wild type). Univariate analysis was conducted using molecular and histological factors.Results: 87 patients with atypical (N=72) and anaplastic meningiomas (N=15) were included in the study. TERT promoter region was found to be mutated in 4 WHO grade II and 2 WHO grade III meningiomas. TERT promoter mutation was associated with shorter progression free survival than TERT wild type meningiomas (median PFS 12.5 vs. 26 months, p=.004). In the univariate analysis, TERT promoter mutation had a strong prognostic value on overall survival (p=.009) and progression free survival.Conclusion: sPresence of TERT promoter mutation is associated with shorter progression free survival and overall survival in meningiomas WHO grade II and III. In these tumors, TERT promoter mutation should be considered as a clinically relevant prognostic factor to identify high risk patients.