Cristina Izquierdo1,2, Marc Barritault3,4,5, Delphine Poncet3,4, Stéphanie Cartalat1, Bastien Joubert1, Jordi Bruna2, Emmanuel Jouanneau4,6,7, Jacques Guyotat8, Alexandre Vasiljevic3,4, Tanguy Fenouil3,4, Yves Berthezène4,9, Jérôme Honnorat1,4,10, David Meyronet3,4,5, François Ducray1,4,5. 1. Hospices Civils de Lyon, Groupe Hos-pitalier Est, Service de Neuro-Oncologie, Lyon, France. 2. Unit of Neuro-Oncology, Hospital Universitari de Bellvitge-ICO L'Hospitalet-IDIBELL, l'Hospitalet de Llo-bregat, Barcelona, Spain. 3. Hospices Civils de Lyon, Groupe Hospitalier Est, Service d'anatomopathologie, Lyon, France. 4. Université Claude Bernard Lyon 1, Lyon, France. 5. Department of Cancer Cell Plasticity, Cancer Research Centre of Lyon, INSERM U1052, CNRS UMR5286, Lyon, France. 6. Hospices Civils de Lyon, Groupement Hospitalier Est, Service de Neurochirurgie B, Lyon, France. 7. Signaling, Metabolism and Tumor Progression, Cancer Research Centre of Lyon, INSERM U1052, CNRS UMR5286, Lyon, France. 8. Hospices Civils de Lyon, Groupement Hospitalier Est, Service de Neurochirurgie D, Lyon, France. 9. Hospices Civils de Lyon, Groupe Hos-pitalier Est, Service de Neuroradiologie, Lyon, France. 10. Institut NeuroMyoGene, INSERM 1217/CNRS 5310, Université de Lyon, Lyon, France.
Abstract
BACKGROUND: Adult IDH-wildtype astrocytomas with TERT promoter mutations (TERTp) are associated with a poor prognosis. OBJECTIVE: To analyze the radiological presentation and natural history of adult IDH-wildtype astrocytomas with TERTp. METHODS: We retrospectively reviewed the characteristics of 40 IDH-wildtype TERTp-mutant astrocytomas (grade II n = 19, grade III n = 21) and compared them to those of 114 IDH-mutant lower grade gliomas (LGG), of 92 IDH-wildtype TERTp-mutant glioblastomas, and of 15 IDH-wildtype TERTp-wildtype astrocytomas. RESULTS: Most cases of IDH-wildtype TERTp-mutant astrocytomas occurred in patients aged >50 yr (88%) and presented as infiltrative lesions without contrast enhancement (73%) that were localized in the temporal and/or insular lobes (37.5%) or corresponded to a gliomatosis cerebri (43%). Thalamic involvement (33%) and extension to the brainstem (27%) were frequently observed, as was gyriform infiltration (33%). This radiological presentation was different from that of IDH-mutant LGG, IDH-wildtype TERTp-mutant glioblastomas, and IDH-wildtype TERTp-wildtype astrocytomas. Tumor evolution before treatment initiation was assessable in 17 cases. Ten cases demonstrated a rapid growth characterized by the apparition of a ring-like contrast enhancement and/or a median velocity of diametric expansion (VDE) ≥8 mm/yr but 7 cases displayed a slow growth (VDE <8 mm/yr) that could last several years before anaplastic transformation. Median overall survival of IDH-wildtype TERTp-mutant astrocytomas was 27 mo. CONCLUSION: IDH-wildtype TERTp-mutant astrocytomas typically present as nonenhancing temporo-insular infiltrative lesions or as gliomatosis cerebri in patients aged >50 yr. In the absence of treatment, although rapid tumor growth is frequent, an initial falsely reassuring, slow growth can be observed.
BACKGROUND: Adult IDH-wildtype astrocytomas with TERT promoter mutations (TERTp) are associated with a poor prognosis. OBJECTIVE: To analyze the radiological presentation and natural history of adult IDH-wildtype astrocytomas with TERTp. METHODS: We retrospectively reviewed the characteristics of 40 IDH-wildtype TERTp-mutant astrocytomas (grade II n = 19, grade III n = 21) and compared them to those of 114 IDH-mutant lower grade gliomas (LGG), of 92 IDH-wildtype TERTp-mutant glioblastomas, and of 15 IDH-wildtype TERTp-wildtype astrocytomas. RESULTS: Most cases of IDH-wildtype TERTp-mutant astrocytomas occurred in patients aged >50 yr (88%) and presented as infiltrative lesions without contrast enhancement (73%) that were localized in the temporal and/or insular lobes (37.5%) or corresponded to a gliomatosis cerebri (43%). Thalamic involvement (33%) and extension to the brainstem (27%) were frequently observed, as was gyriform infiltration (33%). This radiological presentation was different from that of IDH-mutant LGG, IDH-wildtype TERTp-mutant glioblastomas, and IDH-wildtype TERTp-wildtype astrocytomas. Tumor evolution before treatment initiation was assessable in 17 cases. Ten cases demonstrated a rapid growth characterized by the apparition of a ring-like contrast enhancement and/or a median velocity of diametric expansion (VDE) ≥8 mm/yr but 7 cases displayed a slow growth (VDE <8 mm/yr) that could last several years before anaplastic transformation. Median overall survival of IDH-wildtype TERTp-mutant astrocytomas was 27 mo. CONCLUSION:IDH-wildtype TERTp-mutant astrocytomas typically present as nonenhancing temporo-insular infiltrative lesions or as gliomatosis cerebri in patients aged >50 yr. In the absence of treatment, although rapid tumor growth is frequent, an initial falsely reassuring, slow growth can be observed.
Authors: Shota Tanaka; Tracy T Batchelor; A John Iafrate; Dora Dias-Santagata; Darrell R Borger; Leif W Ellisen; Daniel Yang; David N Louis; Daniel P Cahill; Andrew S Chi Journal: Acta Neuropathol Commun Date: 2019-04-29 Impact factor: 7.801
Authors: Jurij Rosen; Gabriele Stoffels; Philipp Lohmann; Elena K Bauer; Jan-Michael Werner; Michael Wollring; Marion Rapp; Jörg Felsberg; Martin Kocher; Gereon R Fink; Karl-Josef Langen; Norbert Galldiks Journal: Sci Rep Date: 2021-10-21 Impact factor: 4.379