• Neurosurgery · Sep 2019

    Radiological Characteristics and Natural History of Adult IDH-Wildtype Astrocytomas with TERT Promoter Mutations.

    • Cristina Izquierdo, Marc Barritault, Delphine Poncet, Stéphanie Cartalat, Bastien Joubert, Jordi Bruna, Emmanuel Jouanneau, Jacques Guyotat, Alexandre Vasiljevic, Tanguy Fenouil, Yves Berthezène, Jérôme Honnorat, David Meyronet, and François Ducray.
    • Hospices Civils de Lyon, Groupe Hos-pitalier Est, Service de Neuro-Oncologie, Lyon, France.
    • Neurosurgery. 2019 Sep 1; 85 (3): E448-E456.

    BackgroundAdult IDH-wildtype astrocytomas with TERT promoter mutations (TERTp) are associated with a poor prognosis.ObjectiveTo analyze the radiological presentation and natural history of adult IDH-wildtype astrocytomas with TERTp.MethodsWe 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.ResultsMost 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.ConclusionIDH-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.Copyright © 2018 by the Congress of Neurological Surgeons.

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