Radiological Characteristics and Natural History of Adult IDH-Wildtype Astrocytomas with TERT Promoter Mutations. Issue 3 (8th November 2018)

• Record Type: Journal Article
• Authors: Izquierdo, Cristina; Barritault, Marc; Poncet, Delphine; Cartalat, Stéphanie; Joubert, Bastien; Bruna, Jordi; Jouanneau, Emmanuel; Guyotat, Jacques; Vasiljevic, Alexandre; Fenouil, Tanguy; Berthezène, Yves; Honnorat, Jérôme; Meyronet, David; Ducray, François
• Is Part Of: Neurosurgery. Volume 85:Issue 3(2019)
• Page Start: E448
• Page End: E456
• Publication Date: 2018-11-08
• Subjects: IDH-wildtype astrocytoma -- Lower grade gliomas -- Telomerase reverse transcriptase promoter; Nervous system -- Surgery -- Periodicals; 617.48005
• DOI: 10.1093/neuros/nyy513
• Languages: English
• ISSNs: 0148-396X

Abstract

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.