Stéphan Grimaldi1,2, Vincent Harlay1, Romain Appay3,4, Céline Bequet1, Grégorio Petrirena1, Chantal Campello1, Maryline Barrié1, Didier Autran1, Sébastien Boissonneau5, Thomas Graillon5, Dominique Figarella-Branger3,4, Isabelle Nanni6, Olivier Chinot1,4, Emeline Tabouret7,8. 1. Neuro-Oncologie Department, APHM, CHU Timone, University Hospital La Timone, Aix-Marseille University, 264 rue Saint Pierre, 13005, Marseille, France. 2. Department of Neurology and Movement Disorders, AP-HM, Aix-Marseille University, Marseille, France. 3. Neuropathology Department University Hospital Timone, Aix Marseille University, Marseille, France. 4. Inst Neurophysiopathol, CNRS, INP, Aix-Marseille University, Marseille, France. 5. Neurosurgery Department, CHU Timone, AP-HM, Aix-Marseille University, Marseille, France. 6. Oncobiology Department, Aix-Marseille University, Marseille, France. 7. Neuro-Oncologie Department, APHM, CHU Timone, University Hospital La Timone, Aix-Marseille University, 264 rue Saint Pierre, 13005, Marseille, France. Emeline.tabouret@gmail.com. 8. Inst Neurophysiopathol, CNRS, INP, Aix-Marseille University, Marseille, France. Emeline.tabouret@gmail.com.
Abstract
BACKGROUND: Adult thalamic gliomas are a rare entity whose management is challenging for physicians. The aim of this study is to describe the characteristics and prognostic factors of thalamic gliomas in adult patients. METHODS: We retrospectively analyzed the clinical, neuro-radiological, histological, and molecular characteristics of all cases of adult thalamic glioma in our regional center. RESULTS: We included 38 adult patients. Median age at diagnosis was 56.5 years old (range, 24-80). Median KPS at diagnosis was 70%. Two-thirds of patients presented with tumor necrosis on MRI. Bithalamic lesions were present in four patients. The median volume of enhancement associated with lesions was relatively small (14 mm3). Two patients had undergone partial surgical resection. All other patients underwent biopsy. Median PFS was 7.1 months (95% CI [3.7-10.5]) and median OS was 15.6 months (95% CI [11.7-19.6]). Among 20 patients with available tumor samples for molecular analyses, only 4 (20%) presented with H3K27M mutation. Patients with H3K27M mutation had longer survival compared to those without. Finally, we identified a long-term survivor population characterized by a younger age, no cognitive impairment, low steroid dose treatment and the presence of H3K27M mutation. CONCLUSION: Thalamic adult glioma differs from bithalamic glioma in children with regards to its clinical, radiological and molecular profiles. Long-term survival is observed in young patients with limited symptoms and H3K27M mutation. A larger prospective cohort is needed to validate these findings.
BACKGROUND: Adult thalamic gliomas are a rare entity whose management is challenging for physicians. The aim of this study is to describe the characteristics and prognostic factors of thalamic gliomas in adult patients. METHODS: We retrospectively analyzed the clinical, neuro-radiological, histological, and molecular characteristics of all cases of adult thalamic glioma in our regional center. RESULTS: We included 38 adult patients. Median age at diagnosis was 56.5 years old (range, 24-80). Median KPS at diagnosis was 70%. Two-thirds of patients presented with tumor necrosis on MRI. Bithalamic lesions were present in four patients. The median volume of enhancement associated with lesions was relatively small (14 mm3). Two patients had undergone partial surgical resection. All other patients underwent biopsy. Median PFS was 7.1 months (95% CI [3.7-10.5]) and median OS was 15.6 months (95% CI [11.7-19.6]). Among 20 patients with available tumor samples for molecular analyses, only 4 (20%) presented with H3K27M mutation. Patients with H3K27M mutation had longer survival compared to those without. Finally, we identified a long-term survivor population characterized by a younger age, no cognitive impairment, low steroid dose treatment and the presence of H3K27M mutation. CONCLUSION: Thalamic adult glioma differs from bithalamic glioma in children with regards to its clinical, radiological and molecular profiles. Long-term survival is observed in young patients with limited symptoms and H3K27M mutation. A larger prospective cohort is needed to validate these findings.
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