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Adult H3K27M mutated thalamic glioma patients display a better prognosis than unmutated patients

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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.

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Data availability

The datasets generated during and/or analysed during the current study are available from the corresponding author on reasonable request.

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Acknowledgements

We thank the ARTC-Sud patients’s association (Association pour le Recherche sur les Tumeurs Cérébrales). We thank the Cancéropôle PACA. We thank the AP-HM Tumor Bank (authorization number: AC2018-31053; CRB BB-0033-00097) for providing tissue samples.

Funding

ARTC-Sud.

Author information

Author notes

  1. Stéphan Grimaldi and Vincent Harlay—co-primary.

Authors and Affiliations

  1. Neuro-Oncologie Department, APHM, CHU Timone, University Hospital La Timone, Aix-Marseille University, 264 rue Saint Pierre, 13005, Marseille, France

    Stéphan Grimaldi, Vincent Harlay, Céline Bequet, Grégorio Petrirena, Chantal Campello, Maryline Barrié, Didier Autran, Olivier Chinot & Emeline Tabouret

  2. Department of Neurology and Movement Disorders, AP-HM, Aix-Marseille University, Marseille, France

    Stéphan Grimaldi

  3. Neuropathology Department University Hospital Timone, Aix Marseille University, Marseille, France

    Romain Appay & Dominique Figarella-Branger

  4. Neurosurgery Department, CHU Timone, AP-HM, Aix-Marseille University, Marseille, France

    Sébastien Boissonneau & Thomas Graillon

  5. Oncobiology Department, Aix-Marseille University, Marseille, France

    Isabelle Nanni

  6. Inst Neurophysiopathol, CNRS, INP, Aix-Marseille University, Marseille, France

    Romain Appay, Dominique Figarella-Branger, Olivier Chinot & Emeline Tabouret

Authors
  1. Stéphan Grimaldi
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Contributions

Conceptualization (ET); data curation (SG, VH, RA, CB, GP, CC, MB, DA, SB, TG, DFB, IN, OC); formal analysis (SG, VH, ET); methodology (ET); supervision (DFB, OC, ET); writing—original draft (SG, VH, ET); writing—review and editing (All authors).

Corresponding author

Correspondence to Emeline Tabouret.

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The study was approved by our local ethics committee in accordance with the Declaration of Helsinki.

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Grimaldi, S., Harlay, V., Appay, R. et al. Adult H3K27M mutated thalamic glioma patients display a better prognosis than unmutated patients. J Neurooncol 156, 615–623 (2022). https://doi.org/10.1007/s11060-022-03943-7

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  • DOI: https://doi.org/10.1007/s11060-022-03943-7

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