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Diffuse Midline H3K27-Altered Gliomas in the Spinal Cord: A Systematic Review

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Abstract

Purpose

To systematically review the clinical features, management, and outcomes of diffuse midline H3K27-altered gliomas of the spinal cord (DMG-SCs).

Methods

PubMed, Ovid EMBASE, Scopus, and Web of Science were searched from database inception to 23 September 2023 for histologically confirmed cases of DMG-SC. Patient demographics, tumor characteristics, management information, and survival outcomes were extracted and analyzed.

Results

A total of 279 patients from 39 studies were collected. Patients were mostly male (61%), with an average age of 32 years. Patients were treated with surgery, radiotherapy, and chemotherapy combined (31%) or surgery only (24%), and extent of resection was most often subtotal (38%). Temozolomide was the most common chemotherapeutic agent (81%). Radiation therapy was delivered with mean dose of 47 Gy in 23 fractions. At mean follow-up time of 21 months, 13% of patients were alive. Average median overall survival was 24 months (range of 13 to 40 months) with a median progression-free survival of 14 months. Historical WHO grades of 2 or 3 appeared to exhibit a longer average median overall survival time than that of grade 4 DMG-SCs (32 vs. 23 months, p = 0.009).

Conclusions

Outcomes for DMG-SCs are poor overall but appear to be favorable compared to intracranial DMGs. Despite the recent WHO 2021 grade 4 classification for all DMGs, given the differences in overall survival reported based on historical grading systems, future studies on DMG-SCs are needed to further define if DMG-SCs may represent a heterogeneous group of tumors with different prognoses.

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Data availability

Data is available upon request in spreadsheet format.

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Acknowledgements

G.W. performed material preparation, data collection, analysis, and writing of the original draft. J.M.W., B.E., and M.F.K. performed data collection, analysis, and manuscript review. C.O. performed material preparation and data collection. G.E.U., A.M., and O.B. performed study supervision and manuscript review. P.P. performed material preparation, supervision, manuscript review, project conception and design. All authors read and approved the final manuscript.

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The authors declare that no funds, grants, or other support were received during the preparation of this manuscript.

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Authors and Affiliations

  1. John A. Burns School of Medicine, University of Hawai’i, Honolulu, HI, USA

    Gina Watanabe & Jennifer Manyu Wong

  2. University of Kansas School of Medicine, Kansas City, KS, USA

    Bradley Estes

  3. Indiana University School of Medicine, Indianapolis, IN, USA

    Mohammad Faizan Khan

  4. Department of Neurosurgery, University of Texas Medical Branch, Galveston, TX, USA

    Christian Ogasawara

  5. Department of Neurosurgery, Trauma Center, Gamma Knife Center, Cannizzaro Hospital, Catania, Italy

    Giuseppe E. Umana

  6. Department of Neurological Surgery, University of California, Davis, Sacramento, CA, USA

    Allan R. Martin, Orin Bloch & Paolo Palmisciano

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  1. Gina Watanabe
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Watanabe, G., Wong, J.M., Estes, B. et al. Diffuse Midline H3K27-Altered Gliomas in the Spinal Cord: A Systematic Review. J Neurooncol 166, 379–394 (2024). https://doi.org/10.1007/s11060-024-04584-8

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