Abstract
AB088. Spinal cord diffuse midline glioma in adults: a case report and literature review
Ming Yang1, Brian Yuan-Lang Chan1, Yuan Guang Lim2, Jia Sheng Low1, Sai Liang2, Vincent Diong Weng Nga2, Yeo Tseng Tsai2, Eugene Weiren Yang1
1Division of Neurosurgery, Department of Surgery, Khoo Teck Puat Hospital, Singapore, Singapore;
2Division of Neurosurgery, Department of Surgery, National University Hospital Singapore, Singapore, Singapore
Correspondence to: Ming Yang, MD. Division of Neurosurgery, Department of Surgery, Khoo Teck Puat Hospital, 90 Yishun Central, Singapore 768828, Singapore; Division of Neurosurgery, Department of Surgery, National University Hospital Singapore, Singapore, Singapore. Email: yangming.yami@gmail.com.
Background: Spinal cord diffuse midline gliomas are rare, infiltrative entities with an extremely grim prognosis. Standard of care is limited and extrapolated from those for intracranial gliomas, focusing on maximal safe resection, chemotherapy and radiation therapy. These do not prolong survival significantly and while advances in molecular profiling and targeted therapy have been promising, further research still needs to be performed. Here, we present a case of a young lady with a cervical cord diffuse midline glioma, along with a literature review of the disease and treatment options.
Case Description: A 35-year-old female presented with progressive neck pain and left sided weakness. MRI revealed an intramedullary cervical spinal cord lesion. The lesion progressed rapidly to the medulla, resulting in lower cranial nerve palsies and left hemiplegia. Investigations for autoimmune and infective causes were negative. Cervical laminectomy and debulking was performed. Histological analysis showed high grade diffuse glioma, IDH-wildtype, loss of H3K27me3 staining and H3K27M positivity. The patient was treated with fractionated radiation and temozolamide, followed by lomustine and bevacizumab. A literature review was performed to better understand the molecular features, natural history and treatment options for spinal cord high grade gliomas. Our case highlights the importance of maintaining broad differentials for patients exhibiting features of cervical myelopathy. Malignant spinal cord tumours could be a differential. Molecular testing can aid in achieving an accurate diagnosis to better understand prognosis and determine treatment options. Early, function-preserving debulking with neuromonitoring is feasible. Adjuvant therapy with chemotherapy and radiation can prolong survival.
Conclusions: Spinal cord diffuse midline gliomas H3 K27-altered demonstrate rapid progression and a poor prognosis. They should be considered as a differential in patients with cervical myelopathy. Molecular testing for H3 K27 alterations facilitates an accurate diagnosis. Surgical debulking and adjuvant therapy are viable treatment options.
Keywords: Spinal cord diffuse midline glioma; high grade; case report
Acknowledgments
Funding: None.
Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://cco.amegroups.com/article/view/10.21037/cco-24-ab088/coif). The authors have no conflicts of interest to declare.
Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and national research committees and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.
Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the noncommercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.
Cite this abstract as: Yang M, Chan BYL, Lim YG, Low JS, Liang S, Nga VDW, Tsai YT, Yang EW. AB088. Spinal cord diffuse midline glioma in adults: a case report and literature review. Chin Clin Oncol 2024;13(Suppl 1):AB088. doi: 10.21037/cco-24-ab088
