Spinal Cord Diffuse Midline Glioma, H3K27M- mutant Effectively Treated with Bevacizumab: A Report of Two Cases

Yabuno, Satoru; Kawauchi, Satoshi; Umakoshi, Michiari; Uneda, Atsuhito; Fujii, Kentaro; Ishida, Joji; Otani, Yoshihiro; Hattori, Yasuhiko; Tsuboi, Nobushige; Kohno, Shohei; Noujima, Mai; Toji, Tomohiro; Yanai, Hiroyuki; Yasuhara, Takao; Date, Isao

doi:10.2176/nmccrj.cr.2021-0033

Cited by 10 publications

(8 citation statements)

References 28 publications

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“…We summarized the reported cases, as shown in Table 1 . [ 8 , 19 ] Although our patient could not receive chemoradiotherapy due to his severely disabled condition, the other two patients could. Due to the limited number of documented cases involving DMG arising in the thalamus or the spinal cord, the significance of H3K27 mutation in terms of prognosis remains insufficiently understood.…”

Section: Discussionmentioning

confidence: 96%

“…Very few spinal cord DMG cases with rapid neurological deterioration have been reported. [ 8 , 19 ] Herein, we report a rare case of spinal cord DMG with unusual magnetic resonance (MR) imaging findings and rapid neurological deterioration. We emphasized that it is important to suspect DMG early on neuroimaging and discussed the timing of image acquisition.…”

Section: Introductionmentioning

confidence: 99%

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Spinal cord diffuse midline glioma with postoperative acute swelling: A case report and review of literature

Karita,

Tsurubuchi,

Amano

et al. 2023

Surgical Neurology International

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Background: H3K27-altered diffuse midline glioma (DMG) is a newly classified disease according to the 5th edition of the World Health Organization classification of the central nervous system tumors. However, little is known about its progression pattern and the timing of surgical intervention, especially regarding spinal cord lesions. Case Description: A 26-year-old man presented with rapid muscle weakness progression in both upper and lower extremities and urinary dysfunction. Magnetic resonance imaging showed diffuse swelling of the cervicothoracic spinal cord. He underwent decompressive laminectomy with expansive duroplasty and tumor biopsy. The surgical specimen revealed DMG. Immediately after surgery, deterioration of limb paresis was observed, and the patient developed respiratory failure the day after surgery. Head-and-neck computed tomography on the 7th day after surgery showed spinal cord swelling and acute obstructive hydrocephalus. Conclusion: We report a rare case of a spinal DMG with acute postoperative swelling. Neurological deterioration in patients with spinal cord DMG is often exacerbated, so it is essential to suspect DMG at an early stage based on neuroimaging, and if surgery is performed on the edematous spinal cord, further rapid swelling can occur, as in the present case.

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Section: Discussionmentioning

confidence: 96%

Section: Introductionmentioning

confidence: 99%

Spinal cord diffuse midline glioma with postoperative acute swelling: A case report and review of literature

Karita,

Tsurubuchi,

Amano

et al. 2023

Surgical Neurology International

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“…Furthermore, specific markers can be useful in an individualized approach, such as anti-EGFR antibody, which has lower toxicity. Other medications, such as monoclonal antibodies, tyrosine kinase inhibitors, and angiogenesis inhibitors have also been described as possible therapies for diffuse midline gliomas [15].…”

Section: Discussionmentioning

confidence: 99%

Spinal cord diffuse midline glioma with H3K27M mutation: case report in a pediatric patient

Rizzo,

Ramos,

Mota

et al. 2024

Arch Pediatr Neurosurg

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Introduction/Background: Pediatric diffuse midline gliomas are predominantly localized within the brainstem, pons, or are bi-thalamic, while in adolescents and adults, they predominantly arise unilaterally in the thalamus or spinal cord. Epidemiological data remain scarce for this recently described entity. They represent 10-15% of all pediatric brain tumors and 75% of all pediatric brainstem tumors. Case presentation: Herein we report a case of Diffuse Midline Glioma in a 10-year-old male pediatric patient, with an unusual location in the spinal cord, presenting with atypical clinical features. Histopathological and immunohistochemical analysis confirmed the diagnosis and the presence of K27M (lysine to methionine at codon 27) mutations in the histone 3 gene. Conclusions: Early and differential diagnosis of atypical spinal cord tumors is crucial to avoid dismal consequences for the patient and provide them with a better quality of life. Highlighting rare cases with fatal outcomes is essential for emphasizing the importance of understanding this pathological entity within the medical community.

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“… 14 These tumors are commonly located in the pons, with only a few cases reported in the spinal cord. 15 A recent study of 35 cases of spinal cord H3 K27M-mutant diffuse midline gliomas identified affected locations spanning cervical (29%), cervicothoracic (20%), thoracic (43%), and thoracolumbar (9%) regions. 14 Prognosis tends to be poor with rapid neurologic decline.…”

Section: Discussionmentioning

confidence: 99%

A 28-Year-Old Woman With Left-Sided Weakness and Atypical MRI Lesions

Safadi,

Osborne,

Chitnis

et al. 2023

Neurol Neuroimmunol Neuroinflamm

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A 28-year-old woman presented with subacute relapsing left-sided weakness. MRI demonstrated both enhancing C3-C6 and nonenhancing T2-T4 lesions. Initial provisional diagnosis was inflammatory/autoimmune. Her left-sided weakness progressed despite immunosuppressive therapies. We reassessed our original suspected diagnosis because of an atypical clinicoradiologic course, leading to biopsy and a definitive diagnosis.

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Spinal Cord Diffuse Midline Glioma, H3K27M- mutant Effectively Treated with Bevacizumab: A Report of Two Cases

Cited by 10 publications

References 28 publications

Spinal cord diffuse midline glioma with postoperative acute swelling: A case report and review of literature

Spinal cord diffuse midline glioma with postoperative acute swelling: A case report and review of literature

Spinal cord diffuse midline glioma with H3K27M mutation: case report in a pediatric patient

A 28-Year-Old Woman With Left-Sided Weakness and Atypical MRI Lesions

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