Hypofractionated radiotherapy combined with bevacizumab plus low-dose ifosfamide, carboplatin, and etoposide as second-line chemoradiotherapy for progressing spinal diffuse midline glioma, H3K27-altered: illustrative case

Spinal cord diffuse midline glioma (DMG) is a relatively rare disease with a poor prognosis and no effective treatment. A 45-year-old man presented with rapidly progressive paraplegia in both lower extremities, along with bladder and bowel disturbance. Spinal magnetic resonance imaging (MRI) showed...

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Published inJournal of neurosurgery. Case lessons Vol. 8; no. 7
Main Authors Nakayasu, Shintaro, Tanji, Masahiro, Uto, Megumi, Takeuchi, Yasuhide, Makino, Yasuhide, Yamamoto Hattori, Etsuko, Terada, Yukinori, Sano, Noritaka, Mineharu, Yohei, Mizowaki, Takashi, Arakawa, Yoshiki
Format Journal Article
LanguageEnglish
Published United States American Association of Neurological Surgeons 12.08.2024
Subjects
Case Lesson
bevacizumab
spinal diffuse midline glioma
ICE
H3K27-altered
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Summary:Spinal cord diffuse midline glioma (DMG) is a relatively rare disease with a poor prognosis and no effective treatment. A 45-year-old man presented with rapidly progressive paraplegia in both lower extremities, along with bladder and bowel disturbance. Spinal magnetic resonance imaging (MRI) showed a heterogeneously contrast-enhanced mass at the T1-4 levels. A biopsy via T1-4 decompressive laminectomy with expansive duraplasty was performed. The histopathological diagnosis was DMG, H3K27-altered, World Health Organization grade 4. Radiation plus concomitant temozolomide was started; however, follow-up MRI showed tumor progression. Additional hypofractionated radiotherapy (HFRT; 24 Gy/5 fractions) was performed, with bevacizumab (BEV) plus low-dose ifosfamide-carboplatin-etoposide (ICE) as second-line treatment. One month later, MRI showed tumor regression with significant improvement in the peritumoral edema. The chemotherapy regimen was repeated every 4-6 weeks, and the patient remained stable. After 13 courses of chemotherapy, the size of the spinal DMG increased markedly, with dissemination to the temporal lobe. The patient died approximately 21 months after the initial diagnosis. Spinal DMG is a malignant tumor with a poor prognosis. However, treatment with additional HFRT combined with BEV plus low-dose ICE may inhibit tumor progression to prolong the progression-free period and survival. https://thejns.org/doi/10.3171/CASE2464.
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INCLUDE WHEN CITING Published August 12, 2024; DOI: 10.3171/CASE2464.
Disclosures Dr. Mizowaki reported grants from Varian Medical Systems, Hitachi, and BrainLab outside the submitted work. Dr. Arakawa reported grants from Philips, Otsuka, Chugai, Nihon Medi-Physics, Daiichi Sankyo, Stryker, Eisai, Japan Blood Products Organization, Ono Pharmaceutical, Taiho Pharma, Sumitomo Dainippon Pharma, Astellas Pharma, Incyte Biosciences, and Servier; personal fees from Nippon Kayaku, Novocure, UCB Japan, Ono Pharmaceutical, Brainlab, Merck, Chugai, Eisai, Daiichi Sankyo, Carl Zeiss, Nihon Medi-Physics, and Stryker outside the submitted work.
ISSN:2694-1902
2694-1902
DOI:10.3171/CASE2464