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KMID : 1035520210090010001
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Brain Tumor Research and Treatment : BTRT
2021 Volume.9 No. 1 p.1 ~ p.8
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The Korean Society for Neuro-Oncology (KSNO) Guideline for Adult Diffuse Midline Glioma: Version 2021.1
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Yoon Hong-In
Wee Chan-Woo
Kim Young-Zoon
Seo Young-Beom
Im Jung-Ho
Dho Yun-Sik
Kim Kyung-Hwan
Hong Je-Beom
Park Jae-Sung
Choi Seo-Hee
Kim Min-Sung
Moon Jang-Sup
Hwang Ki-Hwan
Park Ji-Eun
Cho Jin-Mo
Yoon Wan-Soo
Kim Se-Hoon
Kim Young-Il
Kim Ho-Sung
Sung Kyoung-Su
Song Jin-Ho
Lee Min-Ho
Han Myung-Hoon
Lee Se-Hoon
Chang Jong-Hee
Lim Do-Hoon
Park Chul-Kee
Lee Youn-Soo
Gwak Ho-Shin
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Abstract
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Background: There have been no guidelines for the management of adult patients with diffuse midline glioma (DMG), H3K27M-mutant in Korea since the 2016 revised WHO classification newly defined this disease entity. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, had begun preparing guidelines for DMG since 2019.
Methods: The Working Group was composed of 27 multidisciplinary medical experts in Korea. References were identified through searches of PubMed, MEDLINE, EMBASE, and Cochrane CENTRAL using specific and sensitive keywords as well as combinations of keywords. As ‘diffuse midline glioma’ was recently defined, and there was no international guideline, trials and guidelines of ‘diffuse intrinsic pontine glioma’ or ‘brain stem glioma’ were thoroughly reviewed first.
Results: The core contents are as follows. The DMG can be diagnosed when all of the following three criteria are satisfied: the presence of the H3K27M mutation, midline location, and infiltrating feature. Without identification of H3K27M mutation by diagnostic biopsy, DMG cannot be diagnosed. For the primary treatment, maximal safe resection should be considered for tumors when feasible. Radiotherapy is the primary option for tumors in case the total resection is not possible. A total dose of 54 Gy to 60 Gy with conventional fractionation prescribed at 1?2 cm plus gross tumor volume is recommended. Although no chemotherapy has proven to be effective in DMG, concurrent chemoradiotherapy (± maintenance chemotherapy) with temozolomide following WHO grade IV glioblastoma's protocol is recommended.
Conclusion: The detection of H3K27M mutation is the most important diagnostic criteria for DMG. Combination of surgery (if amenable to surgery), radiotherapy, and chemotherapy based on comprehensive multidisciplinary discussion can be considered as the treatment options for DMG.
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KEYWORD
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Korean Society for Neuro-Oncology, Guideline, Diffuse midline glioma, Practice
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