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The Korean Society for Neuro-Oncology (KSNO) Guideline for Adult Diffuse Midline Glioma: Version 2021.1

 Hong In Yoon  ;  Chan Woo Wee  ;  Young Zoon Kim  ;  Youngbeom Seo  ;  Jung Ho Im  ;  Yun Sik Dho  ;  Kyung Hwan Kim  ;  Je Beom Hong  ;  Jae Sung Park  ;  Seo Hee Choi  ;  Min Sung Kim  ;  Jangsup Moon  ;  Kihwan Hwang  ;  Ji Eun Park  ;  Jin Mo Cho  ;  Wan Soo Yoon  ;  Se Hoon Kim  ;  Young Il Kim  ;  Ho Sung Kim  ;  Kyoung Su Sung  ;  Jin Ho Song  ;  Min Ho Lee  ;  Myung Hoon Han  ;  Se Hoon Lee  ;  Jong Hee Chang  ;  Do Hoon Lim  ;  Chul Kee Park  ;  Youn Soo Lee  ;  Ho Shin Gwak 
 Brain Tumor Research and Treatment, Vol.9(1) : 1-8, 2021-04 
Journal Title
Brain Tumor Research and Treatment
Issue Date
Diffuse midline glioma ; Guideline ; Korean Society for Neuro-Oncology ; Practice
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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Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Neurosurgery (신경외과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Radiation Oncology (방사선종양학교실) > 1. Journal Papers
Yonsei Authors
Kim, Se Hoon(김세훈) ORCID logo https://orcid.org/0000-0001-7516-7372
Yoon, Hong In(윤홍인) ORCID logo https://orcid.org/0000-0002-2106-6856
Chang, Jong Hee(장종희) ORCID logo https://orcid.org/0000-0003-1509-9800
Choi, Seo Hee(최서희) ORCID logo https://orcid.org/0000-0002-4083-6414
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