Brain Tumor Res Treat.  2021 Apr;9(1):1-8. 10.14791/btrt.2021.9.e8.

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

Affiliations
  • 1Department of Radiation Oncology, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Korea
  • 2Department of Radiation Oncology, SMG-SNU Boramae Medical Center, Seoul, Korea
  • 3Division of Neurooncology and Department of Neurosurgery, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon, Korea
  • 4Department of Neurosurgery, Yeungnam University Hospital, Yeungnam University College of Medicine, Daegu, Korea
  • 5Department of Radiation Oncology, CHA Bundang Medical Center, CHA University School of Medicine, Seongnam, Korea
  • 6Department of Neurosurgery, Chungbuk National University Hospital, Chungbuk National University College of Medicine, Cheongju, Korea
  • 7Department of Neurosurgery, Chungnam National University Hospital, Chungnam National University School of Medicine, Daejeon, Korea;
  • 8Department of Neurosurgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 9Department of Neurosurgery, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 10Department of Radiation Oncology, Yongin Severance Hospital, Yonsei University College of Medicine, Yongin, Korea
  • 11Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
  • 12Department of Genomic Medicine, Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
  • 13Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea
  • 14Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
  • 15Department of Neurosurgery, Catholic Kwandong University, International St. Mary’s Hospital, Incheon, Korea
  • 16Department of Neurosurgery, Incheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 17Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
  • 18Department of Neurosurgery, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Suwon, Korea
  • 19Department of Neurosurgery, Dong-A University Hospital, Dong-A University College of Medicine, Busan, Korea
  • 20Department of Radiation Oncology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 21Department of Neurosurgery, Uijeongbu St. Mary’s Hospital, The Catholic University of Korea, Uijeongbu, Korea
  • 22Department of Neurosurgery, Hanyang University Guri Hospital, Hanyang University College of Medicine, Guri, Korea
  • 23Division of Hematology/Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 24Department of Neurosurgery, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
  • 25Department of Radiation Oncology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 26Department of Hospital Pathology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 27Department of Cancer Control, Graduate School of Cancer Science and Policy, National Cancer Center, Goyang, Korea

Abstract

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.

Keyword

Korean Society for Neuro-Oncology; Guideline; Diffuse midline glioma; Practice
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