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

doi:10.14791/btrt.2021.9.e8

. 2021 Apr;9(1):1-8.

doi: 10.14791/btrt.2021.9.e8.

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

Hong In Yoon¹, Chan Woo Wee², Young Zoon Kim³, Youngbeom Seo⁴, Jung Ho Im⁵, Yun Sik Dho⁶, Kyung Hwan Kim⁷, Je Beom Hong⁸, Jae Sung Park⁹, Seo Hee Choi¹⁰, Min Sung Kim¹¹, Jangsup Moon¹², Kihwan Hwang¹³, Ji Eun Park¹⁴, Jin Mo Cho¹⁵, Wan Soo Yoon¹⁶, Se Hoon Kim¹⁷, Young Il Kim¹⁸, Ho Sung Kim¹⁴, Kyoung Su Sung¹⁹, Jin Ho Song²⁰, Min Ho Lee²¹, Myung Hoon Han²², Se Hoon Lee²³, Jong Hee Chang²⁴, Do Hoon Lim²⁵, Chul Kee Park^#²⁶, Youn Soo Lee^#²⁷, Ho Shin Gwak^#²⁸; KSNO Guideline Working Group

Affiliations

¹ Department of Radiation Oncology, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Korea.
² Department of Radiation Oncology, SMG-SNU Boramae Medical Center, Seoul, Korea.
³ Division of Neurooncology and Department of Neurosurgery, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon, Korea.
⁴ Department of Neurosurgery, Yeungnam University Hospital, Yeungnam University College of Medicine, Daegu, Korea.
⁵ Department of Radiation Oncology, CHA Bundang Medical Center, CHA University School of Medicine, Seongnam, Korea.
⁶ Department of Neurosurgery, Chungbuk National University Hospital, Chungbuk National University College of Medicine, Cheongju, Korea.
⁷ Department of Neurosurgery, Chungnam National University Hospital, Chungnam National University School of Medicine, Daejeon, Korea.
⁸ Department of Neurosurgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea.
⁹ Department of Neurosurgery, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
¹⁰ Department of Radiation Oncology, Yongin Severance Hospital, Yonsei University College of Medicine, Yongin, Korea.
¹¹ Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
¹² Department of Genomic Medicine, Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
¹³ Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.
¹⁴ Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
¹⁵ Department of Neurosurgery, Catholic Kwandong University, International St. Mary's Hospital, Incheon, Korea.
¹⁶ Department of Neurosurgery, Incheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
¹⁷ Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
¹⁸ Department of Neurosurgery, St. Vincent's Hospital, College of Medicine, The Catholic University of Korea, Suwon, Korea.
¹⁹ Department of Neurosurgery, Dong-A University Hospital, Dong-A University College of Medicine, Busan, Korea.
²⁰ Department of Radiation Oncology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
²¹ Department of Neurosurgery, Uijeongbu St. Mary's Hospital, The Catholic University of Korea, Uijeongbu, Korea.
²² Department of Neurosurgery, Hanyang University Guri Hospital, Hanyang University College of Medicine, Guri, Korea.
²³ Division of Hematology/Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
²⁴ Department of Neurosurgery, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
²⁵ Department of Radiation Oncology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
²⁶ Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea. nschpark@snu.ac.kr.
²⁷ Department of Hospital Pathology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. lys9908@catholic.ac.kr.
²⁸ Department of Cancer Control, Graduate School of Cancer Science and Policy, National Cancer Center, Goyang, Korea. nsghs@ncc.re.kr.

^# Contributed equally.

PMID: 33913265
PMCID: PMC8082289
DOI: 10.14791/btrt.2021.9.e8

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

Hong In Yoon et al. Brain Tumor Res Treat. 2021 Apr.

. 2021 Apr;9(1):1-8.

doi: 10.14791/btrt.2021.9.e8.

Authors

Affiliations

¹ Department of Radiation Oncology, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Korea.
² Department of Radiation Oncology, SMG-SNU Boramae Medical Center, Seoul, Korea.
³ Division of Neurooncology and Department of Neurosurgery, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon, Korea.
⁴ Department of Neurosurgery, Yeungnam University Hospital, Yeungnam University College of Medicine, Daegu, Korea.
⁵ Department of Radiation Oncology, CHA Bundang Medical Center, CHA University School of Medicine, Seongnam, Korea.
⁶ Department of Neurosurgery, Chungbuk National University Hospital, Chungbuk National University College of Medicine, Cheongju, Korea.
⁷ Department of Neurosurgery, Chungnam National University Hospital, Chungnam National University School of Medicine, Daejeon, Korea.
⁸ Department of Neurosurgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea.
⁹ Department of Neurosurgery, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
¹⁰ Department of Radiation Oncology, Yongin Severance Hospital, Yonsei University College of Medicine, Yongin, Korea.
¹¹ Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
¹² Department of Genomic Medicine, Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
¹³ Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.
¹⁴ Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
¹⁵ Department of Neurosurgery, Catholic Kwandong University, International St. Mary's Hospital, Incheon, Korea.
¹⁶ Department of Neurosurgery, Incheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
¹⁷ Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
¹⁸ Department of Neurosurgery, St. Vincent's Hospital, College of Medicine, The Catholic University of Korea, Suwon, Korea.
¹⁹ Department of Neurosurgery, Dong-A University Hospital, Dong-A University College of Medicine, Busan, Korea.
²⁰ Department of Radiation Oncology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
²¹ Department of Neurosurgery, Uijeongbu St. Mary's Hospital, The Catholic University of Korea, Uijeongbu, Korea.
²² Department of Neurosurgery, Hanyang University Guri Hospital, Hanyang University College of Medicine, Guri, Korea.
²³ Division of Hematology/Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
²⁴ Department of Neurosurgery, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
²⁵ Department of Radiation Oncology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
²⁶ Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea. nschpark@snu.ac.kr.
²⁷ Department of Hospital Pathology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. lys9908@catholic.ac.kr.
²⁸ Department of Cancer Control, Graduate School of Cancer Science and Policy, National Cancer Center, Goyang, Korea. nsghs@ncc.re.kr.

^# Contributed equally.

PMID: 33913265
PMCID: PMC8082289
DOI: 10.14791/btrt.2021.9.e8

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.

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

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Conflict of interest statement

The authors have no potential conflicts of interest.

Figures

Fig. 1. Diagnosis algorithm for diffuse midline glioma (DMG). To diagnose DMGs, detection of H3K27M mutation, the location of tumor (midline location), and infiltrating feature are essential (➀+➁+➂). In other words, physicians are not recommended to diagnose DMG if the tumor is not located at the midline location (➁), even if H3K27M mutation is detected (➀). Also, if the diagnostic biopsy is not possible or H3K27M mutation is not identified (➃), the tumor cannot be diagnosed as DMG even if both location (midline) (➁) and infiltrating feature (➂) are satisfied.

**Fig. 2. Overview of the Korean Society for Neuro-Oncology (KSNO) Guideline for Adult Diffuse Midline Glioma: Version 2021.01.**

See this image and copyright information in PMC

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References

1. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK. WHO classification of tumours of the central nervous system. Revised 4th ed. Lyon: International Agency for Research on Cancer; 2016.
1. Karremann M, Gielen GH, Hoffmann M, et al. Diffuse high-grade gliomas with H3 K27M mutations carry a dismal prognosis independent of tumor location. Neuro Oncol. 2018;20:123–131. - PMC - PubMed
1. Schreck KC, Ranjan S, Skorupan N, et al. Incidence and clinicopathologic features of H3 K27M mutations in adults with radiographically-determined midline gliomas. J Neurooncol. 2019;143:87–93. - PMC - PubMed
1. Wu G, Broniscer A, McEachron TA, et al. Somatic histone H3 alterations in pediatric diffuse intrinsic pontine gliomas and non-brainstem glioblastomas. Nat Genet. 2012;44:251–253. - PMC - PubMed
1. Nikbakht H, Panditharatna E, Mikael LG, et al. Spatial and temporal homogeneity of driver mutations in diffuse intrinsic pontine glioma. Nat Commun. 2016;7:11185. - PMC - PubMed

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[1] Louis DN, Ohgaki H, Wiestler OD, Cavenee WK. WHO classification of tumours of the central nervous system. Revised 4th ed. Lyon: International Agency for Research on Cancer; 2016.

[2] Louis DN, Ohgaki H, Wiestler OD, Cavenee WK. WHO classification of tumours of the central nervous system. Revised 4th ed. Lyon: International Agency for Research on Cancer; 2016.

[3] Karremann M, Gielen GH, Hoffmann M, et al. Diffuse high-grade gliomas with H3 K27M mutations carry a dismal prognosis independent of tumor location. Neuro Oncol. 2018;20:123–131. - PMC - PubMed

[4] Karremann M, Gielen GH, Hoffmann M, et al. Diffuse high-grade gliomas with H3 K27M mutations carry a dismal prognosis independent of tumor location. Neuro Oncol. 2018;20:123–131. - PMC - PubMed

[5] Schreck KC, Ranjan S, Skorupan N, et al. Incidence and clinicopathologic features of H3 K27M mutations in adults with radiographically-determined midline gliomas. J Neurooncol. 2019;143:87–93. - PMC - PubMed

[6] Schreck KC, Ranjan S, Skorupan N, et al. Incidence and clinicopathologic features of H3 K27M mutations in adults with radiographically-determined midline gliomas. J Neurooncol. 2019;143:87–93. - PMC - PubMed

[7] Wu G, Broniscer A, McEachron TA, et al. Somatic histone H3 alterations in pediatric diffuse intrinsic pontine gliomas and non-brainstem glioblastomas. Nat Genet. 2012;44:251–253. - PMC - PubMed

[8] Wu G, Broniscer A, McEachron TA, et al. Somatic histone H3 alterations in pediatric diffuse intrinsic pontine gliomas and non-brainstem glioblastomas. Nat Genet. 2012;44:251–253. - PMC - PubMed

[9] Nikbakht H, Panditharatna E, Mikael LG, et al. Spatial and temporal homogeneity of driver mutations in diffuse intrinsic pontine glioma. Nat Commun. 2016;7:11185. - PMC - PubMed

[10] Nikbakht H, Panditharatna E, Mikael LG, et al. Spatial and temporal homogeneity of driver mutations in diffuse intrinsic pontine glioma. Nat Commun. 2016;7:11185. - PMC - PubMed

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

Affiliations

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

Authors

Affiliations

Abstract

Conflict of interest statement

Figures

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