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The Korean Society for Neuro-Oncology (KSNO) Guideline for WHO Grade III Cerebral Gliomas in Adults: Version 2019.01

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The Korean Society for Neuro-Oncology (KSNO) Guideline for WHO Grade III Cerebral Gliomas in Adults: Version 2019.01 Brain Tumor Res Treat 2019;7(2):63-73 / pISSN 2288-2405 / eISSN 2288-2413 ORIGINAL ARTICLE https://doi.org/10.14791/btrt.2019.7.e42 The Korean Society for Neuro-Oncology (KSNO) Guideline for WHO Grade III Cerebral Gliomas in Adults: Version 2019.01 Young Zoon Kim1, Chae-Yong Kim2, Jaejoon Lim3, Kyoung Su Sung4, Jihae Lee5, Hyuk-Jin Oh6, Seok-Gu Kang7, Shin-Hyuk Kang8, Doo-Sik Kong9, Sung Hwan Kim10, Se-Hyuk Kim11, Se Hoon Kim12, Yu Jung Kim13, Eui Hyun Kim7, In Ah Kim14, Ho Sung Kim15, Tae Hoon Roh11, Jae-Sung Park16, Hyun Jin Park17, Sang Woo Song18, Seung Ho Yang19, Wan-Soo Yoon20, Hong In Yoon21, Soon-Tae Lee22, Sea-Won Lee23, Youn Soo Lee24, Chan Woo Wee14, Jong Hee Chang7, Tae-Young Jung25, Hye Lim Jung26, Jae Ho Cho21, Seung Hong Choi27, Hyoung Soo Choi28, Je Beom Hong29, Do Hoon Lim30*, Dong-Sup Chung20*; KSNO Guideline Working Group Received August 20, 2019 Background There was no practical guideline for the management of patients with central nervous Revised August 22, 2019 system tumor in Korea in the past. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisci- Accepted September 30, 2019 plinary academic society, developed the guideline for glioblastoma successfully and published it in Correspondence Brain Tumor Research and Treatment, the official journal of KSNO, in April 2019. Recently, the KSNO Do Hoon Lim guideline for World Health Organization (WHO) grade III cerebral glioma in adults has been established. Department of Radiation Oncology, Samsung Medical Center, Methods The Working Group was composed of 35 multidisciplinary medical experts in Korea. Sungkyunkwan University School of References were identified by searches in PubMed, MEDLINE, EMBASE, and Cochrane CENTRAL Medicine, 81 Irwon-ro, Gangnam-gu, databases using specific and sensitive keywords as well as combinations of keywords. Scope of the Seoul 06351, Korea disease was confined to cerebral anaplastic astrocytoma and oligodendroglioma in adults. Tel: +82-2-3410-2600 Whenever radiological feature suggests high grade glioma, maximal safe resection if Fax: +82-2-3410-2609 Results feasible is globally recommended. After molecular and histological examinations, patients with ana- E-mail: [email protected] plastic astrocytoma, isocitrate dehydrogenase (IDH)-mutant should be primary treated by standard Dong-Sup Chung brain radiotherapy and adjuvant temozolomide chemotherapy whereas those with anaplastic astrocy- Department of Neurosurgery, toma, NOS, and anaplastic astrocytoma, IDH-wildtype should be treated following the protocol for Incheon St. Mary’s Hospital, glioblastomas. In terms of anaplastic oligodendroglioma, IDH-mutant and 1p19q-codeletion, and ana- College of Medicine, The Catholic University of Korea, plastic oligodendroglioma, NOS should be primary treated by standard brain radiotherapy and neoad- 56 Dongsu-ro, Bupyeong-gu, juvant or adjuvant PCV (procarbazine, lomustine, and vincristine) combination chemotherapy. Incheon 21431, Korea Conclusion The KSNO’s guideline recommends that WHO grade III cerebral glioma of adults Tel: +82-32-280-5841 should be treated by maximal safe resection if feasible, followed by radiotherapy and/or chemotherapy Fax: +82-32-280-5991 E-mail: [email protected] according to molecular and histological features of tumors. *These authors contributed equally to this Key Words Korean Society for Neuro Oncology; Guideline; Grade III Gliomas; Practice. work as a corresponding author. INTRODUCTION anaplastic oligoastrocytoma, anaplastic ependymoma, and anaplastic pleomorphic xanthoastrocytoma as grade III glio- Historically, the 2007 World Health Organization (WHO) mas. Actually, WHO grade III gliomas are not common as classification of central nervous system (CNS) tumours clas- glioblastomas. All other malignant gliomas including WHO sified anaplastic astrocytoma, anaplastic oligodendroglioma, grade II and III gliomas are composed of 10.2% of all prima- ry CNS tumors in the United State [1] and 5.3% in Korea [2]. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (https://creativecommons.org/licenses/by-nc/4.0) Such classification was based on morphological features on which permits unrestricted non-commercial use, distribution, and reproduction in any light microscopy of hematoxylin and eosin-stained sections, medium, provided the original work is properly cited. Copyright © 2019 The Korean Brain Tumor Society, The Korean Society for Neuro- immunohistochemical expression of lineage associated pro- Oncology, and The Korean Society for Pediatric Neuro-Oncology teins, and ultrastructural characterization [3]. However, stud- 63 KSNO’s Guideline for WHO Grade III Gliomas ies over the past two decades have clarified the genetic basis patients with WHO grade III gliomas [6]. of tumorigenesis for some brain tumor entities, raising the However, it is a critical point that circumferences of treating possibility that such an understanding may contribute to patients with WHO grade III gliomas in Korea are much dif- new classification of these tumors [4]. The use of integrated ferent from those in other countries. The incidence of WHO phenotypic and genotypic parameters for classification of gli- grade III gliomas is relatively lower [2] and clinical practice is omas adds a level of objectivity that has been missing from much limited in Korea because Korean government supports some aspects of the diagnostic process in the past [5]. Ac- medical care financially by the National Health Insurance cording to the new WHO classification of CNS tumours pub- System which limits application of updated therapeutic mo- lished in 2016, the following gliomas are classified as WHO dalities to patients. grade III gliomas: 1) anaplastic astrocytoma, isocitrate dehy- Although the clinical practical guideline for WHO grade drogenase (IDH)-mutant, 2) anaplastic oligodendroglioma, IV gliomas has been just developed and published recently by IDH-mutant and 1p19q-codeleted, 3) anaplastic pleomorphic the Korean Society for Neuro-Oncology (KSNO), a multidis- xanthoastrocytoma, 4) ependymoma, v-rel avian reticuloen- ciplinary academic society for CNS tumors [14], the actual dotheliosis viral oncogene homolog A (RELA) fusion-positive, guideline for the management of patients with other CNS tu- and 5) anaplastic ependymoma [4]. mors in Korea is not available yet. The KSNO established a In terms of genotypic characteristics, mutation status of Working Group for developing practice guideline for CNS tu- IDH1/2 and codeletion of 1p19q are more important to diag- mor in February 2018. Recently it is developing a guideline nose WHO grade III gliomas than methylation status of the for CNS tumor based on updated information. The objective O6-methyl guanine DNA methyltransferase (MGMT) gene of KSNO guideline is to provide physicians with evidence- promoter. Several WHO grade III gliomas in recent classifi- based recommendations and consensus expert opinion for cation have morphological characteristics of those in previ- managing patients with glioblastomas in daily clinical prac- ous pathological classification. If they do not have IDH-mu- tice. It will also serve as a source of knowledge for institutions tation, they have a behavior of WHO grade IV gliomas. and insurance companies involved in cancer care in Korea. Therefore, although these gliomas are not categorized as grade III, they should be treated with the same protocol for KSNO GUIDELINE WORKING GROUP WHO grade IV gliomas [4]. Despite WHO grade III gliomas have better outcome and A Working Group was appointed by the KSNO in Febru- longer survival than WHO grade IV gliomas, results of treat- ary 2018 to establish guidelines on the management of glio- ment are still very disappointing. There are certain obstacles blastoma patients. These guidelines should be optimized to overcome these tumors because of frequent recurrence, considering the unique medical circumstance in Korea. The common progression to higher grade gliomas, and refracto- KSNO Guideline Working Group was composed of 35 medi- riness to conventional therapies, etc. [6]. Therefore, many cal experts in Korea, including 18 neurosurgeons, 8 radiation clinical trials have been performed and many are also ongo- oncologists, 1 medical oncologist, 2 neuroradiologists, 3 pe- ing to overcome these serious diseases. Although there are diatric oncologists, 2 pathologists, and 1 neurologist. As no new therapeutic modalities such as those for WHO grade there is no medical specialty for neuro-oncology in Korea, IV gliomas, combinations of conventional cytotoxic chemo- neurosurgeons (especially brain tumor surgeons) usually therapy agents and other agents such as target therapy and/ play the role of neuro-oncologists in clinical practice. or immunotherapy have been developed [7-9]. To apply re- References were identified by searching PubMed, MED- cently established combinations or newly developed agents LINE, EMBASE, and Cochrane CENTRAL databases using for patients with grade III gliomas, comprehensive and ad- specific and sensitive keywords as well as combinations of vanced cancer networks or academic societies for CNS tu- keywords. Scope of the disease was confined to cerebral ana- mors of western countries such as the National Comprehen- plastic astrocytoma and anaplastic oligodendroglioma in sive Cancer Network (NCCN) [10-12] and the European adults aged ≥18 years because other WHO grade III gliomas Association for Neuro-Oncology (EANO) [13] are updating are relatively uncommon
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