Original Article
Chordoid glioma: a clinicopathological study
Wang Leiming, Shao Liwei, Cheng Bo, Zhao Huanying, Zhao Lihong, Yao Yingying, Gui Qiuping, Lu Dehong, Teng Lianghong
Published 2021-08-08
Cite as Chin J Pathol, 2021, 50(8): 865-869. DOI: 10.3760/cma.j.cn112151-20210111-00025
Abstract
ObjectiveTo analyze the clinicopathological features of chordoid glioma.
MethodsA total of 12 cases of chordoid gliomas from 2009 to 2020 in Xuanwu Hospital of Capital Medical University and General Hospital of Chinese People′s Liberation Army were retrospectively analyzed. The clinical and imaging characteristics, pathologic and molecular characteristics were analyzed, and the relevant literature was reviewed.
ResultsAll 12 patients (4 males and 8 females) aged from 25 to 67 years (mean 39 years) and mainly had a history of headache or/and vision loss. MRI showed that the lesions located in the third ventricle, and they showed abnormal enhancement. Pathologically, these 12 cases displayed the morphologic characteristics of chordoid gliomas, including papillary structures in two cases. Immunohistochemically, GFAP and vimentin were expressed in all 12 cases (12/12). TTF1 was also expressed in all cases (10/10). CD34 and CKpan were seen in 11 cases (11/12). EMA with dot-and/or-ring like positivity was seen in 9 cases (9/10). Tissues were available in nine chordoid gliomas for Sanger sequencing to detect PRKCA and IDH gene mutation, and eight cases (8/9) showed PRKCA gene D463H mutation. None of these cases showed IDH1 R132 and IDH2 R172 mutation. All 12 patients underwent surgery, and four were lost to follow up. The remaining eight patients were progression or recurrence free at last follow-up in January 2021.
ConclusionsChordoid gliomas have relatively distinguishing clinical and histopathological features. PRKCA gene mutation in chordoid gliomas can be considered as a biomarker for the diagnosis and differential diagnosis of chordoid gliomas, and may provide a direction for future targeted therapy.
Key words:
Glioma; DNA mutational analysis; Third ventricle
Contributor Information
Wang Leiming
Department of Pathology, Xuanwu Hospital, Capital Medical University, Beijing 100053, China
Shao Liwei
Department of Pathology, the First Medical Center of Chinese People′s Liberation Army General Hospital, Beijing 100853, China
Cheng Bo
Department of Pathology, Rocket Force Medical Center, Beijing 100088, China
Zhao Huanying
Genomics Research Platform Core Facilities Center, Capital Medical University, Beijing 100069, China
Zhao Lihong
Department of Pathology, Xuanwu Hospital, Capital Medical University, Beijing 100053, China
Yao Yingying
Department of Pathology, Xuanwu Hospital, Capital Medical University, Beijing 100053, China
Gui Qiuping
Department of Pathology, the First Medical Center of Chinese People′s Liberation Army General Hospital, Beijing 100853, China
Lu Dehong
Department of Pathology, Xuanwu Hospital, Capital Medical University, Beijing 100053, China
Teng Lianghong
Department of Pathology, Xuanwu Hospital, Capital Medical University, Beijing 100053, China
