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脊索样胶质瘤:一种在放射学、组织学和临床上均罕见且令人困惑的病变。

Chordoid glioma: a rare radiologically, histologically, and clinically mystifying lesion.

作者信息

Bongetta Daniele, Risso Andrea, Morbini Patrizia, Butti Giorgio, Gaetani Paolo

机构信息

Neurosurgery, Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, Università degli Studi di Pavia, Piazzale Golgi 19, 27100, Pavia, Italy.

Unit of Pathology, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico S. Matteo, Piazzale Golgi 19, 27100, Pavia, Italy.

出版信息

World J Surg Oncol. 2015 May 28;13:188. doi: 10.1186/s12957-015-0603-9.

DOI:10.1186/s12957-015-0603-9
PMID:26018908
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4453048/
Abstract

Chordoid glioma (CG) is a rare central nervous system neoplasm (WHO grade II) of uncertain origin whose typical localization is in the anterior part of the third ventricle. Its clinical, radiological, and histological features may vary and furthermore mimic other kind of benign lesions usually associated with a better outcome. We report a case of a 43-year-old female who underwent gross total removal of a lesion of the third ventricle causing hydrocephalus. The imaging studies and the intraoperative examination led at first to a hypothesis of meningioma. Early surgical and neurological outcomes were good. The patient underwent multiple complications related to hypothalamic dysfunctions and thrombohemorragic issues and eventually died because of systemic infections. Definitive examination was of chordoid glioma of the third ventricle. Reviewing literature, we evaluated possible pitfalls in radiological and histological diagnosis as well as in surgical and medical treatment of CGs. Despite their benign presentation, a high incidence of multiple possible severe complications is reported. Early alertness and combined treatment strategies could improve overall CGs treatment strategies.

摘要

脊索样胶质瘤(CG)是一种起源不明的罕见中枢神经系统肿瘤(世界卫生组织二级),其典型定位在第三脑室前部。其临床、放射学和组织学特征可能各不相同,而且可能会与其他通常预后较好的良性病变相似。我们报告一例43岁女性患者,其第三脑室病变导致脑积水,接受了病变全切手术。影像学检查和术中检查最初提示为脑膜瘤。早期手术和神经功能预后良好。该患者出现了与下丘脑功能障碍和血栓出血问题相关的多种并发症,最终因全身感染死亡。最终检查确诊为第三脑室脊索样胶质瘤。通过回顾文献,我们评估了在脊索样胶质瘤的放射学和组织学诊断以及手术和药物治疗中可能存在的陷阱。尽管其表现为良性,但据报道其多种严重并发症的发生率较高。早期警觉和联合治疗策略可能会改善脊索样胶质瘤的整体治疗策略。

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Ann Med Surg (Lond). 2025 Mar 19;87(4):2504-2509. doi: 10.1097/MS9.0000000000003199. eCollection 2025 Apr.
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Glioma: bridging the tumor microenvironment, patient immune profiles and novel personalized immunotherapy.胶质瘤:连接肿瘤微环境、患者免疫特征与新型个性化免疫疗法
Front Immunol. 2024 Jan 11;14:1299064. doi: 10.3389/fimmu.2023.1299064. eCollection 2023.
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Chordoid glioma in the thalamus of a child: Rare location and atypical imaging findings.

本文引用的文献

1
Expression of thyroid transcription factor 1 in a chordoid glioma.甲状腺转录因子1在脊索样胶质瘤中的表达
J Neurol Sci. 2014 Nov 15;346(1-2):362-3. doi: 10.1016/j.jns.2014.09.005. Epub 2014 Sep 16.
2
Therapeutic approach to chordoid glioma of the third ventricle.第三脑室脊索样胶质瘤的治疗方法。
Neurol Med Chir (Tokyo). 2013;53(4):249-55. doi: 10.2176/nmc.53.249.
3
From the radiologic pathology archives: intraventricular neoplasms: radiologic-pathologic correlation.从放射病理学档案中:脑室内肿瘤:放射病理学相关性。
儿童丘脑的脊索样胶质瘤:罕见部位及非典型影像学表现。
BJR Case Rep. 2021 Jan 8;7(3):20200108. doi: 10.1259/bjrcr.20200108. eCollection 2021 May 1.
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Occurrence of Chordoid Glioma With Sodium Ion Metabolism Disorder 5 Years After Meningioma Surgery and Whole-Exome Sequencing: A Case Report and Literature Review.脑膜瘤手术后5年伴钠离子代谢紊乱的脊索样胶质瘤的发生及全外显子测序:1例报告及文献复习
Front Genet. 2021 May 10;12:617575. doi: 10.3389/fgene.2021.617575. eCollection 2021.
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Chordoid Glioma of the Third Ventricle: A Case Report and a Treatment Strategy to This Rare Tumor.第三脑室脊索样胶质瘤:一例报告及针对这种罕见肿瘤的治疗策略
Front Oncol. 2020 Apr 9;10:502. doi: 10.3389/fonc.2020.00502. eCollection 2020.
6
Chordoid Glioma as a Differential Diagnosis of Anterior Third Ventricle Tumours: A Rare Case Report and Five-Year Follow-Up.脊索样胶质瘤作为第三脑室前部肿瘤的鉴别诊断:1例罕见病例报告及5年随访
Case Rep Radiol. 2019 Dec 4;2019:3584837. doi: 10.1155/2019/3584837. eCollection 2019.
7
Usefulness of neuroimaging and immunohistochemical study for accurate diagnosis of chordoid glioma of the third ventricle: A case report and review of the literature.神经影像学和免疫组织化学研究对准确诊断第三脑室脊索样胶质瘤的价值:一例报告并文献复习
Surg Neurol Int. 2018 Nov 2;9:226. doi: 10.4103/sni.sni_306_18. eCollection 2018.
8
A recurrent point mutation in PRKCA is a hallmark of chordoid gliomas.PRKCA 中的反复突变是脊索样胶质瘤的一个标志。
Nat Commun. 2018 Jun 18;9(1):2371. doi: 10.1038/s41467-018-04622-w.
9
Neurogenic fever in a patient with a chordoid glioma.一名患有脊索样胶质瘤患者的神经性发热
BMJ Case Rep. 2016 Nov 17;2016:bcr2016218205. doi: 10.1136/bcr-2016-218205.
Radiographics. 2013 Jan-Feb;33(1):21-43. doi: 10.1148/rg.331125192.
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Neuropathology. 2013 Apr;33(2):134-9. doi: 10.1111/j.1440-1789.2012.01333.x. Epub 2012 Jun 21.
5
Chordoid glioma in suprasellar location with extension into the third ventricle: smear preparation morphology of a rare tumor.鞍上部位的脊索样胶质瘤并延伸至第三脑室:一种罕见肿瘤的涂片制备形态学
Diagn Cytopathol. 2012 Feb;40(2):155-8. doi: 10.1002/dc.21606. Epub 2011 Feb 13.
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CD99 is expressed in chordoid glioma and suggests ependymal origin.CD99在脊索样胶质瘤中表达,提示其起源于室管膜。
Virchows Arch. 2012 Jan;460(1):119-22. doi: 10.1007/s00428-011-1170-2. Epub 2011 Nov 20.
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Radiographics. 2011 Nov-Dec;31(7):1889-905. doi: 10.1148/rg.317115083.
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Chordoid glioma: a case report and literature review.脊索样胶质瘤:一例报告及文献综述
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Skull Base. 2010 Mar;20(2):125-38. doi: 10.1055/s-0029-1246223.
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Diabetes insipidus, panhypopituitarism, and severe mental status deterioration in a patient with chordoid glioma: case report and literature review.脊索样胶质瘤患者出现尿崩症、全垂体功能减退和严重精神状态恶化:病例报告及文献综述
Endocr Pract. 2009 Apr;15(3):240-5. doi: 10.4158/EP.15.3.240.