综述与专论: 脊髓星形细胞瘤研究进展
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作者单位:

1)首都医科大学,北京市神经外科研究所分子神经病理室,北京 100070;2)首都医科大学附属北京天坛医院,北京 100070

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基金项目:

北京市科技新星计划(Z201100006820118) 和国家自然科学基 金(8217100897) 资助项目


Reviews and Monographs: Research Progress on Astrocytoma of Spinal Cord
Author:
Affiliation:

1)Department of Molecular Neuropathology, Beijing Neurosurgical Institute, Capital Medical University, Beijing 100070, China;2)Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China

Fund Project:

This work was supported by grants from Beijing Science and Technology Nova Program (Z201100006820118) and The National Natural Science Foundation of China (8217100897) .

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    摘要:

    脊髓星形细胞瘤是一种罕见的中枢神经系统恶性肿瘤,在流行病学、肿瘤临床学表型、分子遗传标记、治疗及研究方面有着独特特征。虽然随着手术技术的进步以及分子病理的发展,脑胶质瘤的研究和治疗取得较大进展,但脊髓星形细胞瘤的研究和治疗却发展缓慢。其原因一方面在于临床样本较少,难以开展研究,另一方面因其分子遗传独特性,对脑胶质瘤一线化疗药替莫唑胺敏感性差。因而亟需理清脊髓星形细胞瘤的研究现状,为改善其临床疗效梳理潜在方向。基于此,本文综述脊髓星形细胞瘤的临床特征、病理分型、分子遗传特征和当前治疗方法等方面的研究进展,在描绘脊髓星形细胞瘤的临床治疗现状和研究进展的基础上,提出了未来研究和治疗潜在方向。

    Abstract:

    Spinal cord astrocytoma is a rare malignant tumor of the central nervous system, with unique characteristics in epidemiology, clinical tumor phenotype, molecular genetic markers, and therapeutic research. With the development of surgical techniques and molecular pathology, significant progress has been made in the research and treatment of brain glioma. However, there are only limited advances in the research and treatment of spinal cord astrocytoma. The potential cautions include: (1) it is difficult to carry out research because of the small number of clinical samples; (2) the resistance of the spinal cord to temozolomide, the first-line chemotherapy drug for brain glioma. Therefore, it is urgent to clarify the research status and potential direction of spinal cord astrocytoma to provide clues for improving its clinical efficacy. Here, we comprehensively reviewed the clinical features, pathological classification, molecular characteristics, current treatments, and ongoing studies of spinal cord astrocytoma. In conclusion, the incidence of spinal astrocytoma is less than 1/10 of brain diffuse gliomas. Although spinal cord astrocytoma has younger age than brain hemisphere diffuse glioma, it mainly occurs in adults which is unlike diffuse brain stem gliomas. According to histological phenotype, spinal cord astrocytomas are divided into astrocytoma and glioblastoma. Almost all patients are IDH wild-type, and a high proportion of patients carry the H3 K27M mutation (about 40%). For WHO grade 2/3 patients, maximal surgical resection was associated with a better prognosis, but for WHO grade 4 patients, the prognosis was associated with histological grade, preoperative spinal cord function, and NLR (peripheral blood neutrophil to lymphocyte ratio). Currently, there is no effective treatment for spinal cord astrocytoma other than radiotherapy. Studies on molecular targeted therapy and immunotherapy have brought new hope for spinal cord glioma, but the lack of effective experiment models has limited their progression. Studies in large clinical cohorts, the development of cell and animal experiment models, and the usage of novel study approaches (such as single-cell technology) should be performed as soon as possible on the spinal cord astrocytoma to improve its precision diagnosis and therapy.

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庞波,王永志,柴睿超.综述与专论: 脊髓星形细胞瘤研究进展[J].生物化学与生物物理进展,2022,49(11):2142-2149

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历史
  • 收稿日期:2022-08-31
  • 最后修改日期:2022-10-18
  • 接受日期:2022-10-11
  • 在线发布日期: 2022-11-22
  • 出版日期: 2022-11-20