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开启新视野:IDH 突变型、1p/19q 共缺失少突胶质细胞瘤的治疗进展

Unlocking new horizons: advances in treating IDH-mutant, 1p/19q-codeleted oligodendrogliomas.

作者信息

Bao Jing, Pan Zhenjiang, Wei Shepeng

机构信息

Department of Neurosurgery, Shidong Hospital, No. 999, Shiguang Road, Yangpu District, Shanghai, 200438, China.

出版信息

Discov Oncol. 2025 May 31;16(1):971. doi: 10.1007/s12672-025-02815-6.

Abstract

Oligodendrogliomas are a distinct subtype of diffuse gliomas characterized by IDH mutations and 1p/19q codeletion, classified as grade 2 or 3 based on histological features. This review examines current advancements in the diagnosis, treatment, and prognosis of oligodendrogliomas, with an emphasis on personalized approaches driven by molecular insights. Surgery remains the cornerstone of treatment, aiming for maximal safe resection to obtain tissue for diagnosis and alleviate symptoms. For grade 2 tumors with residual disease but no symptomatic progression, the IDH inhibitor vorasidenib has emerged as a promising option to delay the need for radiation therapy (RT) and chemotherapy. For grade III oligodendrogliomas, postoperative combined-modality therapy with RT and chemotherapy, such as the PCV regimen, demonstrates significant survival benefits, while temozolomide is an alternative due to its ease of administration and reduced toxicity. Recurrent oligodendrogliomas present therapeutic challenges, necessitating tailored strategies based on prior treatments and the interval since initial therapy. Options include repeat surgery, reirradiation, or novel targeted therapies. Advances in molecular diagnostics, such as homozygous CDKN2A/B deletion as a prognostic marker, have refined risk stratification and informed treatment decisions. Despite these strides, further research is needed to optimize long-term outcomes and address resistance mechanisms. This review underscores the importance of integrating molecular diagnostics with clinical management to achieve personalized, evidence-based care for patients with oligodendrogliomas.

摘要

少突胶质细胞瘤是弥漫性胶质瘤的一种独特亚型,其特征为异柠檬酸脱氢酶(IDH)突变和1p/19q共缺失,根据组织学特征分为2级或3级。本综述探讨了少突胶质细胞瘤在诊断、治疗和预后方面的当前进展,重点关注由分子见解驱动的个性化方法。手术仍然是治疗的基石,目标是最大程度地安全切除肿瘤,以获取组织用于诊断并缓解症状。对于有残留病灶但无症状进展的2级肿瘤,IDH抑制剂沃拉西尼已成为一种有前景的选择,可延迟放疗(RT)和化疗的需求。对于III级少突胶质细胞瘤,术后采用RT和化疗的联合治疗方案,如PCV方案,显示出显著的生存益处,而替莫唑胺因其给药方便且毒性较低,是一种替代选择。复发性少突胶质细胞瘤带来了治疗挑战,需要根据先前的治疗方法和初始治疗后的时间间隔制定针对性策略。选择包括再次手术、再放疗或新型靶向治疗。分子诊断方面的进展,如纯合子CDKN2A/B缺失作为预后标志物,已完善了风险分层并为治疗决策提供了依据。尽管取得了这些进展,但仍需要进一步研究以优化长期疗效并解决耐药机制。本综述强调了将分子诊断与临床管理相结合的重要性,以便为少突胶质细胞瘤患者提供个性化的循证护理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/16fc/12126372/a56f7643aaf7/12672_2025_2815_Fig1_HTML.jpg

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