异柠檬酸脱氢酶 1 和 2（IDH1 和 IDH2）参与人体细胞的关键代谢过程，调节分化、增殖和氧化损伤反应。 IDH突变与神经胶质瘤、胆管癌、软骨肉瘤和其他肿瘤类型等各种实体瘤的发生和进展有关，并已成为分子分类和预后评估的关键标志物。 D-2-羟基戊二酸 (D-2-HG) 的瘤内和血清水平可作为识别 IDH 突变 (IDHmut) 肿瘤的诊断生物标志物。因此，越来越多的临床试验正在评估 IDH1/IDH2 突变的靶向治疗。最近的研究表明，这些新治疗策略的重点不仅在于 IDHmut 酶的新形态活性，还在于 IDH 突变诱导的表观遗传转变以及联合治疗的潜在作用。在这里，我们概述了实体瘤中 IDH 突变的当前知识，特别关注可用的 IDH 靶向治疗和临床试验的新结果，旨在探索 IDHmut 肿瘤特异性特征并确定 IDH 的临床益处靶向治疗及其联合策略。还包括对未来前景以及循环生物标志物和放射组学特征的新兴作用的见解。

The isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) enzymes are involved in key metabolic processes in human cells, regulating differentiation, proliferation, and oxidative damage response. IDH mutations have been associated with tumor development and progression in various solid tumors such as glioma, cholangiocarcinoma, chondrosarcoma, and other tumor types and have become crucial markers in molecular classification and prognostic assessment. The intratumoral and serum levels of D-2-hydroxyglutarate (D-2-HG) could serve as diagnostic biomarkers for identifying IDH mutant (IDHmut) tumors. As a result, an increasing number of clinical trials are evaluating targeted treatments for IDH1/IDH2 mutations. Recent studies have shown that the focus of these new therapeutic strategies is not only the neomorphic activity of the IDHmut enzymes but also the epigenetic shift induced by IDH mutations and the potential role of combination treatments. Here, we provide an overview of the current knowledge about IDH mutations in solid tumors, with a particular focus on available IDH-targeted treatments and emerging results from clinical trials aiming to explore IDHmut tumor-specific features and to identify the clinical benefit of IDH-targeted therapies and their combination strategies. An insight into future perspectives and the emerging roles of circulating biomarkers and radiomic features is also included.