免疫疗法已成为治疗肉瘤的一种有希望的治疗方法，但组织学、临床行为和治疗反应的高度变异性决定了其在这些肿瘤中的作用面临着特殊的挑战。肉瘤的肿瘤免疫微环境（TiME）反映了这些肿瘤起源于间充质细胞并涵盖 100 多种组织学的异质性。对 TiME 复杂性理解的进展导致肉瘤免疫治疗反应性的改善，但一开始的结果令人失望。基于免疫细胞群和肿瘤细胞之间的相互作用而提出的肉瘤免疫分类显示出对免疫疗法具有预后和潜在的预测作用。几项研究探讨了免疫疗法对这些组织型管理的临床影响，导致了有争议的结果。肿瘤浸润淋巴细胞（TIL）的存在似乎与患者生存率的改善以及对免疫治疗的更高反应性相关。在这种情况下，重要的是要考虑到免疫相关基因（IRG）也已被证明在肿瘤发生和肿瘤免疫微环境的构建中发挥着关键作用。软组织和骨肉瘤中 IRG 的特征是几个肿瘤相关基因之间的联系，这些基因可以发挥潜在的预后和预测治疗作用。在本文中，我们回顾了肉瘤治疗中主要免疫策略的最新技术，包括其临床和转化相关性。版权所有 © 2024 Recine, Vanni, Bongiovanni, Fausti, Mercatali, Miserocchi, Liverani, Pieri, Casadei, Cavaliere,法尔博、迪亚诺、易卜拉欣和德维塔。

Immunotherapy has emerged as promising treatment in sarcomas, but the high variability in terms of histology, clinical behavior and response to treatments determines a particular challenge for its role in these neoplasms. Tumor immune microenvironment (TiME) of sarcomas reflects the heterogeneity of these tumors originating from mesenchymal cells and encompassing more than 100 histologies. Advances in the understanding of the complexity of TiME have led to an improvement of the immunotherapeutic responsiveness in sarcomas, that at first showed disappointing results. The proposed immune-classification of sarcomas based on the interaction between immune cell populations and tumor cells showed to have a prognostic and potential predictive role for immunotherapies. Several studies have explored the clinical impact of immune therapies in the management of these histotypes leading to controversial results. The presence of Tumor Infiltrating Lymphocytes (TIL) seems to correlate with an improvement in the survival of patients and with a higher responsiveness to immunotherapy. In this context, it is important to consider that also immune-related genes (IRGs) have been demonstrated to have a key role in tumorigenesis and in the building of tumor immune microenvironment. The IRGs landscape in soft tissue and bone sarcomas is characterized by the connection between several tumor-related genes that can assume a potential prognostic and predictive therapeutic role. In this paper, we reviewed the state of art of the principal immune strategies in the management of sarcomas including their clinical and translational relevance.Copyright © 2024 Recine, Vanni, Bongiovanni, Fausti, Mercatali, Miserocchi, Liverani, Pieri, Casadei, Cavaliere, Falbo, Diano, Ibrahim and De Vita.