Mesenchymal chondrosarcoma (MCS) is a rare subtype of chondrosarcoma with a poor prognosis. Although these tumors are sensitive to radiotherapy/chemotherapy, the standard treatment for localized MCS is only surgical resection, and there are no established treatment guidelines for patients with advanced and metastatic MCS. Due to the low incidence of MCS, the pathology of these tumors is still unknown, and other therapeutic options are lacking. Some studies show the potential role of the PDGF/PPI3K/AKT, PKC/RAF/MEK/ERK, and pRB pathways, and BCL2 overexpression in the pathogenesis of MCS. These findings provide an opportunity to use protein kinases and BCL2 inhibitors as potential therapy in MCS. In this review, we summarize the current knowledge about MCS diagnosis and treatment options. We show the immunological and molecular biomarkers used in the diagnosis of MCS. In addition, we discuss the known prognostic and predictive factors in MCS. Finally, we present the novel trends, including targeted therapies and ongoing clinical trials using protein kinase inhibitors and the death receptor 5 (DR5) agonist, which may be the focus of future MCS treatment studies.
间叶性软骨肉瘤是一种罕见的软骨肉瘤亚型，预后较差。尽管这类肿瘤对放疗/化疗敏感，但局限性间叶性软骨肉瘤的标准治疗仅为手术切除，且针对晚期及转移性患者尚未建立明确的治疗指南。由于发病率较低，该肿瘤的病理机制尚不明确，其他治疗选择也相对匮乏。研究表明PDGF/PPI3K/AKT、PKC/RAF/MEK/ERK信号通路及pRB通路异常，以及BCL2过表达可能在间叶性软骨肉瘤的发病机制中发挥潜在作用。这些发现为使用蛋白激酶抑制剂和BCL2抑制剂作为潜在治疗方案提供了可能。本文综述了当前关于间叶性软骨肉瘤诊断与治疗的研究进展，展示了用于诊断的免疫学及分子生物学标志物，并探讨了已知的预后及预测因素。最后，我们介绍了包括靶向治疗在内的新趋势，以及正在进行的蛋白激酶抑制剂和死亡受体5激动剂的临床试验，这些可能成为未来间叶性软骨肉瘤治疗研究的重点方向。
Mesenchymal Chondrosarcoma from Diagnosis to Clinical Trials
肿瘤(癌症)患者之家