Department of Medical Oncology, Cancer Institute Hospital of the Japanese Foundation for Cancer Research, Tokyo 135-0063, Japan.
Int J Mol Sci. 2018 Nov 28;19(12):3784. doi: 10.3390/ijms19123784.
Chromosomal translocations are observed in approximately 20% of soft tissue sarcomas (STS). With the advances in pathological examination technology, the identification of translocations has enabled precise diagnoses and classifications of STS, and it has been suggested that the presence of and differences in translocations could be prognostic factors in some translocation-related sarcomas. Most of the translocations in STS were not regarded as targets of molecular therapies until recently. However, trabectedin, an alkylating agent, has shown clinical benefits against translocation-related sarcoma based on a modulation of the transcription of the tumor's oncogenic fusion proteins. Many molecular-targeted drugs that are specific to translocations (e.g., anaplastic lymphoma kinase and tropomyosin kinase related fusion proteins) have emerged. The progress in gene technologies has allowed researchers to identify and even induce new translocations and fusion proteins, which might become targets of molecular-targeted therapies. In this review, we discuss the clinical significance of translocation-related sarcomas, including their diagnoses and targeted therapies.
染色体易位约占软组织肉瘤 (STS) 的 20%。随着病理检查技术的进步，易位的鉴定实现了 STS 的精确诊断和分类，并且已经提出易位的存在和差异可能是某些易位相关肉瘤的预后因素。直到最近，STS 中的大多数易位还不被认为是分子治疗的靶点。然而，基于对肿瘤致癌融合蛋白转录的调节，烷化剂 trabectedin 对易位相关肉瘤显示出临床益处。已经出现了许多针对易位的分子靶向药物（例如，间变性淋巴瘤激酶和原肌球蛋白激酶相关融合蛋白）。基因技术的进步使研究人员能够识别甚至诱导新的易位和融合蛋白，这些可能成为分子靶向治疗的靶点。在这篇综述中，我们讨论了易位相关肉瘤的临床意义，包括其诊断和靶向治疗。
