Myxofibrosarcoma (MFS) is a malignant soft tissue sarcoma (STS) that originates in the body’s connective tissues. It is characterized by the presence of myxoid (gel-like) and fibrous components and typically affects patients after the fifth decade of life. Considering the ongoing trend of increasing lifespans across many nations, MFS is likely to become the most common musculoskeletal sarcoma in the future. Although MFS patients have a lower risk of developing distant metastases compared with other STS cases, MFS is characterized by a high frequency of local recurrence. Notably, in 40–60% of the patients where the tumor recurs, it does so multiple times. Consequently, patients may undergo multiple local surgeries, removing the risk of potential amputation. Furthermore, because the tumor relapses generally have a higher grade, they exhibit a decreased response to radio and chemotherapy and an increased tendency to form metastases. Thus, a better understanding of MFS is required, and improved therapeutic options must be developed. Historically, preclinical models for other types of tumors have been instrumental in obtaining a better understanding of tumor development and in testing new therapeutic approaches. However, few MFS models are currently available. In this review, we will describe the MFS models available and will provide insights into the advantages and constraints of each model.
黏液纤维肉瘤是一种起源于人体结缔组织的恶性软组织肉瘤，其病理特征表现为黏液样（胶状）与纤维成分共存，好发于50岁以上人群。随着全球多国人口预期寿命持续增长的趋势，该肿瘤未来可能成为最常见的肌肉骨骼系统肉瘤。相较于其他软组织肉瘤，黏液纤维肉瘤患者发生远处转移的风险较低，但具有显著的局部高复发特性。值得注意的是，在40%-60%的复发患者中会出现多次复发。因此患者可能需接受多次局部手术，甚至面临截肢风险。更值得关注的是，复发肿瘤通常恶性程度更高，表现为对放化疗敏感性降低且转移倾向增强。这提示我们需要深化对黏液纤维肉瘤的认知，并开发更有效的治疗方案。从肿瘤研究发展史来看，临床前模型对于理解肿瘤发生机制及测试新型疗法具有关键作用，但目前可用的黏液纤维肉瘤模型极为有限。本综述将系统阐述现有黏液纤维肉瘤模型，并深入剖析各类模型的优势与局限性。
Modeling Myxofibrosarcoma: Where Do We Stand and What Is Missing?
肿瘤(癌症)患者之家