Schmitt Thomas, Kasper Bernd
University of Heidelberg, Department of Internal Medicine V, Germany.
Expert Rev Anticancer Ther. 2009 Aug;9(8):1159-67. doi: 10.1586/era.09.64.
Soft-tissue sarcomas (STSs) are a heterogeneous group of rare malignant tumors predominantly arising from the embryonic mesoderm. The mainstay of curative therapy is the complete surgical resection of all tumor manifestations with negative histological margins. However, up to 50% of patients will develop distant metastases during the course of their disease. The prognosis for those patients is grim with a 5-year overall survival of less than 10%. First-line systemic therapy with ifosfamide and doxorubicin results in overall response rates of only 20% by conventional response evaluation criteria in solid tumors (RECIST). However, stabilization of disease can be seen in a greater proportion. Therefore, the role of the RECIST criteria has been questioned and the implementation of new imaging studies (e.g., FDG-PET) has shown promising results in assessing early tumor response to therapy. Furthermore, a broader insight into the molecular pathways of sarcomagenesis has been gained in recent years, revealing intriguing targets for new therapeutic approaches (e.g., VEGF, VEGF receptor, IGF receptor, EGF receptor, mTOR and cyclin-dependent kinases). In addition, a growing body of evidence is linking specific genetic aberrations with clinical outcome (e.g., SYT-SXX translocation in synovial sarcoma). With further insight into the biology of STS and the combination of new treatment options with modern imaging techniques, we will most certainly be able to improve clinical outcome in patients with STS in the upcoming years.
软组织肉瘤（STSs）是一组异质性的罕见恶性肿瘤，主要起源于胚胎中胚层。根治性治疗的主要方法是对所有肿瘤表现进行完整的手术切除，且组织学切缘阴性。然而，高达50%的患者在病程中会发生远处转移。这些患者的预后很差，5年总生存率不到10%。按照实体瘤的传统疗效评价标准（RECIST），采用异环磷酰胺和阿霉素进行一线全身治疗的总缓解率仅为20%。然而，更大比例的患者病情可得到稳定。因此，RECIST标准的作用受到质疑，新的影像学检查（如FDG-PET）在评估肿瘤对治疗的早期反应方面已显示出有希望的结果。此外，近年来对肉瘤发生的分子途径有了更广泛的认识，揭示了新治疗方法的有趣靶点（如VEGF、VEGF受体、IGF受体、EGF受体、mTOR和细胞周期蛋白依赖性激酶）。此外，越来越多的证据将特定的基因畸变与临床结果联系起来（如滑膜肉瘤中的SYT-SXX易位）。随着对软组织肉瘤生物学的进一步了解以及新治疗方案与现代成像技术的结合，我们肯定能够在未来几年改善软组织肉瘤患者的临床结果。
