Solitary fibrous tumour is a rare, translocation-associated soft-tissue sarcoma that can be subclassified into typical, malignant, and dedifferentiated variants on the basis of the presence or absence of histological features that are associated with the more aggressive clinical phenotype. Having completed a single-arm phase 2 trial of the multikinase inhibitor, pazopanib, which included a malignant and dedifferentiated solitary fibrous tumour subgroup, Martin-Broto and colleagues now report on the efficacy and safety results of pazopanib in a prospectively defined subgroup of typical solitary fibrous tumour in The Lancet Oncology. In 31 patients with advanced typical solitary fibrous tumour who received treatment in the per-protocol population, pazopanib therapy was associated with radiological evidence of tumour response in 18 (58%) of the participants and after a median follow-up of 18 months (IQR 14–34), median overall survival was more than 4 years, which is markedly favourable compared with historical controls. On the basis of these findings, the authors propose that pazopanib could be considered a standard first-line therapy for typical solitary fibrous tumour.

中文翻译：

---

帕唑帕尼可作为晚期孤立性纤维性肿瘤的选择。

孤立性纤维瘤是一种罕见的易位相关的软组织肉瘤，可根据是否存在与更具侵略性的临床表型相关的组织学特征而分为典型的，恶性的和去分化的变体。Martin-Broto及其同事已经完成了多激酶抑制剂pazopanib的单臂2期试验，该试验包括恶性和去分化性孤立性纤维性肿瘤，该研究小组报告了pazopanib在典型孤立性亚组中的疗效和安全性结果。柳叶刀肿瘤学中的纤维性肿瘤。在按方案人群中接受治疗的31例晚期典型单发性纤维性肿瘤患者中，帕唑帕尼治疗与18例（58％）的受试者以及中位随访18个月（IQR）后的放射学证据相关14-34），中位总生存期超过4年，与历史对照组相比，这显然是有利的。基于这些发现，作者建议将帕唑帕尼视为典型的孤立性纤维性肿瘤的标准一线治疗方法。