Purpose: Alveolar soft part sarcoma (ASPS) is an ultra-rare soft-tissue sarcoma with a high rate of metastasis and no established treatment. This study aimed to explore the efficacy and safety of anlotinib (a tyrosine-kinase inhibitor) and TQB2450 (a PD-L1 inhibitor) in ASPS patients. Methods: This single-arm, phase 2 study evaluated the efficacy of TQB2450, an anti-programmed death ligand 1 (PD-L1) agent, combined with anlotinib, a TKI, in adults with advanced ASPS. TQB2450 was given intravenously (1,200 mg) on day 1, and anlotinib (12 mg/day) was taken orally from day 1 to day 14 every 3 weeks. The primary endpoint was overall response rate, with secondary endpoints including duration of response (DOR), progression-free survival (PFS), and overall survival (OS). Lymphocyte infiltration and tertiary lymphoid structure (TLS) were also analyzed as potential prognostic biomarkers. Results: The study enrolled 29 patients, with 28 evaluable (one withdrew due to acute pancreatitis). An objective response was achieved in 82.1% of patients, including 4 complete and 19 partial responses. The median time to response was 2.8 months, and the DOR was not reached, with an estimated median PFS of 35.2 months. Grade 3-4 treatment-related adverse events occurred in 44.8% of patients, with no study-related deaths. Responders had a higher proportion of TLS area, density, and CD20-positive immune cells. Conclusions: The combination of anlotinib and TQB2450 is effective and tolerable in ASPS patients. TLS may serve as a prognostic biomarker, meriting further investigation.

中文翻译：

---

安罗替尼联合 TQB2450（一种 PD-L1 抗体）治疗晚期肺泡软部分肉瘤患者：一项单臂 2 期试验


目的：肺泡软部分肉瘤 （ASPS） 是一种极其罕见的软组织肉瘤，转移率高，尚无固定治疗方法。本研究旨在探讨安罗替尼 （一种酪氨酸激酶抑制剂） 和 TQB2450 （一种 PD-L1 抑制剂） 在 ASPS 患者中的疗效和安全性。方法： 这项单臂 2 期研究评估了 TQB2450（一种抗程序性死亡配体 1 （PD-L1） 药物）联合安罗替尼（一种 TKI）对晚期 ASPS 成人患者的疗效。第 1 天静脉注射 TQB2450 （1,200 mg），第 1 天至第 14 天每 3 周口服安罗替尼 （12 mg/天）。主要终点是总缓解率，次要终点包括缓解持续时间 （DOR） 、无进展生存期 （PFS） 和总生存期 （OS）。淋巴细胞浸润和三级淋巴结构 （TLS） 也作为潜在的预后生物标志物进行分析。结果： 该研究招募了 29 名患者，其中 28 名可评估 （1 名因急性胰腺炎退出）。82.1% 的患者达到客观反应，包括 4 例完全缓解和 19 例部分缓解。中位缓解时间为 2.8 个月，未达到 DOR，估计中位 PFS 为 35.2 个月。44.8% 的患者发生 3-4 级治疗相关不良事件，无研究相关死亡。应答者的 TLS 面积、密度和 CD20 阳性免疫细胞比例较高。结论： 安罗替尼联合 TQB2450 对 ASPS 患者有效且可耐受。TLS 可能作为预后生物标志物，值得进一步研究。