Yang Hujuan, Qin Zhiquan, He Xianglei, Xue Qian, Zhou Hongying, Sun Jie, Li Xiaoyi, Zhao Tongwei
Graduate School of Clinical Medicine, Bengbu Medical College, Bengbu, Anhui, China.
Cancer Center, Department of Medical Oncology, Zhejiang Provincial People's Hospital (Affiliated People's Hospital, Hangzhou Medical College), Hangzhou, Zhejiang, China.
Front Oncol. 2023 Apr 5;13:1110997. doi: 10.3389/fonc.2023.1110997. eCollection 2023.
Undifferentiated pleomorphic sarcoma (UPS) is a rare and aggressive soft tissue tumor with a high degree of malignancy and rapid progression, usually occurring in the extremities, retroperitoneum, and abdomen, whereas it rarely arises in the mediastinum, and is treated mainly by surgical resection. The prognosis of patients with advanced sarcoma is poor, and doxorubicin monotherapy is the standard first-line chemotherapy for most advanced soft tissue sarcomas (STS), but the prognosis is generally unsatisfactory. Immune checkpoint inhibitors (ICIs) have been established as therapies for many solid cancers in recent years; however, evidence on the efficacy of ICIs in undifferentiated sarcoma is scarce, mostly consisting of small studies, and no ICIs are currently approved for use in sarcomas. We report a case of a middle-aged man with primary mediastinal UPS with high PD-L1 expression (TPS was approximately 80%) and TLS positive. The patient was treated with sequential tislelizumab monotherapy maintenance after 6 cycles of tislelizumab combined with epirubicin, efficacy evaluation was partial remission (PR), progression-free survival (PFS) was 8.5 months, and grade 1 fatigue was identified as an adverse event.
未分化多形性肉瘤（UPS）是一种罕见的侵袭性软组织肿瘤，恶性程度高，进展迅速，通常发生于四肢、腹膜后和腹部，而极少发生于纵隔，主要通过手术切除进行治疗。晚期肉瘤患者的预后较差，多柔比星单药治疗是大多数晚期软组织肉瘤（STS）的标准一线化疗方案，但总体预后通常不尽人意。近年来，免疫检查点抑制剂（ICI）已成为多种实体癌的治疗方法；然而，关于ICI在未分化肉瘤中疗效的证据很少，大多是小型研究，目前尚无ICI被批准用于肉瘤治疗。我们报告一例中年男性原发性纵隔UPS患者，其程序性死亡受体配体1（PD-L1）高表达（肿瘤比例评分约为80%）且三级淋巴结构（TLS）阳性。该患者在接受6个周期的替雷利珠单抗联合表柔比星治疗后，序贯替雷利珠单抗单药维持治疗，疗效评估为部分缓解（PR），无进展生存期（PFS）为8.5个月，且发现1级疲劳为不良事件。
