Radiation-associated sarcomas (RASs) are rare tumors with limited contemporary data to inform prognostication and management. We sought to identify the clinical presentation, patterns of care, and prognostic factors of RASs. RAS patients treated at a single institution from 2015 to 2021 were retrospectively reviewed for clinicopathologic variables, treatment strategies, and outcomes. Thirty-eight patients were identified with a median follow-up of 30.5 months. The median age at RAS diagnosis was 68.4 years (27.9–85.4), with a median latency from index radiotherapy (RT) of 9.1 years (3.7–46.3). RAS histologies included angiosarcoma (26%), undifferentiated pleomorphic sarcoma (21%), and osteosarcoma (18%). Most were high-grade (76%). Genomic profiling revealed low tumor mutational burden, frequent inactivating TP53 mutations (44%), CDKN2A deletions (26%), and MYC amplifications (22%), particularly in breast angiosarcomas. Of 38 patients, 33 presented with localized disease, 26 of whom were treated with curative intent. Overall, the median progression-free survival (PFS) was 9.5 months (1.4–34.7), and the overall survival (OS) was 11.1 months (0.6–31.6). Patients with localized vs. metastatic RASs had a longer PFS (HR, 3.0 [1.1–8.5];p= 0.03) and OS (HR, 3.0 [1.04–8.68];p= 0.03). Among localized RAS patients, high grade was associated with shorter OS (HR, 4.6 [1.04–20.30];p= 0.03) and resection with longer OS (mean 58.8 vs. 6.1 months, HR, 0.1 [0.03–0.28];p< 0.001). Among patients undergoing resection, negative margins were associated with improved OS (mean 71.0 vs. 15.5 months, HR, 5.1 [1.4–18.2];p= 0.006). Patients with localized disease, particularly those undergoing R0 resection, demonstrated significantly better outcomes. Novel strategies are urgently needed to improve treatment outcomes in this challenging group of diseases.
放射相关肉瘤（RASs）是一种罕见肿瘤，目前关于其预后评估和治疗策略的当代数据有限。本研究旨在明确RASs的临床表现、诊疗模式及预后因素。我们对2015年至2021年间在同一机构接受治疗的RAS患者进行了回顾性分析，收集其临床病理学特征、治疗策略及预后数据。共纳入38例患者，中位随访时间为30.5个月。RAS诊断时的中位年龄为68.4岁（范围27.9–85.4），从初始放射治疗（RT）到发生RAS的中位潜伏期为9.1年（范围3.7–46.3）。RAS的组织学类型包括血管肉瘤（26%）、未分化多形性肉瘤（21%）和骨肉瘤（18%）。大多数为高级别肿瘤（76%）。基因组分析显示肿瘤突变负荷较低，常见TP53失活突变（44%）、CDKN2A缺失（26%）和MYC扩增（22%），尤其在乳腺血管肉瘤中多见。38例患者中，33例为局限性病变，其中26例接受了根治性治疗。总体中位无进展生存期（PFS）为9.5个月（范围1.4–34.7），总生存期（OS）为11.1个月（范围0.6–31.6）。与转移性RAS患者相比，局限性RAS患者的PFS（风险比HR=3.0 [1.1–8.5]；p=0.03）和OS（HR=3.0 [1.04–8.68]；p=0.03）更长。在局限性RAS患者中，高级别与较短的OS相关（HR=4.6 [1.04–20.30]；p=0.03），而手术切除则与更长的OS相关（平均58.8个月 vs. 6.1个月，HR=0.1 [0.03–0.28]；p<0.001）。在接受手术切除的患者中，切缘阴性者的OS显著改善（平均71.0个月 vs. 15.5个月，HR=5.1 [1.4–18.2]；p=0.006）。局限性病变患者，尤其是接受R0切除者，预后显著更优。针对这类难治性疾病，亟需探索新的治疗策略以改善患者预后。
Clinical Characteristics, Patterns of Care, and Treatment Outcomes of Radiation-Associated Sarcomas
肿瘤(癌症)患者之家