Department of Pathology, San Antonio Uniformed Services Health Education Consortium, San Antonio, TX, USA.
Department of Laboratory Medicine and Pathology, University of Washington, Seattle, WA, USA.
Int J Surg Pathol. 2023 Aug;31(5):532-547. doi: 10.1177/10668969221105626. Epub 2022 Jun 12.
Radiation-associated sarcomas are an uncommon complication of therapeutic radiation. However, their prevalence has increased with the more widespread use of this treatment modality. The clinical, pathologic and genetic characteristics of radiation-associated sarcomas are not fully understood. In this study we describe the features of 94 radiation-associated sarcomas reviewed at our institution between 1993 and 2018, evaluate their overall survival (OS) and progression-free survival (PFS) outcomes, and compare them with their sporadic counterparts reviewed within the same time period. Histologic subtypes of all radiation-associated sarcomas included 31 (33%) undifferentiated sarcomas, 20 (21%) osteosarcomas, 17 (18%) angiosarcomas, 10 (11%) malignant peripheral nerve sheath tumor (MPNST), 9 (10%) leiomyosarcomas, 4 (4%) myxofibrosarcomas, and 3 (3%) rhabdomyosarcomas. Six patients had a documented cancer predisposition syndrome. The most common preceding neoplasms included adenocarcinoma (47%) and squamous cell carcinoma (19%), with a mean latency of 13 years. Multivariable Cox survival analysis demonstrated that advanced stage at diagnosis based on pT category (AJCC eighth edition) and fragmented resection were associated with worse survival outcomes. In addition, there was a statistically significant difference in PFS between radiation-associated undifferentiated sarcomas and MPNST when compared to their sporadic counterparts using the Kaplan-Meier method and Log-rank analysis. Overall, our study shows that radiation-associated sarcomas comprise a wide clinico-pathologic spectrum of disease, with a tendency for aggressive clinical behavior. This study further delineates the understanding of these uncommon diseases. Future studies are necessary to better understand the genetic and epigenetic changes that drive the differences in behavior between these tumors and their sporadic counterparts, and to offer better treatment options.
放射性肉瘤是治疗性放疗的一种罕见并发症。然而，随着这种治疗方式的广泛应用，其发病率有所增加。放射性肉瘤的临床、病理和遗传特征尚未完全了解。在本研究中，我们描述了我院 1993 年至 2018 年间回顾性分析的 94 例放射性肉瘤的特征，评估了它们的总生存（OS）和无进展生存（PFS）结果，并与同期回顾性分析的散发性肉瘤进行了比较。所有放射性肉瘤的组织学亚型包括 31 例（33%）未分化肉瘤、20 例（21%）骨肉瘤、17 例（18%）血管肉瘤、10 例（11%）恶性外周神经鞘瘤（MPNST）、9 例（10%）平滑肌肉瘤、4 例（4%）黏液纤维肉瘤和 3 例（3%）横纹肌肉瘤。6 例患者有明确的癌症易感性综合征病史。最常见的前发肿瘤包括腺癌（47%）和鳞状细胞癌（19%），潜伏期平均为 13 年。多变量 Cox 生存分析表明，根据 pT 分类（AJCC 第八版）诊断时的晚期分期和碎片状切除与较差的生存结果相关。此外，使用 Kaplan-Meier 方法和 Log-rank 分析，与散发性肉瘤相比，放射性肉瘤中未分化肉瘤和 MPNST 之间在 PFS 方面存在显著统计学差异。总的来说，我们的研究表明，放射性肉瘤是一种广泛的临床病理疾病谱，具有侵袭性的临床行为倾向。本研究进一步阐明了对这些罕见疾病的认识。未来的研究对于更好地理解这些肿瘤与散发性肉瘤之间行为差异的遗传和表观遗传变化，以及提供更好的治疗选择是必要的。
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