Ng Deanna, Bertrand Alexander, Sanfilippo Roberta, Callegaro Dario
Department of Surgery, University of Toronto, Toronto, ON, Canada.
Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
Expert Rev Anticancer Ther. 2023 Jul-Dec;23(11):1169-1178. doi: 10.1080/14737140.2023.2266137. Epub 2023 Oct 27.
Retroperitoneal sarcomas (RPS) are rare mesenchymal tumors that account for only 0.1-0.2% of all malignancies. Management of this disease is challenging, and resection remains the cornerstone of treatment. Ongoing international collaboration has expanded our knowledge of this disease, allowing for a more personalized approach to RPS patients resulting in improved survival over time. Due to the heterogeneity of RPS, with differing recurrence patterns and sensitivities to neoadjuvant therapies based on histology and grade, management of RPS should be tailored to the individual patient.
Our review focuses on a histology-driven approach in the management of primary RPS. We searched relevant articles from 1993 to 2023 that investigated prognostic factors and treatment of patients with RPS and summarized recent advances and future directions in the field.
Deeper understanding of the role of neoadjuvant radiotherapy and ongoing trials investigating the role of neoadjuvant chemotherapy will potentially contribute to the development of individualized treatment pathways.
腹膜后肉瘤（RPS）是罕见的间充质肿瘤，仅占所有恶性肿瘤的0.1-0.2%。这种疾病的管理具有挑战性，手术切除仍然是治疗的基石。正在进行的国际合作扩展了我们对这种疾病的认识，使我们能够对RPS患者采取更个性化的治疗方法，从而随着时间的推移提高生存率。由于RPS的异质性，其复发模式不同，且根据组织学和分级对新辅助治疗的敏感性不同，RPS的管理应根据个体患者进行调整。
我们的综述重点关注原发性RPS管理中基于组织学的方法。我们检索了1993年至2023年期间研究RPS患者预后因素和治疗的相关文章，并总结了该领域的最新进展和未来方向。
对新辅助放疗作用的更深入理解以及正在进行的研究新辅助化疗作用的试验可能会有助于个性化治疗方案的发展。
