Department of Pathology, Brigham and Women's Hospital/Harvard Medical School, Boston, MA.
Division of Vascular & Interventional Radiology, Johns Hopkins University, School of Medicine, Baltimore, MD.
Am Soc Clin Oncol Educ Book. 2020 May;40:445-462. doi: 10.1200/EDBK_280729.
Sarcomas are rare tumors but comprise a wide histologic spectrum. Advances in technology have emerged to address the biologic complexity and challenging diagnosis and treatment of this disease. The diagnostic approach to sarcomas has historically been based on morphologic features, but technologic advances in immunohistochemistry and cytogenetic/molecular testing have transformed the interdisciplinary work-up of mesenchymal neoplasms in recent years. On the therapeutic side, technologic advances in the delivery of radiation have made it a linchpin in the treatment of localized and oligometastatic sarcoma. In this review, we discuss recent advances in the pathologic diagnosis of sarcomas and discuss select sarcoma types that illustrate how newly discovered diagnostic, prognostic, and predictive biomarkers have refined existing classification schemes and substantially shaped our diagnostic approach. Such examples include conventional and epithelioid malignant peripheral nerve sheath tumors (MPNSTs), emerging entities in the group of round cell sarcomas, and other mesenchymal neoplasms with distinct cytogenetic aberrations. Recent advances in radiation oncology, including intensity-modulated, stereotactic, MRI-guided, and proton radiotherapy (RT), will be reviewed in the context of neoadjuvant or adjuvant localized soft-tissue sarcoma and oligometastatic or oligoprogressive disease. Innovations in translational research are expected to be introduced into clinical practice over the next few years and will likely continue to affect the rapidly evolving field of sarcoma diagnostics and therapy.
肉瘤是罕见的肿瘤,但包含广泛的组织学谱。技术的进步已经出现,以解决这种疾病的生物学复杂性和具有挑战性的诊断和治疗。肉瘤的诊断方法历来基于形态学特征,但免疫组织化学和细胞遗传学/分子检测技术的进步近年来改变了间充质肿瘤的跨学科研究。在治疗方面,放射治疗技术的进步使其成为局部和寡转移肉瘤治疗的关键。在这篇综述中,我们讨论了肉瘤病理诊断的最新进展,并讨论了一些选择的肉瘤类型,这些类型说明了新发现的诊断、预后和预测生物标志物如何完善现有的分类方案,并极大地影响了我们的诊断方法。例如,包括常规和上皮样恶性外周神经鞘瘤(MPNST)、圆形细胞肉瘤组中的新兴实体,以及具有独特细胞遗传学异常的其他间充质肿瘤。在局部软组织肉瘤和寡转移或寡进展性疾病的新辅助或辅助治疗背景下,将回顾放射肿瘤学的最新进展,包括强度调制、立体定向、MRI 引导和质子放射治疗(RT)。在未来几年内,转化研究的创新有望引入临床实践,并可能继续影响肉瘤诊断和治疗这一快速发展的领域。