Abstract
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.