Advancements in molecular diagnostics, particularly in next-generation sequencing, have significantly enhanced our understanding of STTs. Consequently, the World Health Organization has updated its tumor classification to better reflect the distinct histological and molecular profiles of these tumors, as well as to categorize mesenchymal STTs with greater accuracy. The growing understanding of the molecular characteristics of mesenchymal STTs opens new possibilities for targeted therapeutic interventions, marking a significant shift in treatment paradigms. This review article concentrates on mesenchymal STTs, specifically addressing sinonasal tract angiofibroma, sinonasal glomangiopericytoma, biphenotypic sinonasal sarcoma, and skull base chordoma. These entities are marked by unique histopathological and molecular features, which challenge conventional treatment approaches and simultaneously open avenues for novel targeted therapies. Our discussion is geared towards delineating the molecular underpinnings of mesenchymal STTs, with the objective of enhancing therapeutic strategies and addressing the existing shortcomings in the management of these intricate tumors.
结果:分子诊断的进步,特别是在下一代测序中,大大增强了我们对STT的理解。因此,世界卫生组织更新了其肿瘤分类,以更好地反映这些肿瘤的独特组织学和分子特征,以及对间充质STT进行更准确的分类。对间充质STT分子特征的日益理解为靶向治疗干预开辟了新的可能性。标志着治疗范式的显著转变。这篇综述文章集中在间充质STT,专门针对鼻窦血管纤维瘤,鼻窦血管外皮细胞瘤,双表型鼻腔鼻窦肉瘤,和颅底脊索瘤.这些实体具有独特的组织病理学和分子特征,这挑战了传统的治疗方法,同时为新型靶向治疗开辟了道路。我们的讨论旨在描绘间充质STT的分子基础,目的是加强治疗策略并解决这些复杂肿瘤管理中的现有缺陷。