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Abstract：
This review aims to provide a comprehensive overview of mesenchymal sinonasal tract tumors (STTs), a distinct subset of STTs. Despite their rarity, mesenchymal STTs represent a unique clinical challenge, characterized by their rarity, often slow progression, and frequently subtle or overlooked symptoms. The complex anatomy of the sinonasal area, which includes critical structures such as the orbit, brain, and cranial nerves, further complicates surgical treatment options. This underscores an urgent need for more advanced and specialized therapeutic approaches.
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.
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.
摘要：
目的：这篇综述旨在提供间叶性鼻窦道肿瘤（STTs）的全面概述，STT的不同子集。尽管它们很少,间充质STT代表了一个独特的临床挑战，以它们的稀有性为特征，通常进展缓慢，和经常微妙或被忽视的症状。鼻窦区域的复杂解剖结构，其中包括轨道等关键结构，大脑,和颅神经，进一步复杂的手术治疗选择。这强调了迫切需要更先进和专门的治疗方法。
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。
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。
