中耳胆脂瘤：生物学基础、侵袭性异质性及严重程度评估的挑战。

文献信息

中文摘要

目的： 中耳胆脂瘤（MEC）是一种良性但具有生物学侵袭性的中耳病变，以进行性和破坏性骨侵蚀为特征。然而，当前的评估系统仍存在异质性，且仅关注有限的解剖结构，导致临床预测价值不足。本综述旨在整合关于MEC相关骨侵蚀生物学机制及评估系统的现有证据，以期为开发更标准化且具有临床意义的严重程度评估模型提供信息。
方法： 在PubMed、Web of Science和Embase数据库中进行叙述性综述，以总结当前关于驱动MEC骨侵蚀机制的知识，并评估用于评估侵蚀严重程度的现有分期系统。关键词包括“胆脂瘤”、“骨侵蚀”、“骨吸收”、“分子机制”、“分期系统”及相关术语。
结果： 现有证据表明，MEC中的骨侵蚀源于过度增殖的上皮、慢性炎症与由细胞因子和信号通路（尤其是RANKL相关机制）介导的破骨细胞活化之间的相互作用。现有分期系统在很大程度上是描述性的，并表现出显著的异质性，这共同削弱了其在指导手术管理或预测患者结局方面的临床实用性。
结论： 当前的分期系统对于描述解剖范围有价值，但未能捕捉胆脂瘤的生物学异质性和侵袭潜力，因此不能完整地描述该疾病。未来的评估策略应整合分子机制、全面的解剖受累情况和影像学发现，以开发更标准化且具有临床意义的严重程度评估系统。

英文摘要

PURPOSE: Middle ear cholesteatoma (MEC) is a benign but biologically aggressive middle ear lesion characterized by progressive and destructive bone erosion. However, current evaluation systems remain heterogeneous and focus on limited anatomical structures, leading to insufficient clinical predictive value. This review aims to integrate current evidence on the biological mechanisms of MEC-associated bone erosion and assessment systems, with the goal of informing the development of more standardized and clinically meaningful severity evaluation models.
METHODS: A narrative review was performed in PubMed, Web of Science, and Embase databases to summarize current knowledge on the mechanisms driving bone erosion in MEC and to evaluate the existing staging systems for assessing erosion severity. Keywords included "cholesteatoma", "bone erosion", "bone resorption", "molecular mechanisms", "staging systems" and related terms.
RESULTS: Available evidence indicates that bone erosion in MEC arises from the interaction between hyperproliferative epithelium, chronic inflammation, and osteoclast activation mediated by cytokines and signaling pathways, particularly RANKL-related mechanisms. Existing staging systems are largely descriptive and exhibit significant heterogeneity, which collectively undermines their clinical utility in guiding surgical management or predicting patient outcomes.
CONCLUSION: Current staging systems, valuable for describing anatomical extension, fail to capture cholesteatoma's biological heterogeneity and invasive potential, thus incompletely depicting the disease. Future evaluation strategies should integrate molecular mechanisms, comprehensive anatomical involvement, and imaging findings to develop more standardized and clinically meaningful severity assessment systems.