阑尾癌（AC）是一种罕见且异质的恶性肿瘤。历史上，阑尾肿瘤被归类为结直肠肿瘤（CRC），治疗策略参照CRC管理指南，因其结构上的相似性和解剖上的邻近性。然而，两者在生物行为和治疗反应方面有显著差异，证据表明它们在基因组上存在明显差异。此外，虽然目前针对阑尾癌的WHO分类仍基于传统组织病理学标准，但研究表明组织学形态与AC的生存或治疗反应无相关性。由于其罕见性，阑尾癌的研究没有像其他胃肠道癌症一样被广泛研究。然而，过去十年间其发病率逐渐增加，这使得寻找新的、更有效的检测和治疗策略变得至关重要。最近对阑尾癌的分子景观进行的研究和理解已揭示了大量信息，这表明阑尾癌具有独特的分子特征，与其他胃肠道癌症不同。本综述主要关注上皮型阑尾癌的表观遗传景观，并旨在全面概述不同阑尾癌亚型的表观遗传变化的现状，并强调在寻找上皮型阑尾肿瘤的生物标志物、治疗靶点、监测标志物以及预测治疗反应和生存的挑战和前景中，应用表观遗传学的潜力。

Appendiceal cancers (AC) are a rare and heterogeneous group of malignancies. Historically, appendiceal neoplasms have been grouped with colorectal cancers (CRC), and treatment strategies have been modeled after CRC management guidelines due to their structural similarities and anatomical proximity. However, the two have marked differences in biological behavior and treatment response, and evidence suggests significant discrepancies in their respective genetic profiles. In addition, while the WHO classification for appendiceal cancers is currently based on traditional histopathological criteria, studies have demonstrated that histomorphology does not correlate with survival or treatment response in AC. Due to their rarity, appendiceal cancers have not been studied as extensively as other gastrointestinal cancers. However, their incidence has been increasing steadily over the past decade, making it crucial to identify new and more effective strategies for detection and treatment. Recent efforts to map and understand the molecular landscape of appendiceal cancers have unearthed a wealth of information that has made it evident that appendiceal cancers possess a unique molecular profile, distinct from other gastrointestinal cancers. This review focuses on the epigenetic landscape of epithelial appendiceal cancers and aims to provide a comprehensive overview of the current state of knowledge of epigenetic changes across different appendiceal cancer subtypes, highlighting the challenges as well as the promise of employing epigenetics in the quest for the detection of biomarkers, therapeutic targets, surveillance markers, and predictors of treatment response and survival in epithelial appendiceal neoplasms.