本主题综述旨在概述关于肝实质细胞中巨型线粒体或超线粒体出现的当前知识状态。它们的存在和积聚被认为是肝实质细胞的主要病理学标志，会导致组织整体恶化，最终导致器官功能衰竭。关于巨型线粒体的首次描述可以追溯到上世纪60年代，与临床诊断实验室中第一代电子显微镜的可用性相吻合。详细的超微结构描述主要出现在患有酒精性肝病、慢性肝炎、肝细胞癌和非酒精性肥肝病的患者中。有趣的是，从这次广泛的文献调查中可以明显看出，巨型线粒体或超线粒体可以有或没有高度有序的类晶体样线粒体内含物。巨型线粒体的起源、形成和潜在作用至今仍然没有明确答案。同样，有关这些井然有序的类晶体样内含物的生化组成及其对线粒体功能的可能影响还不清楚。在本文中，将探讨它们形成的可能机制和三维结构的概念。此外，我们还将讨论它们在诊断中的重要性，包括未来的研究展望和针对引入巨型线粒体的肝脏疾病的潜在治疗干预措施。©2023 The Authors. Liver International由John Wiley＆Sons Ltd.出版。

This thematic review aims to provide an overview of the current state of knowledge about the occurrence of giant mitochondria or megamitochondria in liver parenchymal cells. Their presence and accumulation are considered to be a major pathological hallmark of the health and fate of liver parenchymal cells that leads to overall tissue deterioration and eventually results in organ failure. The first description on giant mitochondria dates back to the 1960s, coinciding with the availability of the first generation of electron microscopes in clinical diagnostic laboratories. Detailed accounts on their ultrastructure have mostly been described in patients suffering from alcoholic liver disease, chronic hepatitis, hepatocellular carcinoma and non-alcoholic fatty liver disease. Interestingly, from this extensive literature survey, it became apparent that giant mitochondria or megamitochondria present themselves with or without highly organised crystal-like intramitochondrial inclusions. The origin, formation and potential role of giant mitochondria remain to-date largely unanswered. Likewise, the biochemical composition of the well-organised crystal-like inclusions and their possible impact on mitochondrial function is unclear. Herein, concepts about the possible mechanism of their formation and three-dimensional architecture will be approached. We will furthermore discuss their importance in diagnostics, including future research outlooks and potential therapeutic interventions to cure liver disease where giant mitochondria are implemented.© 2023 The Authors. Liver International published by John Wiley & Sons Ltd.