Min K W, Leabu M
Department of Pathology, Deaconess Hospital, University of Oklahoma College of Medicine, Oklahoma City, OK 73112, USA.
J Cell Mol Med. 2006 Oct-Dec;10(4):995-1013. doi: 10.1111/j.1582-4934.2006.tb00541.x.
Interstitial cells of Cajal (ICC) is a peculiar cell network composed of cells having processes described by the eminent Spanish neuroanatomist of the 19th century, S. Ramon y Cajal. ICC became a fascinating subject to many investigators and it is estimated that there are over 100 publications yearly on the subject related to ICC, in the last three years. Now it is widely accepted that ICC are pace maker cells of the gut and probable progenitor cells of gastrointestinal stromal tumors (GIST). Lately, interstitial Cajal-like cells (ICLC) are being found in various organs and their physiological role is still to be defined. We have reviewed the literature trying to evaluate the validity of the current concept and found that there are a few salient points to be considered. 1) There has been some important departure in defining the identity of ICC from the original criteria of Cajal. In particular, ICC with myoid feafures in intestinal smooth muscle layers (ICC-DPM) do not seem to fit to the original description of interstitial cell network by Cajal. We have also pointed out that the current reports assigning a pace maker role to ICC vastly depend on the scientific data on "ICC with myoid features", not on "fibroblast-like ICC", which are more abundant and easier to identify. 2) There seem to be an overwhelming amount of data proving the relationship between ICC and GIST. Both are known to express c-Kit and the ultrastructural characteristics seen in GIST roughly parallel those of ICC including minimal myoid differentiation seen in the majority of GIST, supporting the current concept that GIST are ICC tumors. 3) According to the original description of Cajal, ICC was not limited to the gut, suggesting an existence of ICC in other organs. The list of organs reported to contain ICC (currently identified by immunohistochemistry and electron microscopy) is ever growing and further studies are needed to define their identity and pathophysiologic role. 4). Recent data concerning gut development suggest that both c-Kit expressing ICC (fibroblasts-like as well as muscle-like) and gut muscle cells derive from the common progenitor cells of the embryonic gut unifying the histogenetic concept of all GIST with heterogeneous cytomorphologic features. In this review we attempted to incorporate recent information on interstitial Cajal-like cells (ICLC) found in other organs to broaden our understanding of ICC in general in terms of their ultrastructure, physiology, and neoplasia.
Cajal间质细胞（ICC）是一种特殊的细胞网络，由具有突起的细胞组成，这种细胞由19世纪杰出的西班牙神经解剖学家S. Ramon y Cajal描述。ICC成为众多研究者感兴趣的课题，据估计在过去三年中，每年有超过100篇关于ICC的相关出版物。现在人们普遍认为，ICC是肠道的起搏细胞，也是胃肠道间质瘤（GIST）可能的祖细胞。最近，在各种器官中发现了间质 Cajal样细胞（ICLC），其生理作用仍有待确定。我们回顾了相关文献，试图评估当前概念的有效性，发现有几个要点需要考虑。1）在定义ICC的身份方面，与 Cajal的原始标准存在一些重要差异。特别是，肠道平滑肌层中具有肌样特征的ICC（ICC-DPM）似乎不符合 Cajal对间质细胞网络的原始描述。我们还指出，目前认为ICC具有起搏细胞作用的报道很大程度上依赖于关于“具有肌样特征的ICC”的科学数据，而不是“成纤维细胞样ICC”，后者数量更多且更容易识别。2）似乎有大量数据证明了ICC与GIST之间的关系。两者都已知表达c-Kit，并且GIST中所见的超微结构特征大致与ICC的特征平行，包括大多数GIST中所见的最小肌样分化，这支持了当前GIST是ICC肿瘤的概念。3）根据 Cajal的原始描述，ICC并不局限于肠道，这表明在其他器官中也存在ICC。据报道含有ICC的器官列表（目前通过免疫组织化学和电子显微镜鉴定）不断增加，需要进一步研究来确定它们的身份和病理生理作用。4）最近关于肠道发育的数据表明，表达c-Kit的ICC（成纤维细胞样以及肌样）和肠道肌肉细胞都来源于胚胎肠道的共同祖细胞，这统一了所有具有异质细胞形态特征的GIST的组织发生学概念。在这篇综述中，我们试图纳入关于在其他器官中发现的间质 Cajal样细胞（ICLC）的最新信息，以从超微结构、生理学和肿瘤形成方面更全面地理解ICC。
