Suppr超能文献

成人肝脏间叶性错构瘤:与独特临床特征及组织学改变的关联

Mesenchymal hamartoma of the liver in the adult: association with distinct clinical features and histological changes.

作者信息

Cook James R, Pfeifer John D, Dehner Louis P

机构信息

Lauren V. Ackerman Laboratory of Surgical Pathology, Barnes-Jewish and St. Louis Children's Hospitals, Washington University Medical Center, St. Louis, MO, USA.

出版信息

Hum Pathol. 2002 Sep;33(9):893-8. doi: 10.1053/hupa.2002.127442.

Abstract

Mesenchymal hamartoma of the liver (MHL) is an uncommon mass lesion composed of architecturally abnormal bile ducts in an uncommitted myxoid stroma. Most MHL are diagnosed in childhood. More than 50% of cases are seen in the first year of life, although a few cases have been previously reported in adults. The spectrum of pathological findings in the cases presenting in adults, including differences in comparison with MHL in children, has not been fully characterized. In this report, we describe 3 cases of MHL in patients 46, 63, and 66 years of age. Each of the patients was a woman who had a solid or multicystic hepatic mass ranging from 5 to 24 cm in maximum dimension. In contrast to the childhood cases, the stromal component was fibrotic with areas of dense hyalinization and only focal myxoid areas. In 1 case, the mesenchymal component was the predominant feature of the lesion, with only occasional ductal elements identified with thorough tissue sampling. Immunohistochemical analysis with a panel of antibodies showed that, as in normal bile ducts, the ductal structures within the lesion were immunoreactive for cytokeratin 7 and negative for cytokeratin 20. The stroma was composed of a prominent population of fibroblasts and myofibroblasts that were positive for smooth muscle actin and vimentin. Analysis of the current findings, together with the previous case reports, shows that in contrast to MHL in children, this lesion in adults is found more commonly in women who present with abdominal pain. Recognition of the clinicopathologic differences between adult and pediatric cases will facilitate accurate diagnosis of this uncommon lesion.

摘要

肝间叶性错构瘤(MHL)是一种罕见的肿块性病变,由未分化的黏液样基质中结构异常的胆管组成。大多数MHL在儿童期被诊断出来。超过50%的病例在出生后第一年被发现,尽管此前也有少数成人病例的报道。成人病例的病理表现谱,包括与儿童MHL相比的差异,尚未得到充分描述。在本报告中,我们描述了3例年龄分别为46岁、63岁和66岁的MHL患者。每位患者均为女性,肝脏有实性或多囊性肿块,最大直径为5至24厘米。与儿童病例不同,间质成分呈纤维化,有密集透明变性区域,仅局灶性黏液样区域。在1例中,间叶成分是病变的主要特征,经彻底组织取材仅偶尔发现导管成分。一组抗体的免疫组织化学分析显示,与正常胆管一样,病变内的导管结构细胞角蛋白7呈免疫反应性,细胞角蛋白20呈阴性。间质由大量成纤维细胞和平滑肌肌成纤维细胞组成,平滑肌肌动蛋白和波形蛋白呈阳性。对当前发现以及既往病例报告的分析表明,与儿童MHL不同,成人的这种病变更常见于出现腹痛的女性。认识成人和儿童病例之间的临床病理差异将有助于准确诊断这种罕见病变。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验