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肝间叶性错构瘤:一例报告

Mesenchymal hamartoma of the liver: a case report.

作者信息

Tzen C Y, Chen B F, Chang P Y, Wang N L

机构信息

Department of Pathology, Mackay Memorial Hospital, Taipei, Taiwan, ROC.

出版信息

Zhonghua Yi Xue Za Zhi (Taipei). 1998 Jul;61(7):427-31.

PMID:9699396
Abstract

Mesenchymal hamartoma of the liver (MHL) is a rare lesion occurring mainly in infants and children. It is often misdiagnosed clinically as a malignant tumor because of its rapid increase in size within a short period of time, or as a hepatic cyst or abscess because of its cystic appearance. Although a benign lesion, MHL may cause heart failure, due to arteriovenous shunts, or death if untreated, as a result of respiratory complications. A typical case of MHL was recently encountered in a 15-month-old boy. The patient presented with progressive abdominal distension; surgery revealed a large mass arising from the right lobe of the liver. The mass was predominantly solid, but collections of fluid were also present. Loose mesenchymal tissue and branched, tortuous bile ducts were the key diagnostic features. When predominantly cystic, MHL may mimic a lymphangioma both grossly and microscopically. Prudent examination of the cystic structures can establish a correct diagnosis.

摘要

肝间叶性错构瘤(MHL)是一种主要发生于婴幼儿的罕见病变。由于其在短时间内迅速增大,临床上常被误诊为恶性肿瘤;又因其呈囊性外观,也常被误诊为肝囊肿或肝脓肿。尽管MHL是良性病变,但由于动静脉分流可能导致心力衰竭,若不治疗,也可能因呼吸并发症而死亡。最近,一名15个月大的男孩被诊断为典型的MHL病例。该患者表现为进行性腹胀;手术发现肝脏右叶有一个大肿块。肿块主要为实性,但也有液体积聚。疏松的间叶组织和分支、迂曲的胆管是关键的诊断特征。当以囊性为主时,MHL在大体和显微镜下都可能类似淋巴管瘤。对囊性结构进行仔细检查可做出正确诊断。

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