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新生儿膈疝合并肝间叶性错构瘤 1 例报告。

Hepatic mesenchymal hamartoma in a neonate with a Bochdalek diaphragmatic hernia: a case report.

机构信息

Department of Anatomic Pathology, Hospital Universitario Puerta de Hierro, Majadahonda 28222, Spain.

出版信息

J Pediatr Surg. 2012 Nov;47(11):e45-8. doi: 10.1016/j.jpedsurg.2012.07.053.

Abstract

The Bochdalek diaphragmatic hernia (BDH) is a common birth defect that is associated with other congenital malformations. Hepatic mesenchymal hamartoma (HMH) is the second most common benign hepatic tumor in children. The association between BDH and HMH or ectopic hepatic tissue is uncommon. We report a case of a female newborn with a left BDH that at surgery was incidentally found to have a tumor of 3.5 cm in maximum dimension with a serous muscular membrane. The histopathological study revealed vascular-mesenchymal stroma with bile ducts entrapped and peripheral normal hepatic tissue. These findings correlate with the diagnosis of hepatic mesenchymal hamartoma (HMH) in ectopic hepatic tissue. Our objective is to report a case of a rare association between HMH and BDH as well as discuss its differential diagnosis.

摘要

波德加雷克膈疝(Bochdalek diaphragmatic hernia,BDH)是一种常见的先天性膈疝,常伴有其他先天性畸形。肝间叶性错构瘤(hepatic mesenchymal hamartoma,HMH)是儿童期第二常见的良性肝肿瘤。BDH 与 HMH 或异位肝组织之间的关联并不常见。我们报告了一例女性新生儿,左侧 BDH,在手术中偶然发现一个最大径为 3.5 厘米的肿瘤,有浆液性肌膜。组织病理学研究显示,血管间叶基质中有胆管嵌入,周围是正常肝组织。这些发现与异位肝组织中肝间叶性错构瘤(HMH)的诊断相符。我们的目的是报告一例 HMH 与 BDH 之间罕见的关联,并讨论其鉴别诊断。

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