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一名需要肝移植的婴儿肝脏发育停滞,同时患有婴儿型血管内皮瘤和间叶性错构瘤。

Concurrent infantile hemangioendothelioma and mesenchymal hamartoma in a developmentally arrested liver of an infant requiring hepatic transplantation.

作者信息

Bejarano Pablo A, Serrano Maria F, Casillas Javier, Dehner Louis P, Kato Tomoaki, Mitral Naveen, Rodriguez Maria M, Tzakis Andreas

机构信息

Department of Pathology, University of Miami School of Medicine, Jackson Memorial Hospital, 1611 NW 12th Avenue, Holtz Center-Room 2042, Miami, FL 33136, USA.

出版信息

Pediatr Dev Pathol. 2003 Nov-Dec;6(6):552-7. doi: 10.1007/s10024-003-3024-7.

Abstract

A newborn female underwent a surgical resection for a hepatic mass discovered prenatally by ultrasonography, and diagnosed pathologically as a mesenchymal hamartoma. Within 4 months after surgery, multiple cutaneous hemangiomas developed and a multinodular mass was detected in the liver. A liver biopsy showed an infantile hemangioendothelioma with type I features. An orthotopic liver transplant was performed due to the extensive nature of the hepatic involvement and progressive respiratory compromise. Virtually the entire liver was involved by a large infantile hemangioendothelioma. A multicystic mesenchymal hamartoma was also found on the left side. In addition, the uninvolved hepatic parenchyma had features recapitulating the fetal liver. This simultaneous involvement of the liver by a mesenchymal hamartoma and infantile hemangioendothelioma is unique. A review of the literature revealed only one incompletely characterized case with similar findings.

摘要

一名新生女婴因产前超声检查发现肝脏肿块而接受了手术切除,病理诊断为间叶性错构瘤。术后4个月内,出现了多个皮肤血管瘤,肝脏中检测到一个多结节肿块。肝脏活检显示为具有I型特征的婴儿型血管内皮瘤。由于肝脏受累范围广泛且进行性呼吸功能不全,进行了原位肝移植。实际上,整个肝脏都被一个巨大的婴儿型血管内皮瘤所累及。左侧还发现了一个多囊性间叶性错构瘤。此外,未受累的肝实质具有类似胎儿肝脏的特征。肝脏同时被间叶性错构瘤和婴儿型血管内皮瘤累及的情况很独特。文献回顾仅发现一例特征描述不完整的类似病例。

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