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先天性肝母细胞瘤的产前宫内诊断。

Antenatal diagnosis of congenital hepatoblastoma in utero.

作者信息

Shih J C, Tsao P N, Huang S F, Yen B L, Lin J H, Lee C N, Hsieh F J

机构信息

Department of Obstetrics and Gynecology, College of Medicine, National Taiwan University Hospital, Taipei, Taiwan.

出版信息

Ultrasound Obstet Gynecol. 2000 Jul;16(1):94-7. doi: 10.1046/j.1469-0705.2000.00168.x.

Abstract

A fetus with a huge hepatic tumor was detected by sonography at 36 weeks of gestation. The mass appeared as a single, solid and polylobular tumor located in the right lobe of the liver. Foci of hemorrhage, necrosis and some tiny calcifications were seen. The adjacent right kidney appeared normal but was displaced. The right adrenal gland was not visualized. Three-dimensional power Doppler sonography further depicted the corresponding vascular anatomy of the tumor, including its vascularization pattern and blood supply. The tumor was situated to the right of the umbilical vein and portal sinus, possibly deriving its blood supply from the portal circulation. The fundamental findings suggested the diagnosis of hepatoblastoma by exclusion of other possibilities. The baby was delivered by Cesarean section at 36 weeks' gestation, due to signs of fetal distress. Unfortunately, hypotension, tachycardia, and tachypnea developed shortly after birth. Surgical intervention was performed, but intractable bleeding occurred intra-operatively. The infant died at 6 days of age. Autopsy confirmed the diagnosis of hepatoblastoma. We believe this is the first reported case of the antenatal diagnosis of congenital hepatoblastoma.

摘要

妊娠36周时通过超声检查发现一名胎儿患有巨大肝肿瘤。肿块表现为位于肝右叶的单个实性多叶肿瘤。可见出血、坏死灶及一些微小钙化。相邻的右肾外观正常但移位。未见到右肾上腺。三维能量多普勒超声进一步描绘了肿瘤相应的血管解剖结构,包括其血管化模式和血供。肿瘤位于脐静脉和门静脉窦右侧,可能从门静脉循环获得血供。基本检查结果通过排除其他可能性提示肝母细胞瘤的诊断。由于胎儿窘迫迹象,婴儿在妊娠36周时通过剖宫产分娩。不幸的是,出生后不久出现低血压、心动过速和呼吸急促。进行了手术干预,但术中发生难以控制的出血。婴儿于6日龄死亡。尸检证实为肝母细胞瘤。我们认为这是先天性肝母细胞瘤产前诊断的首例报道病例。

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