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先天性肝母细胞瘤合并肝动静脉瘘:一例报告

Congenital hepatoblastoma presenting with hepatic arteriovenous fistulas: a case report.

作者信息

Lin Jiao, Guo Jialing, Chen Chun, Jiang Liqiong, Lai Can, Wang Chunlin

机构信息

Department of Pediatrics, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.

Department of Radiology, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China.

出版信息

Front Pediatr. 2023 Jul 13;11:1199224. doi: 10.3389/fped.2023.1199224. eCollection 2023.

Abstract

AIM

Congenital hepatoblastoma, a rare malignant liver tumor in infancy, typically presents with abdominal distension or mass. Tumors detected antenatally or during the first three months of age are considered congenital hepatoblastoma. Hepatic arteriovenous fistulas (HAVF) are associated with high mortality in the neonatal period and can be caused by many secondary factors. This case report focuses on a patient with congenital hepatoblastoma accompanied by HAVF, highlighting the clinical and imaging characteristics and management strategies.

CASE PRESENTATION

A term infant presented with sudden tachypnea and heart failure on his first day of life. A cystic-solid mixed lesion in the fetus's liver was detected by an antenatal ultrasound scan. Postnatal digital subtraction angiography confirmed the presence of arteriovenous fistulas, which were treated with trans-arterial embolization. However, despite the intervention, the patient's heart failure did not improve. The patient underwent a left hepatectomy, and hepatoblastoma was discovered by histology of the resected hepatic lobe. Unfortunately, metastases were later discovered in the intracranial and ocular regions. Ultimately, the family decided to discontinue further treatment.

CONCLUSION

Congenital hepatoblastoma presenting with hepatic arteriovenous fistulas has not been previously described. Hepatoblastoma should be considered when alpha-fetoprotein levels show a significant elevation in newborns. Prenatal diagnosis may improve pre- and postnatal management.

摘要

目的

先天性肝母细胞瘤是婴儿期罕见的恶性肝脏肿瘤,通常表现为腹胀或肿块。产前或出生后三个月内检测到的肿瘤被认为是先天性肝母细胞瘤。肝动静脉瘘(HAVF)在新生儿期与高死亡率相关,可由多种继发因素引起。本病例报告聚焦于一名伴有HAVF的先天性肝母细胞瘤患者,突出其临床和影像学特征及管理策略。

病例介绍

一名足月儿在出生第一天出现突发呼吸急促和心力衰竭。产前超声扫描检测到胎儿肝脏有一个囊实性混合病变。产后数字减影血管造影证实存在动静脉瘘,采用经动脉栓塞治疗。然而,尽管进行了干预,患者的心力衰竭并未改善。患者接受了左肝切除术,切除的肝叶组织学检查发现肝母细胞瘤。不幸的是,后来在颅内和眼部区域发现了转移灶。最终,家属决定停止进一步治疗。

结论

此前尚未描述过伴有肝动静脉瘘的先天性肝母细胞瘤。当新生儿甲胎蛋白水平显著升高时,应考虑肝母细胞瘤。产前诊断可能改善产前和产后管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dbd2/10373925/4e41f30e0574/fped-11-1199224-g001.jpg

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