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先天性肝血管瘤的临床特征与管理:一项211例病例的队列研究

Clinical characteristics and managements of congenital hepatic hemangioma: a cohort study of 211 cases.

作者信息

Gong Xue, Yang Min, Zhang Zixin, Qiu Tong, Zhou Jiangyuan, Shan Wei, Zhang Xuepeng, Lan Yuru, Bao Pingqian, Zhou Zilong, Yang Congxia, Zhang Yujia, Li Tianliang, Guo Jing, Guo Jun, Lu Guoyan, Kong Feiteng, Zhang Yongbo, Chen Siyuan, Ji Yi

机构信息

Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, 610041, China.

Department of Pediatric Surgery, Sichuan Women and Children's Hospital, Chengdu, 610045, China.

出版信息

Hepatol Int. 2025 Jun;19(3):682-691. doi: 10.1007/s12072-024-10756-5. Epub 2024 Nov 29.

Abstract

BACKGROUND

Hepatic hemangiomas can be classified into three morphologic patterns: focal (congenital hepatic hemangiomas [CHHs]), multifocal, and diffuse. We aimed to identify the clinical characteristics of CHH and evaluate the changes in CHH management at our institution over the last 2 decades.

METHODS

This was a retrospective cohort study of children diagnosed with CHH who were managed at 8 investigation sites. The primary outcome was changes in CHH size in patients at the last follow-up. The primary exposure of interest was management modality in 2 study periods (2003-2012 versus 2013-2022).

RESULTS

Two hundred and eleven patients were analyzed. Four different subtypes of CHH were identified. Rapidly involuting CHH patients had complete involution/nearly complete involution, with a median age of 12.0 months. Noninvoluting CHH presented no change in CHH size. Partially involuting CHH patients presented with partial involution and had a stable tumor size at a median age of 16.0 months. Postnatally proliferating CHHs had an initial postnatal increase in CHH lesion size and underwent involution at a median age of 27.0 months. Further analysis revealed that management strategies for CHHs have shifted over time, with the proportion of patients receiving expectant management increasing from 35.4% before 2013 to 77.7% after 2013 (difference, 42.3%; 95% confidence interval 29.3-53.3%). The survival rate of patients with CHH was high (98.6%).

CONCLUSIONS

We documented 4 subtypes of CHHs. We found that expectant management strategies have increasingly replaced invasive interventions in patients with CHH over the past 2 decades.

RESEARCH REGISTRATION UNIQUE IDENTIFYING NUMBER (UIN): We have already registered at Clinicaltrials.gov. The UIN number is NCT03331744.

摘要

背景

肝血管瘤可分为三种形态学类型:局灶性(先天性肝血管瘤[CHH])、多灶性和弥漫性。我们旨在确定CHH的临床特征,并评估过去20年我院CHH治疗的变化。

方法

这是一项对在8个研究地点接受治疗的诊断为CHH的儿童进行的回顾性队列研究。主要结局是患者在最后一次随访时CHH大小的变化。主要关注的暴露因素是两个研究时期(2003 - 2012年与2013 - 2022年)的治疗方式。

结果

分析了211例患者。确定了CHH的4种不同亚型。快速消退型CHH患者完全消退/几乎完全消退,中位年龄为12.0个月。非消退型CHH的CHH大小无变化。部分消退型CHH患者出现部分消退,中位年龄16.0个月时肿瘤大小稳定。出生后增殖型CHH出生后CHH病变大小最初增加,中位年龄27.0个月时开始消退。进一步分析显示,CHH的治疗策略随时间发生了变化,接受观察等待治疗的患者比例从2013年前的35.4%增加到2013年后的77.7%(差异为42.3%;95%置信区间29.3 - 53.3%)。CHH患者的生存率很高(98.6%)。

结论

我们记录了CHH的4种亚型。我们发现,在过去20年中,观察等待治疗策略越来越多地取代了CHH患者的侵入性干预措施。

研究注册唯一识别号(UIN):我们已在Clinicaltrials.gov注册。UIN编号为NCT03331744。

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