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小儿肝细胞腺瘤:已知与新知

Pediatric Hepatocellular Adenomas: What Is Known and What Is New?

作者信息

Espinoza Andres F, Vasudevan Sanjeev A, Masand Prakash M, Lòpez-Terrada Dolores H, Patel Kalyani R

机构信息

Divisions of Pediatric Surgery and Surgical Research, Michael E. DeBakey Department of Surgery, Pediatric Surgical Oncology Laboratory, Texas Children's Surgical Oncology Program, Texas Children's Liver Tumor Program, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, TX 77030, USA.

Department of Radiology, Baylor College of Medicine and Texas Children's Hospital, Houston, TX 77030, USA.

出版信息

Cancers (Basel). 2023 Sep 29;15(19):4790. doi: 10.3390/cancers15194790.

Abstract

Current understanding and classification of pediatric hepatocellular adenomas (HCA) are largely based on adult data. HCAs are rare in children and, unlike in adults, are often seen in the context of syndromes or abnormal background liver. Attempts to apply the adult classification to pediatric tumors have led to several "unclassifiable" lesions. Although typically considered benign, few can show atypical features and those with beta-catenin mutations have a risk for malignant transformation. Small lesions can be monitored while larger (>5.0 cm) lesions are excised due to symptoms or risk of bleeding/rupture, etc. Management depends on gender, age, underlying liver disease, multifocality, size of lesion, histologic subtype and presence of mutation, if any. In this review, we summarize the data on pediatric HCAs and highlight our experience with their diagnosis and management.

摘要

目前对儿童肝细胞腺瘤(HCA)的认识和分类很大程度上基于成人数据。HCA在儿童中很少见,与成人不同的是,它常出现在综合征或肝脏背景异常的情况下。将成人分类应用于儿童肿瘤的尝试导致了一些“无法分类”的病变。虽然通常被认为是良性的,但少数可能表现出非典型特征,而那些具有β-连环蛋白突变的病变有恶变风险。小病变可进行监测,而较大(>5.0 cm)的病变由于症状或出血/破裂风险等原因需切除。治疗取决于性别、年龄、潜在肝病、多灶性、病变大小、组织学亚型以及是否存在突变(如有)。在本综述中,我们总结了有关儿童HCA的数据,并突出了我们在其诊断和治疗方面的经验。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fa2/10571754/b612c32b92ee/cancers-15-04790-g001.jpg

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